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PROTOCOL (Draft)
Interstitial Lung Disease India, Registry
www.ildindiaregistry.com
An Initiative of
Indian Chest Society
Interstitial Lung Disease Registry, India, Protocol
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CONTENTS
Background
3
Classification of ILD
4
Diagnostic tips of various types of ILD
5
Proforma
7
How to complete the proforma?
17
Consent form
32
Investigator’s terms and conditions
33
Interstitial Lung Disease Registry, India, Protocol
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Background
Interstitial lung disease (ILD) is a crippling pulmonary disease. Many patients do not respond to any treatment and
crave for oxygen even during trivial daily routine activities. Couple of decades back, ILD was considered a rare
disease but now respiratory physicians are getting more and more patients of ILD. Apparently, all ILD patients look
similar but there are different patterns of the disease. About 200 etiologic factors have been identified.
ILD is disease of lung parenchyma. Lung parenchyma consists of alveolar walls comprising of collagen, elastin,
capillary endothelium and interstitium. Broadly, the interstitium extends from the epithelium of the alveoli to the
endothelium of the capillaries. Interstitial lung disease (ILD) is a group of disorders which involves this part of the
lung.
Historical names of ILD
In the nineteenth century, lung fibrosis was called as ‘cirrhosis of the lung’. It was in 1944 that Louis Hamman and
Arnold Rich first described pulmonary fibrosis. They reported cases which presented with subacute respiratory
failure which lead to death. They performed autopsy and found that these patients had interstitial fibrosis. It was
the first pathological description of interstitial fibrosis. Thus, for the next few decades ILD was known as
‘Hamman-Rich syndrome’. It was probably the disease which is identified as acute interstitial pneumonia today.
However, doubts arose when clinicians identified a more chronic form of fibrosis separate from the disease
described by Hamman and Rich. Cryptogenic fibrosing alveolitis was the term coined to describe the disease
better. It is synonymous with IPF. In 1969, Liebow and Carrington differentiated interstitial lung diseases into five
types usual interstitial pneumonia (UIP), desquamative interstitial pneumonia (DIP), bronchiolitis obliterans
interstitial pneumonia (BIP), lymphoid interstitial pneumonia (LIP), and giant-cell interstitial pneumonia (GIP). In
1997, Liebow’s classification was modified with the addition of Respiratory bronchiolitis associated interstitial
lung disease (RB-ILD) and Non specific interstitial pneumonia (NSIP).
The pattern, course and prognosis of ILD varies in different countries. In order to provide better care to the ILD
patient we should have our own data. Therefore, need of ILD registry in India was felt.
Classification of ILD
Since there were various classifications and no standardization, ATS/ERS, in attempt to standardize the approach
towards ILD, issued guidelines in 2002. ATS/ERS classified ILD under the broader group of diffuse parenchymal
lung disease (DPLD). DPLD was classified in to DPLD of known cause, idiopathic interstitial pneumonia (IIP),
granulomatous DPLD and other forms of DPLD such as LAM, histiocytosis etc. IIP was further sub-grouped as
idiopathic pulmonary fibrosis (IPF), non-specific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia
(COP), acute interstitial pneumonia (AIP), respiratory bronchiolitis–associated interstitial lung disease (RB-ILD),
desquamative interstitial pneumonia (DIP), and lymphocytic interstitial pneumonia (LIP).
Interstitial Lung Disease Registry, India, Protocol
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American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the
Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 2002;165: 277–304.
Some characteristics of these ILD are as follows:
DPLD of known causes: These ILD are caused by some etiologic factors such as collagen vascular disease,
hypersensitivity pneumonitis, drugs etc
a) Collagen vascular disease associated ILD
 Milder than idiopathic
 Associated features of collagen vascular disease
 CT findings - UIP or NSIP pattern
b) Hypersensitivity pneumonitis?
Exposure to fungus like thermophilic actinomycetes and aspergillus is responsible for hypersensitivity
pneumonitis. Exposure to damp walls and birds like pigeons, parrot, ducks can lead to hypersensitivity
pneumonitis.
Idiopathic interstitial pneumonia
a. Idiopathic pulmonary fibrosis (IPF)
 Patients are >50yrs of age, disease starts insidiously and cough and dyspnoea re main symptoms.
 Bibasilar Velcro crepts and restrictive defect in spirometry are found. HRCT and biopsy are characteristics.
 Biopsy can be avoided when diagnosis is certain in HRCT.
 Usual interstitial pneumonia (UIP) pattern of lung involvement
 CT findings – Basal, subpleural, peripheral, reticular, traction bronchiectasis, focal ground glass ( area of
reticulation > ground glass)
Familial IPF
When more than one family member is diagnosed as having IPF it is called familial IPF. These patients have less
honeycombing and less lower lobe predominance.
ATS/ERS criterion for diagnosis of IPF
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HRCT pattern of IPF: OFFICIAL DOCUMENT –ATS-ERS-JRS-ALAT (Raghu et al AJRCCM, March 15
2011)
UIP pattern (All four
features)




Subpleural, basal
predominance
Reticulrar
abnormality
Honeycombing
with or without
traction
bronchiectasis
Absence of
features listed as
inconsistent with
UIP pattern (See
third column)
Possible UIP
pattern (All three
features)



Subpleural,
basal
predominance
Reticular
abnormality
Absence of
features listed
as inconsistent
with UIP
pattern (See
third column)
Inconsistent with UIP (Any of the seven features)







