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Year Level 7 [Pulmonary Module]
INTERSTITIAL LUNG DISEASE
Hilario M. Tamondong Jr. M.D.
OUTLINE:
I. Objectives
II. Interactive Activity
III. Pneumoconioses
A. Mineral Dust
B. Headings
C. Bulleting & Indentation
D. Others
IV. Schedule & Deadlines
V. General Rules
I.
OBJECTIVES
To present the clinical manifestations, pathology and
radiologic features of patients with Environmental Lung.
Disease/s Idiopathic Interstitial Pneumonia
______________________________________________________
II. INTERACTIVE ACTIVITY
A. Clinical History
 General data
o 59 year old male
o Filipino
o Roman Catholic
o Retired Seaman
o Married
o Imus, Cavite City

Chief complaint
o Dyspnea of >4 months
 HPI
o 4 months PTA

recurrent exertional dyspnea (mildmoderate activities)

non-productive cough

(-) fever

(-) failure symptoms, orthopnea,
paroxysmal nocturnal dyspnea
o 2 months PTA

progressive dyspnea

whitish productive cough

intermittent undocumented fever

admitted in a local hosital

chest xray: unrecalled/ not available

chest ct scan:

Non-specific pneumonia;
T/C PTB

therapeutics

unrecalled IV antibiotics

anti TB medicines

AFB smear not done

Discharged on the 3rd day
o
1 week PTA

progressive dyspnea even at rest

persistence of whitish productive
cough

no fever

prompted consult  admission

Review of systems
Team 8 | Ann, Kriska, Francine, Greg, Jet, Kai, Archee
July 30, 2010




B.
C.
o (+) dyspnea at rest
o (+) whitish cough
o (+) easy fatigability
o (+) orthopnea
o (+) weight loss
o (-) hemoptysis
o (-) cyanosis
o (-) joint pains
o (-) skin rashes
Past health history
o Unremarkable
Family history
o HPN on father’s side
Social history
o 40 pack pear smoker
o heavy alcohol drinker
Occupational/ environmental history
o fisherman
o seaman, retired
Physical Examination

General
o Conscious, coherent, in respiratory
distress
o BP: 100/60
o HR: 120
o RR: 28
o T: 36C
o O2sat 87% Room Air
o Wt: 60 kg
o Ht: 157 cm
o BMI: 24

Head/ Neck
o anicteric sclerae, pink palpebral
conjunctivae, supple neck, (-) CLAD,
(-) NVE, (-) bruit

Cardiovascular
o adynamic precordium, tachycardic,
regular rhythm, PMI= AB at 5th ICS
LMCL, no murmur

Chest/ Lungs
o SCE, (+) supraclavicular retractions,
decreased breath sounds LLF,
increased vocal and tactile fremitus
LLF, (+) crackles on both lung fields,
no wheezes

Abdomen
o Flabby, NABS, soft, non-tender, no
organomegaly

Extremites
o Grossly normal extremities, no
clubbing, no edema, no bruises, no
cyanosis
Salient Features

59 year old male, retired seaman

chronic/ progressive dyspnea

easy fatigability at rest
Page 1of 18
INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010




D.
E.
F.
chronic non-productive -> productive cough
weight loss, orthopnea
40 pack year smoker
Hospital admission (2months PTA) due to
pneumonia

PE findings of respiratory distress; (+)
supraclavicular retractions, decreased breath
sounds LLF, increased vocal and tactile
fremitus LLF, (+) crackles on both lung, no
wheezes
Admitting Diagnosis

Primary diagnosis/ initial impression
o Chronic Obstructive Pulmonary
disease (COPD), Emphysema

Differential diagnoses
o pneumonia
o Pulmonary TB
o Lung cancer
o Bronchial Asthma
o Bronchiectasis
o Congestive heart failure
o Interstitial lung disease
Problems

Chronic progressive dyspnea

Easy fatigability

Chronic cough
1st Hospital Day

S> dyspnea at rest

Chronic whitish productive cough

Intermittent fever






Result
Hgb
209
Hct
0.63
WBC
9.7
Seg.
0.84
Lymph
0.16
PC
309
G.
Team #
127
Na
136
K
3.72
ABG
Result
pH
7.43
PO2
60.4
PCO2
31.2
HCO3
20.2
BE
-2.9
O2sat
92.2
O2
2 lpm

O> coherent, in mild respiratory distress
BP: 110/60
CR: 125
RR: 26
T: 36
C/L: SCE, supraclavicular retractions, crackles
BLF
Test
Crea
CXR:
patchy
disseminated
multilobar
consolidative alveolo-interstitial infiltrates,
extensive left.

