New Patient with Non-Focal Fibrosis on Chest CT

ILD Process Map

New Patient With Nonfocal Fibrosis on Chest CT Scan

Cough Shortness of Breath Symptoms of Underlying CTD (eg, dry eyes, dry mouth, dysphagia, myalgia, arthralgia, rashes, Raynaud's syndrome)

Physical Exam

Physiology Assessment

History

Phase 1

Crackles Joint Deformities Rashes or Skin Thickening Weakness

Pulmonary Function Test 6-Minute Walk Test

Baseline Evaluation General Medicine

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.

Radiology Review - Identify High-Resolution CT Scan Pattern to Construct Clinical Differential

History

Less Common Patterns

Common Patterns

Exposure Assessment PDF

Medication Review Webpage

Phase 1

Cystic Lung Disease LAM LCH LIP Birt-Hogg-Dube

Phase 2

UIP Pattern

NSIP Pattern CTD-ILD HP Drug Toxicity Idiopathic NSIP

DIP Pattern Smoking Toxic fumes Idiopathic DIP CTD-ILD HIV

LIP Pattern CTD-ILD HIV Immunodeficiences

HP Pattern Nonfibrotic HP Fibrotic HP

Pulmonary Evaluation

Clinical Differential:

Clinical Differential:

Clinical Differential:

Clinical Differential:

Clinical Differential:

Clinical Differential:

IPF CTD-ILD Exposure (eg, HP)

Integration of Clinical, Serological, and Radiographic Data Is diagnostic confidence level >70%?

Management by Disease Subtype

NO

YES

If unclassifiable or low diagnostic confidence, consider bronchoscopy with lavage, cryobiopsy, or surgical biopsy for pathologic diagnosis.

Phase 3

Multidisciplinary Discussion

IPF

HP

CTD-ILD

Smoking-Related ILD

Pneumoconiosis

Other Rare ILD

Abbreviation Key ^

RB-ILD

LCH

DIP

Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis

Diagnosis and Evaluation of Hypersensitivity Pneumonitis: CHEST Guideline and Expert Panel Report

Diagnosis of Hypersensitivity Pneumonitis in Adults: An Official ATS/JRS/ALAT Clinical Practice Guideline

CHEST ILD Toolkit

CHEST ILD Toolkit

Examples of autoimmune serology tests to consider:

• ANA
• RF/CCP
• CK/aldolase
• ANCA
• Scl-70 and PM-Scl
• SSA
• SSB
• RNP
• 11-antibody myositis panel

CHEST ILD Toolkit

2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Screening and Monitoring of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases

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2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases

Abbreviations

CT - computed tomography CTD-ILD - connective tissue disease-associated interstitial lung disease DIP - desquamative interstitial pneumonia HP - hypersensitivity pneumonitis ILD - interstitial lung disease IPF - idiopathic pulmonary fibrosis

LAM - lymphangioleiomyomatosis LCH - Langerhans cell histiocytosis LIP - lymphoid interstitial pneumonia NSIP - nonspecific interstitial pneumonia RB-ILD - respiratory bronchiolitis-associated interstitial lung disease UIP - usual interstitial pneumonia

CHEST ILD Toolkit

CHEST ILD Toolkit