Upper or mid-lung predominance
Peribronchovascular predominance
Extensive ground glass abnormality (extent >
reticular abnormality)
Profuse micronodules (bilateral, predominantly
upper lobes)
Discrete cysts (multiple, bilateral, away from
areas of honeycombing)
Diffuse mosaic attenuation/ air-trapping
(bilateral, in three or more lobes)
Consolidation in bronchopulmonary
segment(s)/ lobe (s)
IIP other than IPF
a) Non specific interstitial pneumonia (NSIP)
 Prognosis is better than UIP
 CT findings – peripheral, subpleural, basal, ground glass attenuation, consolidation
 Associated conditions – collagen vascular disease, hypersensitivity pneumonitis, drug induced
pneumonitis and HIV.
b) Acute interstitial pneumonia (AIP)
 Rapidly progressive disease
 Mean age of presentation – 50 yr
 Patient may have prodromal viral illness
 CT findings – progressive diffuse consolidation, ground glass, lobular sparing.
c) Respiratory bronchiolitis induced interstitial lung disease (RB-ILD)
 Associated with cigarette smoking
 Intraluminal pigmented macrophages
 CT findings – diffuse, ground glass, centrilobular nodules, bronchial wall thickening
d) Cryptogenic organising pneumonia (COP)
 More common in non smokers
 Response to corticosteroids
 Relapse common after stopping steroids
 CT findings – patchy bilateral consolidation, nodules
e) Desquamative interstitial pneumonia (DIP)
 Affects smokers
 Mean age – 45y
 CT findings – lower zones, peripheral, ground glass, reticular lines
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f) Lymphocytic interstitial pneumonia (LIP)
 Associated with Non-Hodgkin’s lymphoma & Sjogren’s syndrome
 CT findings – diffuse centrilobular nodules, ground glass attenuation, septal and bronchovascular
thickening, thin walled cysts
Granulomatous diseases
Sarcoidosis
 Other system involvement
 Staging of sarcoidosis –
 Stage 0: Normal chest radiograph
 Stage 1: Hilar & mediastinal lymph node enlargement
 Stage 2: Lymphadenopathy & parenchymal disease
 Stage 3: Only parenchymal disease
 Stage 4: Pulmonary fibrosis
 CT findings – hilar lymphadenopathy, peribronchovascular nodules, parenchymal fibrosis
Miscellaneous
a) Lymphangioleiomyomatosis
 Premenopausal women
 Associated with –
 Multiple, bilateral cysts
 Recurrent pneumothorax
 Chylothorax
 Renal angioleiyomyomata
b) Histiocytosis X
 Associated with cigarette smoking and farming
 CT findings – upper lobe & middle lobe predominance, interstitial thickening, nodules and cysts
C) Pulmonary alveolar proteinosis
 Mean age – 20-40y
 CT findings – Mid & lower lobe involvement, crazy paving pattern
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ILD- India Registry: Indian Chest Society (Proforma Version 4)
Center code
Date: …………………..…
Email ……….…………………………
Name of the Consultant ……………………….
Mobile no. ……………..……………..
Address for correspondence …………………….…………………….………………………………….
…………………….…………………….………………………………….….….….….….….….….….….
Particulars of the patients ( for identification of the patient, not to be displayed)
Name of the patient
Code number
Date of the birth
Age and sex
Mobile number
Email
Address
Patient code number :
Inclusion: Patients presenting with following
1. Respiratory symptoms such as shortness of breath and cough
2. Bilateral abnormalities in X ray/HRCT scan thorax
3. Restrictive defect in Spirometry
Exclusion: Any Infectious or Malignant diseases
Yes
No
Yes
Yes
No
No
Proforma questionnaire based on patient’s history
1. Symptoms of patient
Symptom
Since when
Any comment
a) _______________________________________________________________________________
b) _______________________________________________________________________________
c) _______________________________________________________________________________
d) _______________________________________________________________________________
e) _______________________________________________________________________________
f) _______________________________________________________________________________
g) _______________________________________________________________________________
h) _______________________________________________________________________________
2. Has the patient noticed any of the following? (put  in respective column)
Yes No
Yes
No
a) Weight loss
g) Bruising skin
b) Difficulty in swallowing
h) Hand ulcers
c) Dry eyes or dry mouth
i) Mouth ulcers
d) Rash or changes in skin
j) Chest pain
e) Oedema on legs
k) Joint pain
f) Blood in urine
l) Symptoms of Gastro-oesophagial reflux (GERD) : (If yes put )
i. Indigestion __________ ii. heartburn __________ iii. acid-sour taste ____ iv. Belching __
v. bloating sensation ___ vi. cough after meals ___ vii. cough at night times/sleeping ___
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3. Has any doctor ever told him (past or known medical history) (put  in respective column)
Never
Before
ILD
Treatment
a) Tuberculosis
b) Extra pulmonary
Tuberculosis
c) Hypertension
d) Diabetes
e) Coronary heart
disease
f) Heart failure
g) Thyroid disease
h) Stroke (CVA)
i) Seizure
j)
Hepatitis A/ B/ C
k) Hapatitis,
other/unknown
l) Kidney disease
m) Anemia
n) Eye inflammation
o) Parasites (worms)
p) Pneumothorax
q) Pleural effusion
r) Pneumonia
s) Asthma
t) Bronchitis
u) Sinus disease
v) Pulmonary
hypertension
Interstitial Lung Disease Registry, India, Protocol
After ILD
treatment
Never
Before
ILD
treatment
w) Pulmonary
embolism
x) Sleep apnoea
y) Lung Cancer
z) GERD
aa) Hiatal hernia
bb) Bleeding disorder
cc) Rheumatologic
disease /collagen
vascular disease
dd) Vasculitis
ee) Raynaud’s
phenomenon
ff) Rheumatoid
arthritis,
gg) lupus
hh) scleroderma
ii) mixed connective
tissue disease
jj) Sjogren’s
Syndrome
kk) Wegener’s,
ll) Polymyositis or
dermatomyositis
mm) Bechet’s
disease
nn) Ankylosing
spondylitis.
oo) overlapping
pp) unspecified/
unclear
Others i)
ii)
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After ILD treatm
Domestic environmental factors
4. Conditions of his/her house
Yes
No
a) Dusts (visible) ----------------------------------------------------------------Yes
No
b) Molds(visible) ----------------------------------------------------------------Yes
No
c) Air conditioner --------------------------------------------------------------No
Yes
d) Cooler --------------------------------------------------------------------------No
Yes
e) Birds in home(caged) (Include pigeons, parrot, hen, crow, modi,
No
f) Any changes in house/housing conditions in recent past ---------Yes
g) IF YES to changes in housing conditions, how many days,
months or years before the cough and/or breathing /chest problems ___________________
5. Does the current or past home have/had following
a) Open cooking on fire wood or cow dung -----------------------------b) Cooking on kerosene stove -----------------------------------------------c) Cooking on coal -------------------------------------------------------------d) Cooking with LPG gas -----------------------------------------------------
Yes
Yes
Yes
Yes
No
No
No
No
6. Previous residential locations (Please list all locations lived for atleast 6 months.)
a) Urban (District headquarter or higher) ---------------------------Yes
No
b) Sub urban (Tehsil HQ) ------------------------------------------------------- Yes
No
c) Rural-village (Panchayat HQ or lower) ---------------------------------Yes
No
Others ______________________________________________________________________________________
7. Work environmental factors :
Working outside house
No
Yes
8. Occupational history: Please include all occupations worked : (include dust, metal, paint, fine
particles, etc) List any exposures that you feel might be related to cough/breathing /chest problems
and /or not listed above? (see appendix A)
Occupation
Years worked
Exposures
_________________________
______________________
___________________
_________________________
______________________
___________________
_________________________
______________________
___________________
_________________________
______________________
___________________
_________________________
______________________
___________________
_________________________
______________________
___________________
9. Exposed to visibly smoky or dusty air? Yes
No
10. Use of protective masks while working
No
Yes
10. Ever taken substance of abuse such as cannabis, opium or smoked ganza, charas etc?
11. Inhaled tobacco use? (If yes put ) Never
current
ever
Tobacco use:
Starting age
Bidi
Cigarette
Hookah/ Chillum
Chewing tobacco
Alcohol
Yes
Ganza, charas
Stopping age
Quantity/Day
Interstitial Lung Disease Registry, India, Protocol
No
Page 9 of 35
13. a) Medication history: Please include all medicines patient has taken especially those given in Appendix D
Name of medicine patient is
Starting
Stopping
Name of medicines patient
Starting
Stopping
taking PRESENTLY
date
date
has taken in the PAST
date
date
a.
l.
b.
m.
c.
n.
d.
o.
e.
p.
f.
q.
g.
r.
h.
s.
i.
t.
j.
u.
k.
v.
13. b) How many courses of antibiotics were taken in last one year? _______________
14. Family medical history (biological): grandparents, parents, brothers, sisters, aunts, uncles, first cousins, or
children have any of the following lung disease?
Yes
No
a) Asthma --------------------------------------------------------------------------------Yes
No
b) Chronic Obstructive Pulmonary disease (COPD) -------------------------------Yes
No
c) Sarcoidosis ----------------------------------------------------------------------------No
Yes
d) Bronchiectasis ------------------------------------------------------------------------No
Yes
e) Pulmonary fibrosis (ILD/IPF) -----------------------------------------------------Yes
No
f) Hypersensitivity pneumonitis -----------------------------------------------------
Yes
No
g) Rheumatologic disease /collagen vascular disease ( i.rheumatoid arthritis, ii. lupus, iii. scleroderma, iv. mixed