ECG: Sinus tachycardia, nsstwc

Sputum GS/CS

Sputum AFB 3x, BCS x 2, PPD were requested

Assessment
o Non resolving pneumonia with
consolidation
o R/O pulmonary mass

Therapeutics
o Ceftazidime 1 gram IV q8
o Clindamycin 500 mg 1 cap qid
o N-Acetylcysteine 600 mg tab od
o Salburamol + Ipratropium neb q6
o O2 inhalation 4 lpm vnc
o IVF D5NSS 1 L x 8 hours
o Phlebotomy 500 cc
2nd- 5th Hospital Day

S/O>
o less dyspneic
o can move with minimal distance
o less productive cough

C/L: SCE, no retractions, decrease crackles

P> cont. antibiotics O2 to 2 LPM
Page2of 18
INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
Test
Result
Sputum CS Moraxella Catarrhalis; Alpha Hemolytic Streptococci
Blood CS
NG x 4 days
AFB smear (-) x3
PPD
Negative
H.
8th Hospital Day

S> sudden progression of dypsnea at rest

O>

Coherent, in respiratory distress

O2 sat 85% at 2 LPM

C/L: supraclavicular retractions, increase
crackles L>R

No cyanosis
CBC
Result
ABG
Result
Hgb
189
pH
7.429
Hct
0.68
PO2
63.8
WBC
7.4
PCO2
37.6
Seg
0.58
HCO3
24.3
Lymp
0.32
BE
0.3
PC
194
O2sat
93
O2
2 lpm



Team #
non resolving pneumonia
o t/c bronchogenic carcinoma
o interstital kung disease
P> Diagnostic s
o Sputum cytology
o Fiberoptic Bronchoscopy (FOB)
Therapeutics
o O2 inhalation 4 LPM nasal prong;
(O2 sat 90-93%)
o Ceftazidime shifted to Ceftriaxone 1
gram IV q8 (culture guided)
o Continue nebulization q4
Figure 1. Chest HRCT: Nonspecific multi segmental/
lobar interstitial disease. Mild centrilobular emphysema.
Old nodal TB
I.
Salient Features

Persistent hypoxemia

Erythrocytosis

Radiographic
findings
of
persistent/
progressive interstitial infiltrates.

Chest HRCT: Nonspecific multi segmental/
lobar interstitial disease.
J.
Final Diagnosis

Non-resolving pneumonia
o t/c interstitial Lung disease

Polycythemia Vera Secondary to Chronic
Hypoxemia

Chronic Obstructive Pulmonary Disease
Always start with the duration of symptoms!
There is a need to quantify the level of exertion: mild to
moderate
There is a need to determine whether previous intervention
was enough
Review of systems: do it from head to toe!
Smoking status: compute for the pack years to know its
significance
ILD is a rare condition, you usually do not consider it as a first
impression!
Case:
o Primary impression is COPD, particularly emphysema
because of his 40 pack year smoking history and easy
fatigability
o Differentials:

Pulmonary TB is always a differential for a
Filipino with chronic cough

Bronchiectasis can cause crackles

Asthma has an adult-onset variant

Congestive heart failure should be considered

Interstitial lung disease
Page3of 18
INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
o
Lab results show markedly increased hemoglobin and
hematocrit

Polycythemia vera secondary to chronic
hypoxemia
o Assessment at this point

Non-resolving pneumonia with consolidation
to rule out pulmonary mass
o 8th hospital day

Improved infiltrated in the upper left lung field
o High-resolution CT scan

Non-specific multi-segmental interstitial space,
almost like honey combing!

