Idiopathic Pulmonary Fibrosis Severe Restrictive Pattern MCQ — NEET PG Practice Question | NEETPGAI
Idiopathic Pulmonary Fibrosis Severe Restrictive Pattern
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stethoscope Medicine
A 68-year-old man with a 2-year history of progressive dyspnea and dry cough undergoes spirometry and HRCT chest. HRCT shows subpleural, basal-predominant reticulation with honeycombing and traction bronchiectasis, consistent with usual interstitial pneumonia (UIP) pattern. His spirometry reveals the pattern marked **A** in the diagram — severely reduced FVC at 42% predicted with a preserved FEV1/FVC ratio of 0.82. His DLCO is 28% predicted. What is the most appropriate next step in management?
A. Start oral corticosteroids and arrange repeat spirometry in 3 months
B. Initiate pirfenidone or nintedanib and refer for lung transplantation evaluation
C. Prescribe inhaled corticosteroids and schedule follow-up in 6 months
D. Observe without pharmacotherapy and manage with supplemental oxygen alone
Explanation
Why "Initiate pirfenidone or nintedanib and refer for lung transplantation evaluation" is right
The spirometric pattern marked A — FVC <50% predicted with preserved FEV1/FVC ratio — is the hallmark restrictive pattern of idiopathic pulmonary fibrosis (IPF) and defines SEVERE disease stage. According to the ATS/ERS/JRS/ALAT 2022 IPF Guideline and Harrison's 21e, an FVC <50% predicted (or DLCO <40%, or rapid decline >10% in 6 months) signals advanced disease and mandates IMMEDIATE LUNG TRANSPLANTATION EVALUATION. Simultaneously, antifibrotic therapy with pirfenidone or nintedanib must be initiated to slow FVC decline by ~50%, as both are now standard of care for all IPF patients regardless of disease stage. This patient's markedly reduced DLCO (28% predicted) further confirms advanced physiology and poor prognosis without transplant.
Why each distractor is wrong
Prescribe inhaled corticosteroids and schedule follow-up in 6 months: Inhaled corticosteroids have no proven benefit in IPF and delay initiation of evidence-based antifibrotic therapy. An FVC <50% is too advanced for observation; 6-month follow-up risks disease progression beyond transplant candidacy.
Start oral corticosteroids and arrange repeat spirometry in 3 months: Systemic corticosteroids are not indicated for stable IPF and may worsen outcomes. They are reserved only for acute exacerbations (rapid worsening with new ground-glass opacities). Delaying antifibrotic therapy and transplant evaluation in severe disease is inappropriate.
Observe without pharmacotherapy and manage with supplemental oxygen alone: Observation without antifibrotic therapy in FVC <50% disease is contraindicated. Supportive oxygen alone does not slow disease progression. The 2022 guideline mandates early antifibrotic initiation and transplant evaluation at this severity threshold.
High-YieldNEET PG
FVC <50% predicted in IPF = SEVERE stage → initiate antifibrotic (pirfenidone or nintedanib) + refer for lung transplantation evaluation immediately; DLCO <40% is the earliest and most sensitive abnormality.
ATS/ERS/JRS/ALAT IPF Guideline 2022; Harrison's Principles of Internal Medicine, 21e
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