connective tissue disease, v. Sjogren’s Syndrome, vi. Wegener’s, vii. Polymyositis or dermatomyositis ,
viii.Bechet’s disease, ix.Ankylosing spondylitis.) x .overlapping xi. unspecified/unclear____
15. Physical examination:
Face/ Head _______ Eye/ ENT ____________ Skin __________________ Joints __________
Clubbing __________ Bibasillar crackles _____ Wheeze _______________ P2 heart) _______
Pallor _____________ Icterus ________________ Lymphademopathy _____ Pulse ___________
BP _______________ JVP ___________________ Cynosis _______________ Height __________
Weight ____________ Others __________________________________________________________
16. Investigations (Pulmonary evaluation):
A. Spirometry, lung volumes and diffusing capacity
Predicted
Actual
Post
FEV1
FVC
PEFR
Total lung capacity
RV
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DLCO (corrected for hb)
KCO
B. ABG (while breathing air at sea level ) pH _____ pO2 _____ pCO2 _____ HCO3 _____
C. 6 minute walk test: Test performed while breathing air
Yes
No
Test performed with supplemental Oxygen
If Yes,……….l/min
Yes
No
Distance covered in 6 minutes _____ baseline SpO2 _____ SpO2 immediately after walk …….
Reason for stopping before 6 minutes : breathless …….Fatigue….leg /joint pain ………..
Borg scale dyspnea : Rest ……… At the end of testing ……..
D. Fiberoptic Bronchoscopy (When relevant)
date_________
Yes
No
Fiberoptic Bronchoscopy Findings/diagnosis
i)Consistent with: alveolar hemorrhage……, proteinosis…, purulent secretions/infection…
ii) Smear for acid fast bacilli, cultures for M Tb /Myc species _____________
iii) Transbronchial biopsy: specific diagnosis - granuloma ____; malignancy _____infection
iv) Consistent with: sarcoidosis …..Hyperensitivity pneumonitis …, lymphangiectatic carcinoma….
E. Open /surgical /thoracoscopic lung biopsy: (If performed)
Yes
No date _________
i)Right lung (upper lobe/middle lobe/lower lobe) __________Left Lung(upper lobe /lingula/lower lobe
ii) Pathology (surgical lung biopsy)
Date____________
Yes
No
UIP Consistent :
definite
probable
possible
definitely not
Appendix B (Per OFFICIAL DOCUMENT –ATS-ERS-JRS-ALAT (Raghu et al AJRCCM, March 15 2011)
F. Chest Xray Recent: bilateral shadows present
Chest Xray, old bilateral shadows present
Yes
No
Yes
No
date _________
date _________
G. HRCT : 1mm cuts Yes
Expiratory views Yes
No
No,
No ; Prone & supine views Yes
Report: Date of HRCT :
___________________________________________________________________________________________
___________________________________________________________________________________________
___________________________________________________________________________________________
UIP:
Yes
No
High resolution computed tomography criteria for UIP pattern in Appendix C.
H. Sinus CT (Optional) ___ SINUSITIS Yes
No
17. Laboratory tests (relevant):
a) CBC: Hb___ Platelets___ MCV ___ DLC: N___ L___ E___ M___ B___
b) B Sugar F ___ PP ___
c) Renal : Urea __________ Creatinine __________
d) Liver function tests: Bilirubin _____ SGOT_____ SGPT ___ Hepatitis Bs __Ab__, Hepatitis C__ Ab__
e) Urinalysis: Protein _____ Sugar _____ Cast _____ Pus cells _____
f) Sputum: AFB_____ Fungus _____
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g) Collagen profile:
ANA + -, RF + - , dsDNA + - , Sm + - ,
RNP + - ,
Scl 70 + - , SSA + -,
SSB + - , CPK + - , Anti Jo-1 + - , ANCA + - ACE _____ EBV titer + h) HIV + i) Lymphocyte transformation testing (metals) ________________________________________
j) Mantoux test (PPD) Optional:
k) Echocardiogram (Optional): Date
EF __________________ Wall asymmetry _____________________ Estimated systolic PA pressure
____________________ RV dilation/thickness ________________
18. Consultant’s impression /opinion __________________________________________________________
____________________________________________________________________________________________
____________________________________________________________________________________________
A) Suspected ILD diagnosis :
Yes
No
a. Idiopathic Interstitial Pneumonias (IIP)
i. NSIP
ii.COP
iii.LIP
b. IPF
c. Pulmonary fibrosis of unknown cause other than IIP
d. Occupational ILD
e. Granulomatous diseases eg. Sarcoidosis
f. Hypersensitivity pneumonitis
g. ILD secondary to collagen vascular disease
h. familial ILD
i. Other rare ILD given in appendix D)
B) Suspect active infection (Active TB or pneumonia) ____________________________________________
C) Other coexisting lung disease ______________________________________________________________
D) Comorbidities ___________________________________________________________________________
19. Follow up of the patient:
Date
A. Symptoms:
Improved
Same
Deteriorated
B. New symptoms /finding/diagnosis
____________________________________________________________________________________
____________________________________________________________________________________
C. Investigations:
FEV1 _____ FVC ______
____________________________________________________________________________________
D. Treatment prescribed
____________________________________________________________________________________
____________________________________________________________________________________
Date
A. Symptoms:
Improved
Same
Deteriorated
B. New symptoms /finding/diagnosis
____________________________________________________________________________________
____________________________________________________________________________________
C. Investigations:
FEV1 _____ FVC ______
____________________________________________________________________________________
D. Treatment prescribed
____________________________________________________________________________________
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Appendix A
Occupations with important exposures
a)
Farm work : Name the crop eg. Wheat, sugar cane etc
b)
Mechanic: Name the job eg. automobile mechanic, electrician etc
c)
Carpenter: Name of the wood
d)
Painter
e)
Welder
f)
Laboratory worker: Name the job eg animal lab, chemistry lab etc
g)
Stone work: name the job eg stone mason, stone drilling/boring/ chisselling, quartz powder handling
h)
Pipe fitter (plumber)
i)
Mine : name the product eg copper mine, marble mine etc
j)
Foundry: name the product eg. steel product manufacturing, metal ornament making etc
k)
Factory: name the product eg soap factory, cotton factory, paper mill, plastic factory etc
l)
Bakery: name the product eg bread, biscuit, cake etc
m)
Exposure at farm: Birds, Feathers, Pesticides, Fertiliser
n)
Metal exposure: Copper, cobalt, tin , iron, aluminum etc
o)
Exposure to dust: Talc, pottery, silica, coal asbestos, cement, ceramic tiles etc.
p)
Miscellaneous: oily nose drops, paint, detergent
Appendix B
HISTOPATHOLOGICAL CRITERIA FOR UIP PATTERN (Per OFFICIAL DOCUMENT –ATS-ERS-JRS-ALAT (Raghu et al
AJRCCM, March 15 2011)
UIP Pattern (All Four Criteria)
1.
Evidence of marked fibrosis/ architectural distortion, 6 honeycombing in a predominantly subpleural/
paraseptal distribution
2.
Presence of patchy involvement of lung parenchyma by fibrosis
3.
Presence of fibroblast foci
4.
Absence of features against a diagnosis of UIP suggesting an alternate diagnosis (see D)
Probable UIP Pattern
1.
Evidence of marked fibrosis / architectural distortion, 6 honeycombing
2.
Absence of either patchy involvement or fibroblastic foci, but not both
3.
Absence of features against a diagnosis of UIP suggesting an alternate diagnosis (see D)
4.
OR
Honeycomb changes only
Possible UIP Pattern (All Three Criteria)
1.
Patchy or diffuse involvement of lung parenchyma by fibrosis, with or without interstitial inflammation
2.
Absence of other criteria for UIP (see UIP PATTERN column A)
3.
Absence of features against a diagnosis of UIP suggesting an alternate diagnosis (see fourth column)
Not UIP Pattern (Any of the Six Criteria)
1.
Hyaline membranes*
2.
Organizing pneumonia*†
3.
Granulomas†
4.
Marked interstitial inflammatory cell infiltrate away from honeycombing
5.
Predominant airway centered changes
6.
Other features suggestive of an alternate diagnosis
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Definition of abbreviations: HRCT 5 high-resolution computed tomography; UIP 5 usual interstitial pneumonia.
* Can be associated with acute exacerbation of idiopathic pulmonary fibrosis.
† An isolated or occasional granuloma and/or a mild component of organizing pneumonia pattern may rarely be
coexisting in lung biopsies with an otherwise UIP pattern.
‡ This scenario usually represents end-stage fibrotic lung disease where honeycombed segments have been
sampled but where a UIP pattern might be present in other areas. Such areas are usually represented by overt
honeycombing on HRCT and can be avoided by pre-operative targeting of biopsy sites away from these areas
using HRCT.
American Thoracic Society Documents 795
Appendix C
HIGH-RESOLUTION COMPUTED TOMOGRAPHY CRITERIA FOR UIP PATTERN (Per OFFICIAL DOCUMENT –ATS-ERSJRS-ALAT (Raghu et al AJRCCM, March 15 2011)
A. UIP Pattern (All Four Features)
1.
Subpleural, basal predominance
2.
Reticular abnormality
3.
Honeycombing with or without traction bronchiectasis
4.
Absence of features listed as inconsistent with UIP pattern (see C)
B. Possible UIP Pattern (All Three Features)
1.
Subpleural, basal predominance
2.
Reticular abnormality
3.
Absence of features listed as inconsistent with UIP pattern (see C)
C. Inconsistent with UIP Pattern (Any of the Seven Features)
1.
Upper or mid-lung predominance
2.
Peribronchovascular predominance
3.
Extensive ground glass abnormality (extent .reticular abnormality)
4.
Profuse micronodules (bilateral, predominantly upper lobes)
5.
Discrete cysts (multiple, bilateral, away from areas of honeycombing)
6.
Diffuse mosaic attenuation/air-trapping (bilateral, in three or more lobes)
7.
Consolidation in bronchopulmonary segment(s)/lobe(s)
Appendix D
RARE ILD :

