How should fibrosis seen on a chest X‑ray be evaluated and managed?

Evaluation and Management of Fibrosis on Chest X-Ray

When fibrosis is identified on chest X-ray, immediately obtain a high-resolution CT (HRCT) scan to characterize the extent and pattern of disease, as chest X-ray findings are insufficient for diagnosis and management decisions. 1, 2

Initial Diagnostic Approach

Immediate CT Imaging

• Chest X-ray abnormalities suggesting fibrosis require HRCT confirmation, as more than half of patients with idiopathic pulmonary fibrosis show interstitial lung abnormalities on chest X-rays even before symptom onset 1
• HRCT is essential to distinguish between interstitial lung abnormalities (ILAs) and established interstitial lung disease (ILD), which fundamentally changes management 3
• The pattern on HRCT determines whether this represents early disease (ILAs) versus established fibrotic ILD requiring treatment 3, 2

Distinguish ILAs from ILD

According to the 2025 American Thoracic Society guidelines, progression from ILAs to ILD requires at least one of the following 3:

Symptomatic criteria:

• Any dyspnea and/or cough attributable to ILD 3

Physiologic criteria (any of):

• FVC, TLC, or DLCO below lower limit of normal attributable to ILD 3
• Meets criteria for progressive pulmonary fibrosis 3

Imaging criteria (any of):

• Fibrotic abnormalities (honeycombing and/or reticulation with traction bronchiectasis) involving ≥5% of total lung volume 3
• Progressive fibrotic abnormality on serial HRCT 3
• Major fibrotic ILD pattern (UIP/probable UIP, fibrotic hypersensitivity pneumonitis, or fibrotic NSIP) 3

Pathology:

• Major fibrotic ILD pattern on biopsy 3

Baseline Assessment

Pulmonary Function Testing

• Obtain complete PFTs including FVC, TLC, and DLCO corrected for hemoglobin 4
• Perform 6-minute walk test with oxygen saturation monitoring 4
• These establish baseline for monitoring progression 4

Laboratory Evaluation

• Measure serum biomarkers associated with progressive pulmonary fibrosis risk: KL-6, surfactant protein D (hSP-D), MMP-7, and CA-125 5
• Screen for connective tissue diseases, particularly rheumatoid arthritis, as these are increasingly recognized causes of pulmonary fibrosis 2

Risk Stratification

• Lower baseline FVC% predicted is a risk factor for developing progressive pulmonary fibrosis 5
• Evaluate for lung cancer risk, as this is increased in fibrotic lung disease 4
• Assess for comorbidities including pulmonary hypertension, gastroesophageal reflux, and obstructive sleep apnea 4

Monitoring Strategy

Surveillance Schedule

• Repeat PFTs and 6-minute walk test every 4-6 months or sooner if clinically indicated 4
• Perform annual HRCT if clinical suspicion of worsening or increased lung cancer risk 4
• Obtain HRCT urgently if concern for acute exacerbation 4

Defining Progressive Pulmonary Fibrosis

According to the 2022 ATS/ERS/JRS/ALAT guidelines, PPF requires at least two of the following three criteria within the past year 4:

1. Worsening respiratory symptoms 4

2. Physiological progression (either):

   • Absolute FVC decline >5% predicted within 1 year 4
   • Absolute DLCO decline >10% predicted within 1 year 4

3. Radiological progression (one or more):

   • Increased extent/severity of traction bronchiectasis and bronchiolectasis 4
   • New ground-glass opacity with traction bronchiectasis 4
   • New fine reticulation 4
   • Increased extent/coarseness of reticular abnormality 4
   • New or increased honeycombing 4
   • Increased lobar volume loss 4

Critical caveat: Exclude alternative explanations for worsening, particularly for respiratory symptoms and DLCO decline, as these have lower specificity for PPF compared to FVC and CT changes 4

Treatment Approach

Antifibrotic Therapy

• For idiopathic pulmonary fibrosis, initiate either nintedanib or pirfenidone at diagnosis 4
• For progressive pulmonary fibrosis in non-IPF ILD, start antifibrotic therapy early once initial management fails to prevent progression 6
• The 2022 guidelines emphasize that patients at increased mortality risk should be evaluated for lung transplantation at diagnosis 4

Nonpharmacological Management

• Provide oxygen supplementation as needed 4
• Refer for pulmonary rehabilitation 4, 6
• Involve palliative care for symptom management (cough, dyspnea, anxiety) 4

Transplant Evaluation

• Refer high-risk patients for lung transplant evaluation at diagnosis 4
• Re-evaluate transplant candidacy if disease progresses despite treatment 4, 6

Special Considerations

Acute Exacerbations

• Treat with corticosteroids 4
• Mechanical ventilation is not recommended for the majority of patients with respiratory failure 4
• Reassess after exacerbations to determine if progression occurred 4

Multidisciplinary Discussion

• Integration of clinical, radiologic, and when available, pathologic data through multidisciplinary discussion is essential for accurate diagnosis and management 2

Clinical Trial Enrollment

• Patients should be made aware of available clinical trials for possible enrollment at all stages of disease 4