Note that honeycombing implies cystic lesions
and are associated with poorer prognosis 
giving the impression that it might be ILD!
______________________________________________________
III. INTERSTITIAL LUNG DISEASE
B.
Figure 2. Diffuse Parenchymal Lung Disease.
A.
Team #
Estimated Relative Frequency of Interstitial Lung
Diseases
Major Categories of Alveolar and Interstitial
Inflammatory Lung Diseases

Lung response
o alveolitis, interstitial inflammation and
fibrosis
o granulomatous
Page4of 18
INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
C.
Pathogenesis of Pulmonary Fibrosis
Figure 3. Pathogenesis of Pulmonary Fibrosis. ILDs represent a
large number of conditions that involve the parenchyma of
the lung—the alveoli, the alveolar epithelium, the capillary
endothelium, and the spaces between these structures, as
well as the perivascular and lymphatic tissues.

Team #
Alveolar Membrane, barrier to diffusion of oxygen,
consists of:
o alveolar epithelial cells
o fused alveolar and capillary basement
membrane
o capillary endothelial cells
o The alveoli are composed of: type I or type II
pneumocytes on one side and the
endothelium on the other
o We can also find the interstitial space (for gas
exchange) where the pathology of Interstitial
Lung Disease (ILD) normally takes place
o The interstitium includes the space between
the epithelial and the endothelial basement
membranes and it is the primary site of injury
in IIPs.
o However, these disorders frequently affect
not only the interstium but also the airspaces,
peripheral airways, and the vessels along with
their respective epithelial and endothelial
linings.
Page5of 18
INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
o
o
o
o


Team #
Cellular bases for the pathogenesis of interstitial lung
disease
o Multiple microinjuries damage and activate
alveolar epithelial cells (top left), which in turn
induce an antifibrinolytic environment in the
alveolar spaces, enhancing wound clot
formation
o Alveolar epithelial cells secrete growth factors
and induce migration and proliferation of
fibroblasts
and
differentiation
into
myofibroblasts (bottom left)
o Subepithelial myofibroblasts and alveolar
epithelial cells produce gelatinases that may
increase basement membrane disruption and
allow fibroblast–myofibroblast migration
(bottom right)
o Angiogenic factors induce neovascularization
o Both
intraalveolar
and
interstitial
myofibroblasts secrete extracellular matrix
proteins, mainly collagens
o An
imbalance
between
interstitial
collagenases and tissue inhibitors of
metalloproteinases provokes the progressive
deposit of extracellular matrix (top right)
o Signals
responsible
for
myofibroblast
apoptosis seem to be absent or delayed in
usual interstitial pneumonia, increasing cell
survival
o Myofibroblasts produce angiotensinogen that
as angiotensin II provokes alveolar epithelial
cell
death,
further
impairing
reepithelialization
Inflammation and Fibrosis
o The initial insult is an injury to the epithelial
surface causing inflammation in the air spaces
and alveolar walls
o If the disease becomes chronic, inflammation
spreads to adjacent portions of the
interstitium and vasculature and eventually
causes interstitial fibrosis
o The development of irreversible scarring
(fibrosis) of alveolar walls, airways, or
vasculature is the most feared outcome in all
of these conditions because it is often
progressive and leads to significant
derangement of ventilatory function and gas
exchange
lungs are small ,heavy, variegated appearance
as parenchyma replaced by pale fibrosis more
marked in the lower lobes, subpleural regions
and interlobular septa
honeycombed spaces up to 1.5 cm in
diameter, although most are less than 0.5 cm
arteries have thick walls
pleura is thin

Granulomatous Lung Disease
o This process is characterized by an
accumulation of T lymphocytes, macrophages,
and epithelioid cells organized into discrete
structures (granulomas) in the lung
parenchyma
o The granulomatous lesions can progress to
fibrosis
o Many patients with granulomatous lung
disease remain free of severe impairment of
lung function, or, when symptomatic, they
improve after treatment
o The main differential diagnosis is between
sarcoidosis and hypersensitivity pneumonitis
D.
Diagnostic Process in DPLD
E.
History: Duration of Illness

Acute
o acute interstitial pneumonia (AIP)
o eosinophilic pneumonia
o hypersensitivity pneumonitis

Subacute
o sarcoidosis
o drug induced ILDs
o Cryptogenic Organizing pneumonia
(COP)
Page6of 18
INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
o

F.
Team #
Acute Immunologic Pneumonia, SLE
or Polymyositis
Chronic
o idiopathic Pulmonary Fibrosis (IPF)
o Sarcoidosis
o Pulmonary
langerhans
cell
histiocytosis (PCLH)
o Pneumoconiosis
o Connective Tissue Disease
Past History: Who are at Risk?