Amyloid

Lymphangioleiomyomatosus (LAM)

Eosinophilic granuloma/Pulmonary Langerhans cell granulomatosis

Pulmonary alveolar proteinosis (PAP)

Veno-occlusive disease

Hemosiderosis

Pulmonary capilaritis

Lipoid pneumonia

Eosinophilic pneumonia

Sarcoidosis
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
Lymphangitic carcinomatosis

Bronchiolitis obliterans organizing pneumonia (BOOP)

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu)

Pulmonary lymphoma

Hermansky Pudlak Syndrome (Often with Puerto Rican ancestry)

Human Immunodeficiency Virus associated LIP

Wegener’s granulomatosis

known inherited conditions( e.g Neurofibromatosis;metabolic storage diseases;Hermansky-Pudlak
syndrome
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Appendix D
i) Antibiotics/infection treatment:
a) Penicillin
b) Isoniazid
c) Nitrofurantoin
d) Minocycline
e) Sulfonamide (Trimethoprim +
Sulfamethaxozole)
f) Cephalosporin
g) Macrlides
ii) Anti-inflammatory /antifibrotic medication:
a) Azathiaprine(Azoran) Colchicin
b) Cyclophosphamide
c) Gold salt
d) Interferon (any)
e) Methotrexate
f) Penicillamine
g) Prednisolone
h) Pirfenidone
i) Mycophenolate
iii) Newer anti inflammatory
a) Etanercept,
b) Infliximab,
c) Interleukins ,
d) Antibodies against TNFalpha, etc)
iv) Medications for pulmonary hypertension :
a) Sildenafil,
b) Endothelin receptor antagonists,
c) Prostacyclin analogues.
d) Calcium channel blockers
v) Cardiovascular medication:
a) Amiodaron(Cordarone)
b) Captopril
c) Hydralazine
d) Hydrochlorthizide
e) Procainamide
f) Sotolol
vi) Cancer therapy
a) Busulfan
b) Bleomycin
c) Cyclophosphamide
d) Etoposide
e)
f)
g)
h)
i)
j)
k)
l)
m)
n)
GMCSF
Mitomycin
Nilutamide
Chlorambucil
Nitrosoureas –
Radiation
Vinblastine
Monoclonal antibodies/receptor blockades
Antihormonal
Other
vii) Gastrointestinal medications:
a) Azulfidine
b) Sulfasalzine
c) Medications for stomach
d) Acid peptic disease (ulcers, GERD)
viii) Neurological medications:
a) Bromocriptine
b) Carbemazepine (Tegretol)
c) L tryptophan
d) Phenytoin (Dilantin).
ix) Miscellaneous therapies:
a) Fenfluramine/ dexfenfluramine
b) Leukotriene inhibitor
c) Propylthiouracil
d) Bladder BCG
e) Inhalers : inhaled corticosteroids inhaled
beta agoists /bronchodilators
f) Alternative medicines:
Ayurvedic/homeopathic/ unknown/ Yoga
x) Supplemental Oxygen use
a) None
b) Intermittent
c) During sleep
d) During exercise
e) Number of hours _______ (Max 24 hrs)
xi) Other medications: new/changes in
medications prior to onset of respiratory
symptoms ____________________________
___________________________________
___________________________________
HOW TO FILL PROFORMA VERSION 4
ILD- India Registry: Indian Chest Society (Proforma Version 4)
Center code
Date: …………………..…
Email ……….…………………………
Name of the Consultant ……………………….
Mobile no. ……………..……………..
Address for correspondence …………………….…………………….………………………………….
…………………….…………………….………………………………….….….….….….….….….….….
Particulars of the patients ( for identification of the patient, not to be displayed)
Name of the patient
Code number
Date of the birth
Age and sex
Mobile number
Email
Address
This is the information about the patient and it will not be disclosed.. Tthe data will be stored in the website
and will be used by a computer program to stop enrolement of a single patient two times from the same center
or from other center. The code number of the patient will be given by the recruiting center by putting
numerical number after center code number. If center code is AC then patient number may be AC 1, AC2,
AC3 …
Patient code no.
Inclusion: Patients presenting with following
1. Respiratory symptoms such as shortness of breath and cough
2. Bilateral abnormalities in X ray/HRCT scan thorax
3. Restrictive defect in Spirometry
Exclusion: Any Infectious or Malignant diseases
Yes
No
Yes
Yes
No
No
On basis of Inclusion and exclusion criteria a patient suspected to have ILD can be identified and thereafter
workup of the patient can be done as follows:
Proforma questionnaire based on patient’s history
1. Symptoms of patient
Symptom
Duration
Any comment
a) Cough
2007
b) Dyspnoea
2008
Grade 2
c) ______________________________________________________________________________
d) ______________________________________________________________________________
e) ______________________________________________________________________________
f) _______________________________________________________________________________
g) ______________________________________________________________________________
h) ______________________________________________________________________________
Please put all the symptoms (related or unrelated) in chronological order. Instead of duration it is better to
write year of onset.
a. Cough: Frequency ? (Do not include clearing of throat.)
a) None (at all) _____________ b) Rarely
_____________________________________________
c) Everyday ________________ d) Severe attacks that interfere with activity _________________
2. How long has s/he been coughing? Days _____ Months _____ Years _____
3. The cough produces: (Check all that apply.)
a. No expectoration _____ b. Expectoration _________ i. Mucoid _________
ii. Yellow-green _________ iii Green _________ iv. Blood tinged ______ v. Blood _____
b. Shortness of breath: Check the single number that describe the point at which S/he becomes
short of breath:
0. not troubled with breathlessness except with strenuous exercise
___________________
1. short of breath when hurrying on the level or walking up a slight hill. ___________________
2. walks slower than people of her/his age on the level because of breathlessness
or S/he has to stop from breath when walking on normal pace on the level.
_____________
3. stops for breath after walking about 100 yards or after a few minutes on the level. __________
4. too breathless to leave the house or breathless on dressing or undressing. _________________
5. When shortness of breath began? _______years_______ Months______
2. Has the patient noticed any of the following? (put  in respective column)
Yes No
Yes
No
a) Weight loss
g) Bruising skin
b) Difficulty in swallowing
h) Hand ulcers
c) Dry eyes or dry mouth
i) Mouth ulcers
d) Rash or changes in skin
j) Chest pain
e) Oedema on legs
k) Joint pain
f) Blood in urine
l) Symptoms of Gastro-oesophagial reflux (GERD) : (If yes put )
i. Indigestion __________ ii. heartburn __________ iii. acid-sour taste ____ iv. Belching __
v. bloating sensation ___ vi. cough after meals ___ vii. cough at night times/sleeping ___
Following symptoms may indicate presence of below mentioned causes or results of ILD
a)Weight loss –Almost all symptomatic patients with ILD specially in Rheumatoid arthritis, SLE ,
Wegener’s granulomatosis, Tuberculosis, Cryptogenic organising pneumonia, Pulmonary Langerhans cell
histiocytosis, Malignancy, Allergic granulomatosis of Churg-Strauss.
b)Difficulty in swallowing- Esophageal Dysmotility in Systemic Sclerosis
c) Dry eyes or dry mouth- Sjogren’s syndrome, Sarcoidosis, Systemic Sclerosis
d) Rash or changes in skin- Connective tissue diseases like SLE, R A, Sarcoidosis, Wegener’s
granulomatosis,Raynaud’s phenomena, Behcet’s diasease; Maculopapular rash- drug induced, CTD,
amyloidosis, gaucher’s disease; Telangictasia: scleroderma; Cutaneous vasculitis; CTD
e) Oedema of legs- Congestive heart failure.
f) Blood in urine- Glomerulonephritis in Goodpasture’s syndrome, SLE, Wegener’s granulomatosis,
Sarcoidosis
g)Bruising skin –use of Steroid, vasculitis
h)Hand ulcers - Systemic sclerosis, Vasculitis
i) Mouth ulcers – SLE, Wegener’s granulomatosis , HIV, Behcet’s diasease
j) Chest pain –Pleuritis in Wegener’s granulomatosis ,SLE ,RA
k) Joint pain- Connective tissue disease like SLE ,RA , Ankylosing Spondylitis, Wegener’s granulomatosis,
Sjogren’s syndrome.
l) GERD – occurs in Systemic Sclerosis. Can be a cause of Aspiration pneumonia and chronic cough
3. Has any doctor ever told him (past or known medical history) (put  in respective column)
Never
a) Tuberculosis
b) Extra pulmonary
Tuberculosis
c) Hypertension
d) Diabetes
e) Coronary heart disease
f) Heart failure
g) Thyroid disease
h) Stroke (CVA)
i) Seizure
j)
Hepatitis A/ B/ C
k) Hepatitis,
other/unknown
l) Kidney disease
m) Anemia
n) Eye inflammation
o) Parasites (worms)
p) Pneumothorax
q) Pleural effusion
r) Pneumonia
s) Asthma
t) Bronchitis
u) Sinus disease
v) Pulmonary hypertension
Before
ILD
Treatment
After ILD
treatment
Never
w) Pulmonary
embolism
x) Sleep apnoea
y) Lung Cancer
z) GERD
aa) Hiatal hernia
bb) Bleeding disorder
cc) Rheumatologic
disease /collagen
vascular disease
dd) Vasculitis
ee) Raynaud’s
phenomenon
ff) Rheumatoid
arthritis,
gg) lupus
hh) scleroderma
ii) mixed connective
tissue disease
jj) Sjogren’s Syndrome
kk) Wegener’s,
ll) Polymyositis or
dermatomyositis
mm) Bechet’s disease
nn) Ankylosing
spondylitis.
oo) overlapping
pp) unspecified/
unclear
Others i)
ii)
Before
ILD
treatment
Various diseases mentioned here are associated with specific type of ILD or starts with complication of
therapy of ILD. Therefore please go through the list carefully and indicate the onset of the disease before
initiation of treatment of ILD or after it. Common associations are as follows:
a) Tuberculosis- associated with bronchocentric granulomatosis. Can cause lung fibrosis and restrictive
pattern in PFT
b) Extra pulmonary Tuberculosis
c) Hypertension – occurs in Systemic Sclerosis, neurofibromatosis, diffuse alveolar hemorrage
d) Diabetes-Patients more prone to get tuberculosis, more severe pneumonia and thus complications.
Conversely steroids may precipitate diabetes.
e) Coronary heart disease-RA, SLE
f) Heart failure –Infiltrative diseases like Sarcoidosis , Systemic Sclerosis , Ankylosing Spondylitis,
Symptoms and CT picture ( in lying down position ) may mimic ILD.
g) Thyroid disease- Autoimmune diseases. Infiltrative disease like Systemic Sclerosis may cause
