How do I work up and treat an elderly patient with fibrotic interstitial lung disease (ILD) showing mid to lower zones dominance on chest X-ray (CXR) who is asymptomatic?

Workup and Management of Asymptomatic Fibrotic ILD with Mid-Lower Zone Dominance in Elderly Patients

Obtain high-resolution computed tomography (HRCT) immediately to characterize the pattern of fibrosis, followed by a detailed exposure history and pulmonary function tests, then proceed to multidisciplinary discussion to establish a specific diagnosis before considering treatment. 1

Initial Diagnostic Workup

Immediate HRCT Imaging

• HRCT is mandatory to characterize the fibrotic pattern and determine if it meets criteria for usual interstitial pneumonia (UIP), probable UIP, indeterminate for UIP, or suggests an alternative diagnosis 1
• The HRCT should be performed with thin collimation (≤2 mm slice thickness), without contrast, with both inspiratory and expiratory images 1
• Look specifically for: subpleural and basal predominant reticulation, honeycombing, traction bronchiectasis, and absence of features suggesting alternative diagnoses (profuse ground-glass opacity, centrilobular micronodules, consolidation) 1

Detailed Exposure and Medication History

• Take a comprehensive environmental exposure history to identify or exclude hypersensitivity pneumonitis, which was found in 47% of patients initially thought to have ILD of unknown cause 1
• Specifically inquire about: mold, birds, down feathers, animals, metal dusts (brass, lead, steel), wood dust (pine), vegetable dust, livestock exposure, and all current/recent medications 1
• Document occupational exposures including hair dressing, farming, and industrial work 1

Serologic Testing

• Screen for connective tissue disease (CTD) with antinuclear antibodies, rheumatoid factor, anti-CCP antibodies, myositis panel, and anti-topoisomerase antibodies, as CTD-ILD can present with minimal extrapulmonary manifestations 1
• Consider MUC5B genotyping in patients with family history of ILD or younger age (<60 years) 1

Baseline Pulmonary Function Testing

• Obtain spirometry with FVC and DLCO to establish baseline lung function and assess disease severity 1
• Perform 6-minute walk test with oxygen saturation monitoring, as oxygen saturation ≤88% at end of test predicts worse prognosis 1

Establishing a Specific Diagnosis

Multidisciplinary Discussion (MDD)

• All cases should undergo MDD involving pulmonologists, radiologists, and pathologists experienced in ILD to integrate clinical, radiological, and if available, pathological features 1
• Complex cases should be referred to expert centers or pulmonology departments experienced in ILDs 1

Role of Lung Biopsy

If HRCT shows UIP pattern: Do NOT perform surgical lung biopsy (SLB), as the diagnosis can be made confidently with clinical and radiographic features alone 1

If HRCT shows probable UIP, indeterminate for UIP, or alternative diagnosis pattern: Consider SLB if the patient is not at high surgical risk (avoid if DLCO <25% after correction for hematocrit, severe hypoxemia at rest, or severe pulmonary hypertension) 1

• SLB obtains adequate specimens in 100% of cases but carries risks including exacerbations (6.1%), prolonged air leak (5.9%), and respiratory infection (6.5%) 1
• In elderly patients over 70 years with typical HRCT findings, the high pretest probability of IPF may make biopsy unnecessary even without definitive UIP pattern 2

Treatment Considerations

When to Initiate Antifibrotic Therapy

For confirmed IPF (UIP pattern on HRCT or appropriate HRCT-histopathology combination):

• Initiate antifibrotic therapy with pirfenidone or nintedanib even in asymptomatic patients, as these medications slow FVC decline and may reduce mortality 3, 4
• Pirfenidone reduced mean FVC decline from -428 mL to -235 mL at 52 weeks in clinical trials 3
• Both medications are FDA-approved for IPF and have shown efficacy in slowing disease progression 3, 4

For other fibrosing ILDs with progressive phenotype:

• Nintedanib is approved for chronic fibrosing ILDs with progressive phenotype and systemic sclerosis-associated ILD 5, 4
• Consider immunosuppressive therapy for CTD-ILD, hypersensitivity pneumonitis, or other inflammatory ILDs based on specific diagnosis 5, 4

What NOT to Do

• Do NOT initiate triple therapy with prednisone, azathioprine, and N-acetylcysteine in patients with definite IPF 1
• Do NOT use antacid medication for the purpose of improving respiratory outcomes in IPF, though it may be appropriate for GERD symptoms 1

Monitoring Strategy

Baseline Prognostic Assessment

• Assess prognosis using: severity of dyspnea, FVC and DLCO values, oxygen saturation at end of 6-minute walk test, extent of honeycombing on HRCT, and signs of pulmonary hypertension on echocardiography 1
• Calculate GAP (gender, age, physiology) score for 1-, 2-, and 3-year survival estimates 1

Ongoing Monitoring

• Repeat PFTs every 3-6 months to detect progression, defined as ≥10% decline in FVC or ≥15% decline in DLCO over 6 months 1, 6
• Monitor symptoms at each visit for worsening dyspnea or cough 6, 5
• Repeat HRCT when clinically indicated to assess for progression of fibrosis or development of complications 6, 5

Special Considerations in Elderly Patients

• Elderly patients (median age 66 years at IPF diagnosis) have increased risk of medication adverse effects, particularly gastrointestinal symptoms (nausea, diarrhea, anorexia) with both pirfenidone and nintedanib 2
• Polypharmacy is common in elderly patients and requires careful medication reconciliation 2
• Surgical lung biopsy carries higher risk in elderly patients, and diagnostic confidence may be sufficient without biopsy in patients >70 years with typical HRCT findings 2
• Early palliative care discussions should be incorporated into management planning 2