Age
o 20-40 y/o

sarcoidosis

ILD associaited with CTD

Lymphanioleiomyomatosis

Pulmonary
Langerhans
Histiocytosis (PLCH)

Inherited forms of ILD
Familial IPF
Gaucher’s
disease
HermanskyPudlak
syndrome
o 50 y/o

Gender
o Female

LAM

Pulmonary involvement in
tuberous sclerosis

ILD in Hermansky-Pudlak
syndrome

CTDs
o Male

ILD in rheumatoid arthritis
(RA)

pneumoconioses

Smoking History
o PLCH
o Desquamative
interstitial
pneumonia (DIP)
o Goodpasture’s syndrome
o Repiratory bronchiolitis
o Pulmonary alveolar proteinosis

Family history
o Autosomal dominant

Tuberous sclerosis

Neurofibromatosis
o Autosomal Recessive

Niemann-Pick disease

Gaucher's disease

Hermansky-Pudlak
syndrome

Medications
Agent
Clinical Syndrome
Bleomycin,
Methotrexate
Pulmonary fibrosis and HP
Cyclophosphamide,
Carmustine (BCNU),
Azathioprine
Pulmonary fibrosis
Nitrofurantoin
Acute Infiltrates with Eos –
1mo
Chronic – 6 mos. to 2 y
Amiodarone
Pulmonary fibrosis
Table 1. Drugs Associated with ILD


Occupational history
Environmental exposure
Disease
Farmer’s lung
Antigen
Faeni rectivirgula
Humidifier lung
Thermoactinomyces
Bagassosis
Thermoactinomyces
vulgaris
Avian
droppings,
feathers, serum
Alternaria
sp.,
wood dust
Pigeon
breeder’s
lung
Woodworkers lung
Source
Moldy hay, grain,
silage
Contaminated
water reservoirs
Moldy sugarcane
Parakeets, pigeons,
chickens, turkeys
Oak,
cedar,
mahogany
dust,
pine and spruce
pulp
Table 2. Environmental/ Occupational Exposures
G.
General Pulmonary Symptoms

progressive dyspnea- over months to years

cough- nonproductive

fatigue and weight loss

Pleuritic chest pain – rare

Wheezing- hypersensitivity pneumonitis

Hemoptysis – rare

Other sign and symptoms: clubbing, joint
swelling
H.
General Pulmonary Findings

bibasilar end-inspiratory dry crackles (Velcro
rales)

resting tachypnea and tachycardia

increase in pulmonic component of 2nd heart
sound, tricuspid insufficiency

peripheral edema- cor pulmonale

digital clubbing

cyanosis

extra pulmonary findings
Page7of 18
INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
2.
High Resolution CT Scan (HRCT)
Figure 4. Clinical Profile of LCP Patients Diagnosed with ILD 19982007 (Flaviano & Diaz).
I.
What are the Ways to Diagnose ILD
1. Chest Radiography

reticular pattern

nodular pattern
o sarcoidosis
o PLCH
o Chronic hypersensitivity pneumonitis
o Silicosis
o Beryliosis
o RA (necrobiotic nodular form)
o Ankylosing spondylitis

mixed pattern – most common pattern

honeycombing- poor prognosis
Figure 6. High Resolution CT Scan (HRCT) of ILD.