hypothyroidism as well as ILD
h) Stroke(CVA) – Vasculitis. Prone for Aspiration pneumonia
i) Seizures_ - Tuberous Sclerosis, Neurofibromatosis, Gaucher’s disease. Prone for Aspiration pneumonia.
j)Hepatitis A/B/C –Chronic active hepatitis causes ILD
k) Hepatitis Other unknown
l)Kidney diseases-Amylodosis, Goodpasture’s syndrome, Wegener’s granulomatosis, Connective tissue
diseases,Sarcoidosis
m)Anaemia-Connective tissue diseases ,Gauchers Disease, Cytotoxic drugs like Cyclophosphamide used in
treatment of ILD
n) Eye inflammation - Sjogren’s syndrome, Wegener’s granulomatosis, Ankylosing Spondylitis,
sarcoidosis
o)Parasites –Eosinophilic Pneumonias
p)Pneumothorax- Systemic sclerosis, RA, Pulmonary Langerhans cell histiocytosis, Pulmonary
lymphangioleiomyomatosis.
q)Pleural effusion- SLE, RA, Tuberculosis, Pulmonary lymphangioleiomyomatosis.
r)Pneumonia- Acute interstitial pneumonia, ARDS.
s)Asthma- Eosinophilic pneumonia.
t)Bronchitis- Sjogren’s syndrome, inhalation of toxic fumes, organic dust.
u)Sinus disease- Wegener’s granulomatosis, Allergic granulomatosis of Churg-Strauss, Sarcoidosis.
v)Pulmonary Hypertension- SLE, RA, Systemic sclerosis, Sarcoidosis.
w)Pulmonary embolism- chronic PE causes pulmonary hypertension and presents as dyspnoea and
hypoxemia.
x)Sleep apnoea- causes hypoxemia and eventually pulmonary hypertension.
y)Lung cancer- Associated with Asbestosis, Inhalation of smoke of tobacco, polycyclic hydrocarbons, biomass
fuel
z)GERD- Systemic sclerosis, can cause aspiration pneumonia, common cause of chronic cough.
aa) Hiatal hernia-cause of GERD
bb) Bleeding disorder-Hermansky- Pudlak syndrome
cc) Rheumatologic diseases/ Collagen vascular disease - cause ILD
dd)Vasculitis- occurs in diaeases like wegener’s granulomatosis, allergic granulomatosis of Churg and
Strauss, SLE, RA, Sarcoidosis etc.
ee) Raynaud’s phenomena - Systemic sclerosis, SLE, RA, Dermatomyositis, polymyositis, IPF.
ff) Rheumatoid arthritis,
gg) lupus
hh) scleroderma
ii) mixed connective tissue disease
jj) Sjogren’s Syndrome
kk) Wegener’s,
ll) Polymyositis or dermatomyositis
mm) Bechet’s disease
nn) Ankylosing spondylitis.
oo) overlapping
pp) unspecified/ unclear
Others
Domestic environmental factors
4. Conditions of his/her house
Yes
No
a) Dusts (visible) ----------------------------------------------------------------Yes
No
b) Molds(visible) ----------------------------------------------------------------Yes
No
c) Air conditioner --------------------------------------------------------------No
Yes
d) Cooler --------------------------------------------------------------------------No
Yes
e) Birds in home(caged) (Include pigeons, parrot, hen, crow, modi,
No
f) Any changes in house/housing conditions in recent past ---------Yes
g) IF YES to changes in housing conditions, how many days,
months or years before the cough and/or breathing /chest problems ___________________
a) Dusts (visible) – organic dust :hypersensitivity pneumonitis like Bagassosis, Farmers lungs.
dust like silica causes ILD
b) Molds(visible) – hypersensitivity pneumonitis
c)Air conditioner- hypersensitivity pneumonitis ( Air conditioner Lung)
d )Cooler- hypersensitivity pneumonitis (Humidifier lung)
e) Birds- hypersensitivity pneumonitis(Birds fancier’s lung)
f)Changes in house/housing conditions may cause hypersensitivity pneumonitis due to molds
g) To classify them into acute, subacute and chronic hypersensitivity pneumonitis.
5. Does the current or past home have/had following
a) Open cooking on fire wood or cow dung -----------------------------b) Cooking on kerosene stove -----------------------------------------------c) Cooking on coal -------------------------------------------------------------d) Cooking with LPG gas -----------------------------------------------------
Yes
Yes
Yes
Yes
Inorganic
No
No
No
No
a) Open cooking on fire wood or cow dung-Fumes of acrolein and other aldehydes can cause mucosal
irritation and decrease in lung function. To look association with ILD
b) Acid fumes cause bronchitis.
c) Cooking on coal- inhalation of SO2,NO2, cause chronic bronchitis
d) Cooking with LPG gas- to know the frequency of LPG users
6. Previous residential locations (Please list all locations lived for atleast 6 months.)
a) Urban (District headquarter or higher) ---------------------------Yes
No
b) Sub urban (Tehsil HQ) ------------------------------------------------------Yes
No
c) Rural-village (Panchayat HQ or lower) ---------------------------------Yes
No
Others _____________________________________________________________________________
People living in urban areas are more exposed to vehicular pollution while rural patients are more exposes to
molds and many other organic dusts.
7. Work environmental factors :
Working outside house
No
Yes
8. Occupational history: Please include all occupations worked : (include dust, metal, paint, fine
particles, etc) List any exposures that you feel might be related to cough/breathing /chest
problems and /or not listed above? (see appendix A)
Occupation
Years worked
Exposures
_________________________
______________________
___________________
_________________________
______________________
___________________
_________________________
______________________
___________________
_________________________
______________________
___________________
_________________________
______________________
___________________
9. Exposed to visibly smoky or dusty air?
10. Use of protective masks while working
Yes
Yes
No
No
Work environmental factors: Any exposure to organic or inorganic dust should be mentioned. Duration of
exposure is important for ILD eg .Asbestosis develop after ten years of exposure and Coal Workers
Pneumoconiosis develops after 15 to 20 years of exposure. Stone workers are prone to develop silicosis while
pipe fitters may develop asbestosis. Hypersensitivity pneumonitis may develop in farmers and carpenters..
Mask may protect from inhaling dust
11. Ever taken substance of abuse such as cannabis, opium or smoked ganza, charas etc?
12. Inhaled tobacco use? (If yes put ) Never
current
ever
Tobacco use:
Starting age
Bidi
Cigarette
Hookah/ Chillum
Chewing tobacco
Alcohol
Yes
Ganza, charas
Stopping age
Quantity/Day
Opiates may cause lung fibrosis. Tobacco smoke may cause chronic bronchitis, fibrosis. 66% to 75% of the
IPF patients are smokers. Strong association of smoking is also present in PLCH, DIP, Goodpasture’s
syndrome, Respiratory bronchiolitis and pulmonary alveolar proteinosis.
No
13. a) Medication history: Please include all medicines patient has taken especially those given in Appendix D
Name of medicine patient
Starting
Stopping
Name of medicines
Starting
Stopping
is taking PRESENTLY
date
date
patient has taken in the
date
date
PAST
a.
l.
b.
m.
c.
n.
d.
o.
e.
p.
f.
q.
g.
r.
h.
s.
i.
t.
j.
u.
k.
v.
Medication history – Drugs causing ILD are:-Amiodarone, acyclovir, bleomycin, busulphan, methotrexate,
chlorambucil, melphalan, methysergide, nitrofurantoin, sulphonamides, aminoglycosides, opiates, hypnotics,
mitomycin C, gold, polymyxins. Drugs used in treatment of ILD can also predispose many conditions such as
diabetes and increased occurrence of infections.
13. b) How many courses of antibiotics were taken in last one year? _______________
14. Family medical history (biological): grandparents, parents, brothers, sisters, aunts, uncles, first cousins, or
children have any of the following lung disease?
Yes
No
a) Asthma --------------------------------------------------------------------------------Yes
No
b) Chronic Obstructive Pulmonary disease (COPD) -------------------------------Yes
No
c) Sarcoidosis ----------------------------------------------------------------------------No
Yes
d) Bronchiectasis ------------------------------------------------------------------------No
Yes
e) Pulmonary fibrosis (ILD/IPF) -----------------------------------------------------Yes
No
f) Hypersensitivity pneumonitis -----------------------------------------------------
Yes
No
g) Rheumatologic disease /collagen vascular disease ( i. rheumatoid arthritis, ii. lupus, iii. scleroderma, iv.
mixed connective tissue disease, v. Sjogren’s Syndrome, vi. Wegener’s, vii. Polymyositis or
dermatomyositis , viii. Bechet’s disease, ix. Ankylosing spondylitis x. Overlapping
xi. Unspecified/unclear_
Asthma is genetically transmitted. Familial inheritance also exists in Sarcoidosis, rheumatological diseases,
Tuberous sclerosis, neurofibromatosis, Niemann-Pick disease, Gaucher’s disease, Hermansky-Pudlak
syndrome. Familial lung fibrosis may present as different patterns of interstitial pneumonia. Moreover, more
than one family member can be affected if residing in close proximity to mines or other areas of dust exposure.
15. Physical examination:
Face/ Head _______ Eye/ ENT ____________ Skin __________________ Joints __________
Clubbing __________ Bibasillar crackles _____ Wheeze _______________ P2 heart) _______
Pallor _____________ Icterus ________________ Lymphademopathy _____ Pulse __________
BP _______________ JVP ___________________ Cynosis _______________ Height _________
Weight ____________ Others _________________________________________________________
Physical examinationFace & skin- Rashes, ulcers in SLE, RA, Systemic sclerosis, Sjogren’s syndrome, Behcet’s disease,
Sarcoidosis. Prolonged intake of steroids causes moon facies and easy bruising on skin.
Joints – Arthritis and arthralgia in connective tissue diseases, Wegener’s granulomatosis, Sjogren’s
syndrome.
Clubbing-IPF, Primary biliary cirrhosis, Inflammatory bowel diseases.
Bibasillar crackles- most common auscultatory finding in ILD. May also occur in CHF.
Pallor- Connective tissue diseases, Gaucher’s disease, result of cytotoxic drugs.
Icterus- Hepatitis(infective or drug induced), cirrhosis.
Lymphadenopathy- Tuberculosis, HIV, Sarcoidosis, lymphoma, lymphocyatic interstitial pneumonia
Salivary gland enlarged: sarcoidosis, lymphocyatic interstitial pneumonia
