Download Disclosures: None

4/24/2017
Routine Reaction to DPLD (AKA ILD)
No longer living in the
Shadows: ILD hits
primetime
Yolanda Mageto MD, MPH
Professor of Medicine
Director of Interstitial Lung Disease
Center for Advanced Heart and Lung Disease
Baylor Scott and White
The Diagnosis of DPLD is Difficult!
• Diverse group of illnesses of over 150 disorders involving the distal pulmonary
parenchyma
Disclosures: None
• Similar in presentation, physiology, radiology and sometimes pathology
• Successful management of these patients depends on accurate diagnosis based
on history and interpretation of data
• Accurate diagnosis allows
• Provision of accurate prognostic information
• Development of a management strategy
Learning Objectives
1. Highlight the rationale for an accurate diagnostic approach
patients with suspected interstitial lung disease
Diffuse Parenchymal Lung Disease (DPLD)
Idiopathic
interstitial
pneumonias
DPLD of known cause, eg,
drugs or association, eg,
collagen vascular disease
Idiopathic
pulmonary
fibrosis
2. Apply a sequential algorithmic approach to diagnosis in
patients with suspected interstitial lung disease, while
highlighting complexity involved in diagnosis
Classification of DPLD
OR of Known
Cause?
Granulomatous
DPLD, eg,
sarcoidosis
Other forms of
DPLD, eg, LAM,
HX, etc
IIP other than
idiopathic
pulmonary fibrosis
Desquamative interstitial
pneumonia
Respiratory bronchiolitis
interstitial lung disease
Acute interstitial pneumonia
Cryptogenic organizing
pneumonia
Nonspecific interstitial
pneumonia (provisional)
Lymphocytic interstitial
pneumonia
Pleuroparenchymal
fibroelastosis
Travis WD, et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med. 2013;188(6):733-748.
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Diagnostic Tools
Recommended Autoantibody Screening
Clinical Assessment
• Comprehensive medical history ( focusing on social,
occupational, environmental, drug, and family)
• Time course
• Pulmonary function testing
• Physical exam – pulmonary
• Serologic testing
Radiological Assessment
• Chest x-ray
• HRCT
Surgical Assessment
• Bronchoscopy or surgical lung biopsy as indicated
Diagnostic Algorithm for IPF
Case Study (ies):
Patient presents with
suspected ILD
Detailed history
Physical exam
PFT
YES
Positive
•58 year old female with cough and
dyspnea
Identifiable cause of ILD
Serologic testing to exclude
CTD
Definite UIP
HRCT
IPF
Not IPF
Raghu G, et al. Am J Respir Crit Care Med. 2011;183:788-824
Known Causes of ILD:
• Drugs
– eg, Amiodarone, bleomycin,
nitrofurantoin, cocaine,
– www.pneumotox.com
• Radiation
‒ External beam radiation
therapy to thorax
• Connective Tissue Diseases
– Rheumatoid arthritis
– Systemic sclerosis
(scleroderma)
– Idiopathic inflammatory
myopathies
– Vasculitis
– Primary Sjogren’s
– Bechet’s syndrome
• Occupational/Environmental
– Inorganic antigens
(Pneumoconioses)
• Asbestosis
• Coal worker’s
pneumoconiosis
• Silicosis
– Organic antigens
(Hypersensitivity Pneumonitis)
• Birds
• Mold
• Farm antigens
58 year old female with cough and dyspnea
• Presenting symptoms
• Very Active female bikes several miles weekly no significant past medical
history
• Cough dry non productive x 3 months initially attributed to allergy, treated
with inhaler no improvement, progressively worse
• Dry non productive
• Exertional dyspnea progressive over the last year
• Bibasilar crackles on exam
• Further imaging.
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Case Study: 58 year old female (cont’d)
Case Study: 58 year old female (cont’d)
• Lifelong non smoker – no second hand smoke
• No family history of collagen vascular disease or pulmonary issues
• No recent travels
• Physical exam:
• Occasional swelling in her joints,
• Crackles on exam ¼ way up otherwise negative exam
• No history or Raynaud's, rashes or apthous ulcers.
Case Study: 58 year old female (cont’d)
Case Study: 58 year old female (cont’d)
• PFT’s
• Clinical course:
•
•
•
•
FVC 3.26 (86%)
FEV1 2.57 (84%)
FEV1/FVC 0.79
DLCO 60%
• Serologic testing
• ANA 1:60
• RF 40
• All other testing negative
Case Study: 58 year old female (cont’d)
• Initially diagnosed with IPF
•
•
•
•
•
However further history taking revealed patient was a pigeon breeder in her spare time
Diagnosis: chronic hypersensitivity pneumonitis.
Treated with steroids
Birds removed
Symptoms improved and PFT’S remained stable.
Case Study: 58 year old female (cont’d)
• SAME PRESENTATION Different History
• Elevated SCL -70 or CCP antibodies or aldolase – likely CT ILD
• Medications – amiodarone, Methotrexate, Nitrofurantoin – likely medication
induced fibrosis
• NO additional history of findings then likely IPF
• IF UNSURE GET A BIOPSY – to be read by a lung pathologist.
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Care of the ILD patient
Disease Based
Management
Pharmacologic
Therapy
Non
Pharmacologic
Therapy
Education and Self
Management
Symptom Centered
Management
Knowledge
Cough
Dyspnea
Values and
Preferences
Co-Morbidities
and
Complications
Preventative
Care
Advanced Care
Planning
Fatigue and
Deconditioning
Depression and
Anxiety
Adapted from Lee, Joyce; McLaughlin, Sally; Collard, Harold; Current Opinion in Pulmonary Medicine.
17(5):348-354, September 2011.
A few take home thoughts …..
• IIP is an evolving arena – keep an open mind
• Be consistent in your approach to diagnosis
• PFT’s can be normal
• Patients often have comorbidities don’t get tunnel
vision
• If the clinical radiographic picture does not fit acquire
TISSUE!
• All Lower lobe infiltrates are not ‘double pneumonia’
• Make the investment up front -Good history taking is
Key !!
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Tunnel Vision: Concomitant
disease and comorbidities
Summary: Concomitant
Disease/Comorbidities
• Avoid Tunnel vision
• Recognize a change in symptoms may not be due to IPF or ILD
• Consider
•
•
•
•
•
•
OSA
Cancer
CAD
GERD
PAH
Emphysema (COPD exacerbation)
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