HRCT has become an integral part of the evaluation of
the patient with idiopathic interstitial pneumonia
High-resolution computed tomography (HRCT) is
superior to the plain chest x-ray for early detection and
confirmation of suspected ILD
Also, HRCT allows better assessment of the extent and
distribution of disease, and it is especially useful in the
investigation of patients with a normal chest
radiograph.
Coexisting disease is often best recognized on HRCT
scanning, e.g., mediastinal adenopathy, carcinoma, or
emphysema.
In the appropriate clinical setting HRCT may be
sufficiently characteristic to preclude the need for lung
biopsy in IPF, sarcoidosis, hypersensitivity pneumonitis,
asbestosis, lymphangitic carcinoma, and PLCH.
When a lung biopsy is required, HRCT scanning is useful
for determining the most appropriate area from which
biopsy samples should be taken.
Figure 5. Radiographic finding of honeycombing
Team #
Page8of 18
INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010

5.
Sarcoid
Exercise induced hypoxemia common in
ILD
Serology
ACE, Lysozyme
Wegener’s Goodpasture’s
c-ANCA, p-ANCA
Anti-GBM
Rheumatoid
Rheumatoid factor
Scleroderma
SCL-70, anti-centromere
Sjogren’s
SS-A, SS-B
Lupus
ANA, DS-DNA, anti-histone
Polymyositis
Anti Jo-1
ILD
SP-A, SP-B, MCP-1, KL-6
6.
3.
4.
Team #
Spirometry and Lung Volumes

asses lung involvement severity

obstructive vs. restrictive pattern

most ILD, have a restrictive defect

Reductions in TLC, FRC, RV

Decrease in flow rates (FEV1 & FVC)

DLCO reduction common but nonspecific
ABG

normal or may reveal hypoxemia

respiratory alkalosis

Co2 retention in end stage ILD

Normal Po2 does not exclude ILD
Lung Biopsy
a. Surgical Lung Biopsy
 establish a specific diagnosis
o with
atypical
signs/
symptoms
o normal radiographic features
 exclude
neoplastic/
infectious
processes
 assess disease activity
 identify a more treatable process
 definitive
diagnosis/
predict
prognosis prior to initiation of
therapy
 to establish firm clinicopathologic
diagnosis, and allows patient and
clinician to make more informed
decisions about treatment option
 almost all of the current treatments
for the IIPs have potentially serious
risks and side effects, and it is not
reasonable to expose patients to
these risks in the presence of
diagnostic uncertainty
 detection of fibrotic processes
related to specific exposures can
have
important compensation
implications for the patient, and
important
public
health
consequences for the community;
for example, asbestosis
 to establish etiology and stage of ILD
 high diagnostic yield (>92%)
 Open thoracotomy
b. VATS
 same
specimen
adequacy
&
diagnostic accuracy
Page9of 18
INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010

c.
Neutrophils
preferred method in part because
several retrospective studies have
found that morbidity is improved
with VATS compared with open
thoracotomy.
FOB with Transbronchial Lung Biosy/
Bronchioalveolar Lavage (BAL)
 initial procedure of choice
 BAL for malignancy or opportunistic
infection
 Installation of saline into pathologic
areas and retrieval of fluid for
analysis
 Interpretation
in
context
of
clinical/HRCT findings
 BAL cellular counts with no
prognostic significance
 Cellular profiles specific ILDs
Eosinophils
Table 4. Lung conditions and their respective bronchoalveolar
lavage findings.
Lymphocytes
Infection
Eosinophilic PNA
Sarcoidosis
ARDS
Drug-induced ILD Hypersensitivity
pneumonitis
AIP
Churg-Strauss
COP
Hypereosinophilic Radiation
syndrome
pneumonitis
DIP
Tropical
Eosinophilia
Drug-induced ILD
Rheumatologic
lung disease
Figure 8. ATS/ ERS Criteria for Diagnosis of Idiopathic Pulmonary
Fbrosis in Absence of Surgical Lung Biopsy
Table 3. BAL WBC Differential Profiles in ILD
J.
Figure 7. Probability of Diagnosis Diffuse Diseases
Team #
Treatment

Remove offending agent

Supportive management

Drug therapy
o Glucocorticoids

mainstay of therapy for
suppression of alveolitis in
organic dust disease

starting
dose
of
prednisone 0.5–1 mg/kg in
a once-daily oral dose
(based on the patient's
lean body weight) x 4-12
weeks

Maintenance dose 0.250.5 mg/kg x 4-12 weeks
o Cyclophosphamide and Azathioprine

(1–2 mg/kg lean body
weight per day), with or
without glucocorticoids
o Methotrexate
o Colchicine
o Penicillamine
Page10of 18
INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
o
K.
Cyclosporine

