New Patient with Non-Focal Fibrosis on Chest CT - Mobile

ILD Process Map

New Patient With Nonfocal Fibrosis on Chest CT Scan

Phase 1

Baseline Evaluation General Medicine

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

History

Crackles Joint Deformities Rashes or Skin Thickening Weakness

Physical Exam

Pulmonary Function Test 6-Minute Walk Test

Physiology Assessment

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.

History

Exposure Assessment PDF

Medication Review Webpage

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

Common Patterns

UIP Pattern

NSIP Pattern CTD-ILD HP Drug Toxicity Idiopathic NSIP

HP Pattern Nonfibrotic HP Fibrotic HP

Clinical Differential:

Clinical Differential:

Clinical Differential:

IPF CTD-ILD Exposure (eg, HP)

Less Common Patterns

Cystic Lung Disease LAM LCH LIP Birt-Hogg-Dube

DIP Pattern Smoking Toxic fumes Idiopathic DIP CTD-ILD HIV

LIP Pattern CTD-ILD HIV Immunodeficiences

Clinical Differential:

Clinical Differential:

Clinical Differential:

Phase 3

Multidisciplinary Discussion

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

NO

YES

Management by Disease Subtype

Pneumoconiosis

IPF

HP

CTD-ILD

Other Rare ILD

Smoking-Related ILD

RB-ILD

LCH

DIP

NO

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

Abbreviation Key ^

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