Hepatosplenomegaly: Sarcoidosis,CTD, amyloidosis, lymphocyatic interstitial pneumonia
JVP- Cor pulmonale, CHF
16. Investigations (Pulmonary evaluation):
A. Spirometry, lung volumes and diffusing capacity Whose predicted values are being used for Indian origin
? ..worth mentioning
Predicted
FEV1
FVC
PEFR
Total lung capacity
RV
DLCO (corrected for hb)
KCO
Actual
Post
B. ABG (while breathing air at sea level ) pH _____ pO2 _____ pCO2 _____ HCO3 _____
C. 6 minute walk test: Test performed while breathing air
Yes
No
Test performed with supplemental Oxygen
If
Yes
No
Yes,……….l/min
Distance covered in 6 minutes _____ baseline SpO2 _____ SpO2 immediately after walk …….
Reason for stopping before 6 minutes : breathless …….Fatigue….leg /joint pain ………..
Borg scale dyspnea : Rest ……… At the end of testing ……..
Spirometry would use Indian standard of predicted value. Diffusion test will measure DLco
corrected for hemoglobin value
D. Fiberoptic Bronchoscopy (When relevant)
date_________
Yes
No
Fiberoptic Bronchoscopy Findings/diagnosis
i)Consistent with: alveolar hemorrhage……, proteinosis…, purulent secretions/infection…
ii) Smear for acid fast bacilli, cultures for M Tb /Myc species _____________
iii) Transbronchial biopsy: specific diagnosis - granuloma ____; malignancy _____infection
iv) Consistent with: sarcoidosis .. Hypersensitivity pneumonitis …, lymphangiectatic carcinoma….
E. Open /surgical /thoracoscopic lung biopsy: (If performed)
Yes
No date _________
i)Right lung (upper lobe/middle lobe/lowerlobe) __________Left Lung(upper lobe /lingula/lower
lobe
ii) Pathology (surgical lung biopsy)
Date____________
Yes
No
UIP Consistent :
definite
probable
possible
definitely not
Per OFFICIAL DOCUMENT –ATS-ERS-JRS-ALAT (Raghu et al AJRCCM, March 15 2011)
F. Chest Xray Recent: bilateral shadows present
Chest Xray, old bilateral shadows present
Yes
No
Yes
No
date _________
date _________
G. HRCT : 1mm cuts Yes
No ;Prone & supine views Yes
No , Expiratory views Yes No
Report: Date of HRCT :
_______________________________________________________________________________________
________________________________________________________________________________________
UIP:
Yes
No
High resolution computed tomography criteria for UIP pattern in
Appendix C.
Yes
No
H. Sinus CT (Optional) ___ SINUSITIS
INVESTIGATIONS
Spirometry and lung volumes- most common presentation in ILD is restrictive pattern with decreased
TLC, FRC and RV. FEV1 and FVC are also decreased but FEV1/FVC is normal or increased. Obstructive
pattern may also be seen particularly in LAM and Tuberous sclerosis. It also has prognostic value in IPF and
NSIP.
Diffusion capacity- reduced in ILD.
ABG- may be normal or may show hypoxemia and respiratory alkalosis particularly during exercise as
revealed after 6MWT.
Fiberoptic Bronchoscopy- Bronchoalveolar lavage(BAL) findingsPulmonary alveolar proteinosis: BAL yields a cloudy, effluent that contains large amounts of periodic
acid-Schiff–positive lipoproteinaceous material, as evidenced by light microscopy.
5% or more CD1a-positive cells in the BAL fluid is highly specific for the diagnosis of pulmonary
Langerhan’s cell histiocytosis.
BAL finding diagnose Pulmonary hemorrhage, berylliosis, hypersensitivitypneumonitis, and some
pneumoconioses. BAL cell profiles are not diagnostic in idiopathic interstitial pneumonias.
Excess of neutrophils: the proportions of whichcorrespond to the extent of reticular change on HRCT
Excess eosinophils (more than 20%): eosinophilic lung disease
Lymphocytosis above 15%: NSIP, COP, hypersensitivity pneumonitis, sarcoidosis or other granulomatous
lung diseases
Surgical lung biopsy- may be needed in cases of IPF, Sarcoidosis, hypersensitivity pneumonitis, asbestosis,
lymphangitic carcinoma, PLCH.
CXRay – most common presentation is bibasilar reticular pattern. Nodular or mixed pattern are also seen.
HRCT – type of Scan is needed?
High resolution CT (HRCT) with 1.0 mm sections is required for diagnosis of DPLD. Both inspiratory+
expiratory and supine + prone films are required. If clinical features and chest radiograph suggest hilar
enlargement, then contrast is required to delineate mediastinal structures.
Sinus CT- may be useful in wegener’s granulomatosis, sarcoidosis.
17. Laboratory tests (relevant):
a) CBC: Hb___ Platelets___ MCV ___ DLC: N___ L___ E___ M___ B___
b) B Sugar F ___ PP ___
c) Renal : Urea __________ Creatinine __________
d) Liver function tests: Bilirubin _____ SGOT_____ SGPT ___ Hepatitis Bs __Ab__, Hepatitis C__
Ab__
e) Urinalysis: Protein _____ Sugar _____ Cast _____ Pus cells _____
f) Sputum: AFB_____ Fungus _____
g) Collagen profile:
ANA + -, RF + - , dsDNA + - , Sm + - ,
RNP + - ,
Scl 70 + - , SSA + -,
SSB + - , CPK + - , Anti Jo-1 + - , ANCA + - ACE _____ EBV titer + Collagen profile relevant in workup of ILD?
Test
Anti nuclear antibody
Anti double stranded DNA (ds-DNA)
Anti Smith (Sm)
RF
Anti Ribonucleprotein (RNP)
Cytoplasmic antinuclear antibody (c-ANCA)
Perinuclear antinuclear antibody (p-ANCA)
Angiotensin converting enzyme (ACE)
Anti topoisomerase
Anti SSA (Ro)
Anti SSB (La)
Disease
SLE
Non specific
Rheumatoid arthritis
Mixed connective tissue disorder
Wegner’s granulomatosis
Microscopic polyangiitis
Churg strauss
Sarcoidosis
Systemic sclerosis
SLE
Sjogren’s syndrome
h) HIV + i) Lymphocyte transformation testing (metals) ________________________________________
j) Mantoux test (PPD) Optional:
k) Echocardiogram (Optional): Date
EF __________________ Wall asymmetry _____________________ Estimated systolic PA pressure
____________________ RV dilation/thickness ________________
Besides CBC showing anemia or pancytopenia (Gaucher’s disease, marrow suppression by drugs),
RFT and urine examination may show nephritis or nephritic syndrome. LFT should be done for
hepatitis, cirrhosis. Peripheral eosinophilia occur in eosinophilic pneumonia, Churg Strauss
syndrome and drug reactions. Hypercalcemia occurs in sarcoidosis.
ANCA and Anti Basement membrane antibodies are useful in vasculitis. Scl70 in systemic sclerosis,
Anti Jo-1 antibodies in and raised CPK in polymyositis and dermatomyositis.
ECG may show enlargement of RA,RV.
Cardiac echocardiography relevant in work up of DPLD?
Cardiac echocardiography is relevant because –
 Cor pulmonale due to chronic hypoxia
 Concomitant heart disease
18. Consultant’s impression /opinion
_______________________________________________________________________________________
________________________________________________________________________________________
________________________________________________________________________________________
E) Suspected ILD diagnosis :
Yes
No
a. Idiopathic Interstitial Pneumonias (IIP)
i. NSIP
ii. COP
iii. LIP
b. IPF
c. Pulmonary fibrosis of unknown cause other than IIP
d. Occupational ILD
e. Granulomatous diseases eg. Sarcoidosis
f. Hypersensitivity pneumonitis
g. ILD secondary to collagen vascular disease
h. familial ILD
i. Other rare ILD given in appendix D)
F) Suspect active infection (Active TB or pneumonia)
____________________________________________
G) Other coexisting lung disease
______________________________________________________________
H) Comorbidities
___________________________________________________________________________
19. Follow up of the patient:
Date
A. Symptoms:
Improved
Same
Deteriorated
B. New symptoms /finding/diagnosis
____________________________________________________________________________________
____________________________________________________________________________________
C. Investigations:
FEV1 _____ FVC ______
____________________________________________________________________________________
D. Treatment prescribed
____________________________________________________________________________________
____________________________________________________________________________________
Date
A. Symptoms:
Improved
Same
Deteriorated
B. New symptoms /finding/diagnosis
____________________________________________________________________________________
____________________________________________________________________________________
C. Investigations:
FEV1 _____ FVC ______
____________________________________________________________________________________
D. Treatment prescribed
____________________________________________________________________________________
____________________________________________________________________________________
PATIENT INFORMATION AND CONSENT DOCUMENT
(Interstitial Lung Disease, India, Registry)
Version 1.0 dated 20th Feb 2012, English
ILD India Registry is an initiative of Indian Chest Society, aimed to collect epidemiological data, to
understand natural course of the disease, to identify associated risk factors for improving survival of ILD
patients.
Interstitial Lung Disease (ILD) is referred to a group of lung disorders characterized by pathological
involvement of lung interstitium (meshwork of lung tissue/ space around alveoli), most of which causes
progressive lung tissue scarring which is generally irreversible and may lead to Pulmonary Fibrosis.
Inflammation of Interstitium is usually a diffuse process that occurs in whole of the lungs and is not limited
to just one location. Hence, ILD is also known as Diffuse Parenchymal Lung Disease (DPLD).
Dyspnea on exertion and dry cough are the primary symptoms which is followed by shortness of breath
even during rest. Usually, Lung damage has already progressed to an irreversible state at the time patient
notices symptoms and seeks medical advice.
As far as the etiology is concerned, approximately two-thirds of the ILD cases are idiopathic (do not have a
known cause), while one-third have various endogenous or exogenous cause i.e. occupational factors,
infections, environmental factors, drugs and radiation. ILD can be classified according to the cause in
following manner:
ILD due to Known causes