ILD Associated with Connective Tissue Disorders


1.
Progressive Systemic Sclerosis (PSS)

Clinical evidence of ILD is present in about
one-half of patients with PSS, and pathologic
evidence in three-quarters

Pulmonary function tests show a restrictive
pattern and impaired diffusing capacity

Pulmonary vascular disease alone or in
association with pulmonary fibrosis, pleuritis,
or recurrent aspiration pneumonitis is
strikingly resistant to current modes of
therapy.
2.
Rheumatoid Arthritis (RA)

more common in men

Pulmonary manifestations include pleurisy
with or without effusion

ILD in up to 20% of cases
o necrobiotic
nodules
(nonpneumoconiotic intrapulmonary
rheumatoid nodules) with or without
cavities
o Caplan's
syndrome
(rheumatoid
pneumoconiosis)
o pulmonary hypertension secondary to
rheumatoid pulmonary vasculitis
o BOOP
o upper airway obstruction due to cricoarytenoid arthritis.
3.
Systemic Lupus Erythematosus (SLE)

Pleuritis with or without effusion is the most
common pulmonary manifestation

Other lung manifestations include the
following:
atelectasis,
diaphragmatic
dysfunction with loss of lung volumes,
pulmonary vascular disease, pulmonary
Team #
hemorrhage, uremic pulmonary edema,
infectious pneumonia, and BOOP
Acute lupus pneumonitis characterized by
pulmonary capillaritis leading to alveolar
hemorrhage is uncommon
Chronic, progressive ILD is uncommon.
It is important to exclude pulmonary infection.
Although pleuropulmonary involvement may
not be evident clinically, pulmonary function
testing,
particularly
DLCO,
reveals
abnormalities in many patients with SLE.
4.
Polymyositis and Dermatomyositis (PM/DM)

ILD occurs in ~10% of patients with PM/DM.

Diffuse reticular or nodular opacities with or
without an alveolar component occur
radiographically, with a predilection for the
lung bases.

Weakness of respiratory muscles contributing
to aspiration pneumonia may be present.

A rapidly progressive illness characterized by
diffuse alveolar damage may cause respiratory
failure.
5.
Sjogren’s Syndrome

General dryness and lack of airways secretion
cause the major problems of hoarseness,
cough, and bronchitis.

Lymphocytic
interstitial
pneumonitis,
lymphoma, pseudolymphoma, bronchiolitis,
and bronchiolitis obliterans are associated
with this condition.

Lung biopsy is frequently required to establish
a precise pulmonary diagnosis.

Glucocorticoids have been used in the
management of ILD associated with Sjögren's
syndrome with some degree of clinical success
ILD is a histopathologic diagnosis!
Diffuse parenchymal diseases manifest with same symptoms,
differentiated only by histopathology.
ILD classification is difficult to make
Page11of 18
INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
Note that idiopathic interstitial pneumonias pose a 40% risk.
ILD involves lung parenchyma and not airways.
Recall that Type II epithelium produced surfactant.
ILD involves an autoimmune process resulting to fibrosis,
causing stiffness of airways that lead to problems with
respiration leading to hypoxemia.
Note the drugs associated with ILD.
Note that lymphangioleiomyomatosis (LAM) is only seen in
women, usually of the young reproductive age, while most
ILDs manifest in > 50 y.o.
In a study conducted at the lung center, it was found that the
most common presentation among Filipino patients is cough
(96.30%) followed by difficulty of breathing (59.30%).
Measurement of ILD presents as restrictive: Normal FEV1/FVC
ratio, decreased FEV1, decreased FVC
Serology is important with cases of probable known causes.
Connective tissue diseases (CTD) are difficult to rule out,
therefore consider them first.
Most common form of CTD is SLE, which presents with
pleuritis.
Most common with granulomatous lung response is
sarcoidosis.
Most common with UNKNOWN etiology and vague
presentation in Idiopathic Interstitial Lung Disease is
Idiopathic Pulmonary Fibrosis (IPF) or Unspecified Idiopathic
Pneumonia (UIP), which present with honeycombing and has
a 50% 5-year mortality rate.
______________________________________________________
IV.
IDIOPATHIC PULMONARY FIBROSIS
INTERSTITIAL PNEUMONIA (UIP)
(IPF)/
Figure 9. IPF/ UIP. Chest radiograph shows typical peripheral
reticular opacity, most marked at the bases, with honeycombing.
Lower lobe volume loss
USUAL
A. Clinical Manifestations

exertional dyspnea

Nonproductive cough

Inspiratory crackles with or without digital
clubbing may be present on physical
examination.
Figure 10. IPF/ UIP CT images show basal predominant peripheral
predominant reticular abnormality with traction bronchiectasis
and honeycombing.
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INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010

