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Abbreviation Key ^
Clinical Differential: 
Clinical Differential: 
Clinical Differential: 
Clinical Differential: 
UIP Pattern  
Clinical Differential: 
 YES
NO
LCH
DIP
IPF
CTD-ILD
HP
Smoking-Related ILD
Pneumoconiosis
Other Rare ILD
If unclassifiable or low diagnostic confidence, consider bronchoscopy with lavage, cryobiopsy, or surgical biopsy for pathologic diagnosis.
Management by Disease Subtype
Integration of Clinical, Serological, and Radiographic Data 
Is diagnostic confidence level >70%?
IPF 
CTD-ILD 
Exposure (eg, HP)
Phase 1
ILD Process Map
New Patient With Nonfocal Fibrosis on Chest CT Scan
History
Physiology Assessment
Physical Exam
History
Common Patterns
Phase 3
Phase 1
Phase 2
Baseline Evaluation
General Medicine
Pulmonary Evaluation
Multidisciplinary 
Discussion
Pulmonary Function Test 
6-Minute Walk Test
Crackles 
Joint Deformities
Rashes or Skin Thickening 
Weakness
RB-ILD
Less Common Patterns
Radiology Review - Identify High-Resolution CT Scan Pattern to Construct Clinical Differential
Clinical Differential: 
NSIP Pattern


CTD-ILD 
HP 
Drug Toxicity Idiopathic NSIP
HP Pattern 

 Nonfibrotic HP Fibrotic HP
LIP Pattern 


CTD-ILD 
HIV 
Immunodeficiences
DIP Pattern 


Smoking 
Toxic fumes Idiopathic DIP 
CTD-ILD 
HIV
Cystic Lung Disease 


LAM 
LCH
LIP
 Birt-Hogg-Dube
Phase 2 

PULMONARY
EVALUATION
 

Family History 

 


If symptoms or radiographic patterns are suggestive of underlying CTD, send autoimmune serologies           and refer the patient to rheumatology.
 
Cough 
Shortness of Breath 
Symptoms of Underlying CTD 
(eg, dry eyes, dry mouth, dysphagia, myalgia, arthralgia, rashes, Raynaud's syndrome)

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