Hypersensitivity pneumonitis (eg, farmer’s lung,
bird fancier’s disease)

Pneumoconioses (eg, asbestosis, silicosis, coal
worker’s pneumoconiosis)

Drug-induced ILDs (eg, chemotherapeutic agents,
amiodarone, nitrofurantoin)

Connective tissue disease–associated ILDs (eg,
Rheumatoid arthritis, polymyositis, scleroderma)
Smoking-related ILDs
Pulmonary Langerhans cell histiocytosis
Respiratory bronchiolitis–associated ILD
Desquamative interstitial pneumonia






Acute eosinophilic pneumonia
Radiation-induced ILDs
Aspiration pneumonia
Residual of Adult Respiratory Distress Syndrome (ARDS)
Toxic inhalation–induced ILDs (eg, cocaine, zinc
chloride [smoke bomb], ammonia)
Unknown causes
A. Idiopathic Interstitial pneumonia
a. Idiopathic pulmonary fibrosis
b. IIP other than IPF
1. RB ILD
2. Cryptogenic organizing pneumonia
3. Nonspecific interstitial pneumonia
4. Acute interstitial pneumonia
5. Lymphocytic interstitial pneumonia
6. Desqamative interstitial pneumonia
B. Granulomatous diseases such as Sarcoidosis
C. Miscellaneous:
a. Pulmonary lymphangioleiomyomatosis
b. Pulmonary alveolar proteinosis
c. Many other rare disorders
ILD India Registry, has following objectives