Often mechanical ventilation is required but is
usually not successful, with a hospital
mortality rate of up to three-fourths of the
patients.

In those that survive, a recurrence of acute
exacerbation is common and usually results in
death at those times.

Lung transplantation should be considered for
those patients who experience progressive
deterioration despite optimal medical
management and who meet the established
criteria
______________________________________________________
V.
DESQUAMATIVE INTERSTITIAL PNEUMONIA (DIP)
 DIP is a rare but distinct clinical and pathologic entity
found exclusively in cigarette smokers
 The peak incidence is in the fourth and fifth decades
 Most patients present with dyspnea and cough
Figure 11. IPF/ UIP Pattern. Marked fibrosis consisting of dense
collagenous scarring with remodeling of the lung architecture and
cystic changes
Figure 12. DIP HRCT shows peripheral predominant ground glass
abnormality, with some associated cystic changes
B. Clinical Course

median length of survival from time of
diagnosis varies between 2.5 and 3.5 yr (50%
in 5 yr)

periods of rapid decline that represents
accelerated disease, intercurrent viral
infection with the development of organizing
pneumonia, or diffuse alveolar damage

Improvement in lung physiology and radiologic
abnormalities is rare
C. Treatment

no therapy has been found to be effective in
the management of acute exacerbations of IPF
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INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
Figure 13. DIP Pattern. The alveolar spaces are diffusely involved
by marked alveolar macrophage accumulation and there is mild
interstitial thickening.

DIP course
o prognosis of DIP is generally good
o most patients improve with smoking cessation
and corticosteroids
o The overall survival is about 70% after 10 yr
______________________________________________________
VI. RESPIRATORY BRONCHIOLITIS-ASSOCIATED INTERSTITIAL
LUNG DISEASE (RB-ILD)
Figure 15. RB-ILD Pattern. Faintly pigmented alveolar
macrophages fill the lumen of this respiratory bronchiole and the
surrounding airspaces. There is mild thickening of the wall of the
respiratory bronchiole.


Many patients improve after cessation of smoking.
Progression to dense pulmonary fibrosis has not been
reported.

As the number of patients studied to date is small,
definitive reports of its natural history are not available.

Survival: 5% in 5 yr
______________________________________________________
VII. ACUTE INTERSTITIAL PNEUMONIA (AIP, HAMMAN-RICH
SYNDROME)

Rare, fulminant form of lung injury

Most patients are older than 40 years

AIP is similar in presentation to the acute respiratory
distress syndrome (ARDS) and probably corresponds to
the subset of cases of idiopathic ARDS.

The onset is usually abrupt in a previously healthy
individual

A prodromal illness, usually lasting 7–14 days before
presentation, is common

Fever, cough, and dyspnea are frequent manifestations
at presentation.

HRCT shows a geographic distribution of ground glass
abnormality with consolidation
Figure 14. RB- ILD HRCT lung scanning shows bronchial wall
thickening, centrilobular nodules, ground-glass opacity, and
emphysema with air trapping.
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INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
Figure 16. AIP Pattern: The lung shows diffuse alveolar wall
thickening by proliferating connective tissue and inflammation


There is no proven treatment
mortality rates are high (>50-60%), most deaths
occurring between 1 and 2 mo of illness onset.

Survivors of AIP may experience recurrences and
chronic, progressive interstitial lung disease
______________________________________________________
Figure 17. COP. Chest radiograph shows multifocal consolidation
(arrows).
VIII. CRYPTOGENIC ORGANIZING PNEUMONIA (COP)

Clinicopathologic syndrome of unknown etiology

The onset is usually in the fifth and sixth decades

The presentation may be of a flulike illness with cough,
fever, malaise, fatigue, and weight loss

Inspiratory crackles are frequently present on
examination

Pulmonary function is usually impaired, with a
restrictive defect and arterial hypoxemia being most
common.