In order to understand this fatal disease, a network of most privileged clinical scientists in the field
of Interstitial Lung Disease will be established in India.
To evaluate pattern and natural course of the disease in India.
To identify associated causative factors and to suggest improved methods of prevention in high risk
group.
To establish more advanced diagnostic and treatment plans for Interstitial Lung Disease.
Methodology
The ILD India Registry, is an electronic database of medical history, clinical examination, laboratory and
radiological investigations of patients suffering from Interstitial Lung Disease. Clinicians volunteering to
participate in the registry will be evaluated for their bio-data, ILD patient load and relevant infrastructure
facilities. The selected investigators will be requested to fill the details of the prescribed proforma after
obtaining written informed consent from the patient. The project will be started after obtaining approval
from ethics committee.
Site investigators will send completed proforma along with relevant documents such as PFT report, X-Ray
chest, CT scan (preferably CD) and histopathology slides along with copy of biopsy report. The blinding
process will be done by putting patient code number in place of the name. National coordinator will be
responsible to manage all the data in electronic format. An independent expert panel will review data of
each patient to confirm the diagnosis. The expert panel will have two radiologists, two clinicians and two
histopathologists. Data of the patient along with confirmed diagnosis will be entered in the registry.
Diagnostic query of the panel will be sent to the investigator, in case the diagnosis is not verified.
Information of the evaluation report of the expert panel will be communicated to the submitting site
investigator. All original documents will be returned to the investigators.
The participating centers will also send reports of the patient coming on follow-up visits. Any unforeseen
event such as death of the patient will also be informed to the registry.
Expected Duration of Participation
Participating patient will be part of registry till survival or termination of the project.
Risks to the subject
ILD India Registry is an observational study and it will collect the relevant data. Therefore, no project
specific risk is associated while participating in this registry.
Benefit to the subject
No benefit is expected to the participating patient.
Compensation
Since ILD India Registry is an observational study with collection of data and thus does not involve any
project specific risk. Therefore, it will not provide any sort of compensation.
Financial Benefits & Reimbursements
Patient will not receive any sort of financial benefits or reimbursement for his participation in the
registry. All investigations and follow-up visits of the patient will be planned and scheduled by the site
investigator on the basis of clinical requirement as judged by him.
Confidentiality
Confidentiality of records identifying the Subject will be maintained. Every patient will be provided a
code number and source data will be with the investigator. Identity of the patient will not be revealed.
Participant’s Rights & Responsibilities
Participants have all rights to ask for any project related queries and participation is voluntary. A subject
can withdraw from the registry at any time. Refusal to participate will not involve any penalty or loss of
benefits to which the subject is otherwise entitled.
ILD India Registry National Coordinator Contact
Dr Virendra Singh (National Coordinator, ILD India Registry)
Professor of Medicine
Head, Division of Allergy and Pulmonary Medicine
SMS Medical College & Hospital, Jaipur
Ph. +91.141.2281010
M. +91.9414051212
E mail: [email protected]
CONSENT FORM
Subject’s Initials: _______________
Subject’s Name:_______________
Date of Birth / Age: _________________
(i)
Please do initial
in box (Subject)
I confirm that I have read and understood the information sheet for the
[
]
above project and have had the opportunity to ask questions.
(ii)
I understand that my participation in the registry is voluntary and that I
am free to withdraw at any time, without giving any reason, without my
medical care or legal rights being affected.
[
]
(iii)
I understand that the site investigator, others working on the
investigator’s behalf, the Ethics Committee and the regulatory
authorities will not need my permission to look at my health records
both in respect of the current project and any further research that may
be conducted in relation to it, even if I withdraw from the study. I agree
to this access. However, I understand that my identity will not be
revealed in any information released to third parties or published.
[
]
(iv)
I agree not to restrict the use of any data or results that arise from this
project provided such a use is only for scientific purpose(s)
[
]
(v)
I agree to take part in the ILD India Registry.
[
]
Signature of the Subject:___________________________
Date: _____/_____/______
Signatory’s Name: ______________________________________________________
Signature of Legally Acceptable Representative: __________________ Date: _____/_____/______
Name of LAR: __________________________________________________
Signature of the Investigator: ________________________
Date: _____/_____/______
Site Investigator’s Name: __________________________________________________
Signature of the Witness _____________________
Date:_____/_____/_______
Name of the Witness: ______________________________________________________
TERMS AND CONDITIONS OF THE PROJECT
Name : ILD India Registry
Purpose of the Registry: ILD India Registry is established to collect epidemiological data, to
understand natural course of the disease and to identify associated risk factors for improving
survival of ILD patients. The clinical spectrum, probable etiologic agents and factors affecting
natural course of the disease will be discovered. Therefore, in the long run, the ILD India Registry
will serve to understand ILD better and it is intended to lead to the development of better care of
ILD patients. These clinical data will be used to understand natural course of disease in view to
identify all associated causative factors and to suggest improved methods of prevention in high risk
group as well as to establish more advanced diagnostic and treatment plans for Interstitial Lung
Disease.
Ownership of the registry: The project has been initiated by Indian Chest Society. Dr Virendra Singh
has been designated national coordinator of the project.
Dr Virendra Singh (National Coordinator, ILD India Registry)
Professor of Medicine
Head, Division of Allergy and Pulmonary Medicine
SMS Medical College & Hospital, Jaipur
Ph. +91.141.2281010
M. +91.9414051212
E mail: [email protected]
Participants: Specialists caring patients of ILD can volunteer to become collaborating centers.
Method of collaboration
Collaborating specialists will include patients with various forms of Interstitial Lung Disease after written
informed consent. The patients will be asked the details of the questionnaire about their illness.
Observations of physical examination and details of diagnostic tests will be recorded in a structured manner
and stored in a database managed centrally by the registry.
Site investigators will send completed proforma along with copy of relevant documents such as PFT report,
X-Ray chest, CT scan (preferably CD) and histopathology slides along with copy of biopsy report. The
blinding process will be done by putting patient code number in place of the name. National coordinator will
be responsible to manage all the data in electronic format.
Review panel: An independent expert panel will review data of each patient to confirm the diagnosis. The
expert panel will have two radiologists, two clinicians, two histopathologists and statistics experts. Data of
patient with confirmed diagnosis will be entered in the registry. Diagnostic query of the panel will be sent to
the investigator, in case diagnosis is not verified. Information of the evaluation report of the expert panel
will be communicated to the submitting site investigator. All original documents will be returned to the
investigators.
Follow-up reports: The participating centers will also send reports of the patient coming on follow-up visits.
Any unforeseen event such as death of the patient will also be informed to the registry.
Rights and obligations of Principal Investigator
1) The ownership of site specific data will stay with the respective sites. However national coordinator will
play a coordinating role to pool all the data and will take responsibility for data management and data
analysis.
Publication rights: Individual sites may use the site specific data for presentation at the national and
international meetings and for publication in the medical journals. Pooled data will be published as
common paper with the authorship as follows:
The participating centers and members of expert panel will be included in the list of authors of the paper.
The sequence of authors will depend on number of evaluable subjects recruited by each site. The
contribution of expert panel will be equal to one patient when they will evaluate data of 5 patients. Any
member volunteering to write a paper will be given priority in the author’s sequence. The name of national
coordinator will be the last author.
2. The Site Investigator shall be responsible for protection of the resources of ILD India Registry to the best
of his knowledge and belief. Identity of the patient will not be disclosed.
3. The Site investigator is responsible for the accuracy and completeness of the data obtained from the
patient and stored in the ILD India Registry.
4. The Site Investigator will not include any patient before ethics committee approval which is the
responsibility of ILD India Registry. Site Investigator is responsible for ensuring that the patient receives
sufficient and competent advice, and for obtaining the patient’s informed consent. ILD India Registry
undertakes to provide the Site Investigators with specimen forms of all necessary documents (patient
information & consent form).
5. The Site investigator is entitled to terminate his collaboration in the ILD India Registry at any time.
However, he has to provide all relevant data collected before the date of termination to ILD India registry.
6. Since we do not have sufficient funding therefore site investigators are requested to mobilize resources
to fund their activity. All courier expenses or sample transportation facilities will be provided or born by
Indian chest Society.
For ILD India Registry
Signature
Date & Place
Name & Designation
For the Participant
Signature
Date & Place
Name & Designation