Non-specific reaction to lung injury found adjacent to
other pathologic processes or a component of other
primary pulmonary disorders:
o Cryptococcosis
o Wegener's granulomatosis
o Lymphoma
o Hypersensitivity pneumonitis
o Eosinophilic pneumonia
Figure 18. COP. HRCT shows areas of air-space consolidation,
ground-glass opacities, small nodular opacities, and bronchial wall
thickening and dilation. These changes occur more frequently in
the periphery of the lung and in the lower lung zone.
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INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
Figure 19. COP Pattern. Consists of polypoid plugs of loose
organizing connective tissue within an alveolar duct and the
adjacent alveolar spaces.
F
Figure 20. NSIP. HRCT shows bilateral, subpleural ground-glass
opacities, often associated with lower lobe volume loss. Patchy
areas of airspace consolidation and reticular abnormalities may be
present, but honeycombing is unusual.

Majority of patients recover completely on
administration of oral corticosteroids

A significant number relapse within 1 to 3 months when
the corticosteroids are reduced or stopped.

Prolonged treatment for 6 months or longer is advised.

A small proportion of patients recovers spontaneously

Death is rare
______________________________________________________
IX.
NON-SPECIFIC INTERSTITIAL PNEUMONIA (NSIP)

Idiopathic NSIP is a subacute restrictive process with a
presentation similar to IPF but usually at a younger age,
most commonly in women who have never smoked

It is often associated with a febrile illness.
Team #
Figure 21. NSIP Pattern. The key histopathologic features of NSIP
are the uniformity of interstitial involvement across the biopsy
section, and this may be predominantly cellular or fibrosis. There
is less temporal and spatial heterogeneity than in UIP and little or
no honeycombing is found.
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INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010

The prognosis of NSIP is more variable than in IPF and
appears to depend on the extent of fibrosis

Some patients experience almost complete recovery,
and most of the remainder stabilize or improve on
treatment

Relapse may occur.

A minority of patients progress and die (10% in 5 yr)
______________________________________________________
X.
LYMPHOCYTIC INTERSTITIAL PNEUMONIA (LIP)
Figure 23. LIP Pattern. There is diffuse thickening of alveolar walls
by a moderately severe infiltrate of lymphocytes and plasma cells.



F
Figure 22. LIP. HRCT shows diffuse ground glass attenuation with
multiple lung cysts
Team #
Corticosteroids are the most widely used treatment,
and are thought to arrest or improve symptoms in a
large proportion of patients.
More than one-third progress to diffuse fibrosis, and it
is unclear whether treatment influences the course of
the disease or has a significant effect on lung
physiology.
Occasional cases resolve or improve substantially.
Note those that respond to therapy:
o LIP

Corticosteroids relieve symptoms
o COP

Unknown etiology

Responds to corticosteroids
Lung transplantation has not been done in the country as of
date.
DIP
o Peak incidence is 4th to 5th decade
o Prognosis is generally good
IPF and DIP are associated with smoking!
Role of pulmonologists in ILD is the provision of a high index
of suspicion.
AIP (Hamman-Rich Syndrome) presents like ARDS and with
unspecific symptoms such as fever, cough and dyspnea
NSIP
o Subacute restrictive
o Younger women who are never smokers
o 10% mortality rate in 5 years
o No honeycombing! Better prognosis!
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INTERSTITIAL LUNG DISEASE
Year Level 7 [Pulmonary Module]|July 30, 2010
XI.
DPLD (OTHER FORMS)





Pulmonary Langerhans Cell Histiocytosis (PLCH)
Pulmonary Alveolar Proteinosis (PAP)
Pulmonary Lymphangioleiomyomatosis
Syndromes of ILD with Diffuse Alveolar Hemorrhage
Goodpasture's Syndrome
FOR THE EXAM, note the following: 
o Those that respond to therapy
o Differences in histopathology
o The most common ones mentioned
o Those that are common with not the usual
patients (old/ smokers)
o Honeycombing= worse prognosis
Team #
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