Interstitial Lung Disease — Viva

Q1: Define ILD and describe the restrictive pattern on PFTs (2 min)

• ILD (DPLD) — a heterogeneous group of over 200 disorders causing inflammation and/or fibrosis of the lung interstitium (alveolar walls, septa, peribronchovascular and perilymphatic spaces), producing a restrictive ventilatory defect with diffuse imaging abnormality, after excluding infection, malignancy, and cardiac failure.
• Restrictive pattern on PFTs — low TLC, low FVC, normal or raised FEV1/FVC (mirror image of obstructive), and reduced DLCO (often the earliest abnormality). The "TLC below 80 percent predicted" cut-off is exam convention, not a number from the cited abstracts.
• Reduced compliance → low lung volumes; thickened alveolar–capillary membrane and capillary loss → low DLCO.^[6]

Q2: How is IPF diagnosed? (3 min)

• Multidisciplinary diagnosis, no single test in isolation. Martinez: exclude other ILDs or overlapping conditions and identify the UIP pattern, usually with HRCT; lung biopsy might be required in some patients.
• Clinical context — often elderly men who smoke (Kishaba); progressive exertional dyspnoea, dry cough, basal Velcro crackles, clubbing.
• UIP on HRCT (Martinez) — predominantly bilateral, peripheral and basal reticular changes associated with traction bronchiectasis and clusters of subpleural cystic airspaces.
• ATS/ERS/JRS/ALAT 2022 — radiological and histopathological criteria for IPF were updated by consensus. Transbronchial lung cryobiopsy is a conditional acceptable alternative to surgical lung biopsy in centres with appropriate expertise.
• Exclude known causes — CTD serology, drugs (amiodarone, bleomycin, methotrexate, nitrofurantoin, checkpoint inhibitors, targeted agents, ADCs), occupation, hypersensitivity (known antigen + assemblage of findings).
• The older four-item "definite UIP / probable UIP / indeterminate" checklist is exam convention; it is not enumerated in the 2022 abstract.^[1][6][10]

Q3: Discuss the management of IPF, including two antifibrotics with sourced doses (3 min)

• MDT-led management. Treat an identified cause (antigen avoidance in HP, drug withdrawal, CTD immunosuppression).
• Pirfenidone — ASCEND: 2403 mg per day for 52 weeks (555 patients). 47.9 percent relative reduction in ≥10-point FVC decline or death; 132.5 percent relative increase in no FVC decline; 6MWT P=0.04; PFS P less than 0.001; death NS (P=0.10). GI and skin AEs more common; rarely discontinue. Do not teach the unsourced 200/400/600 mg TDS titration as if it were ASCEND.^[3]
• Nintedanib — 150 mg twice daily (INPULSIS, INBUILD, SENSCIS). INPULSIS: FVC −114.7 vs −239.9 mL and −113.6 vs −207.3 mL. Diarrhoea about 62 percent vs about 18 percent; discontinuation less than 5 percent. Do not escalate to 300 mg BID. Acute-exacerbation effect was not uniform (INPULSIS-1 HR 1.15 NS; INPULSIS-2 HR 0.38).^[2]
• DO NOT use prednisone + azathioprine + NAC (PANTHER: 8 vs 1 deaths, 23 vs 7 hospitalisations, stopped at mean 32 weeks).^[7]
• DO NOT treat IPF with antacids or antireflux surgery (ATS 2022 against).^[1]
• Oxygen for hypoxaemia; named PaO₂ ≤55 / SpO₂ ≤88 / 15 h/day thresholds are BTS/NICE convention, not trial end-points here.
• Lung transplantation — refer early; the only way to replace the fibrotic lung.
• Palliative care integrated early.^[6]

Q4: Prognosis and complications of IPF (2 min)

• Ley: IPF has an overall poor prognosis. Exam texts quote median survival 3–5 years; that band is not in the cited abstracts.
• GAP index — Gender, Age, FVC, DLCO. Stages I–III 1-year mortality 6, 16 and 39 percent. The point table and 3-year 16/48/62 bands are not in the Ley abstract.
• Acute exacerbation — IWG 2016 triggered versus idiopathic; exclude heart failure and volume overload. In-hospital 50 percent / 3–4 month figures dropped as sourced numbers.
• Pulmonary hypertension (group 3) and cor pulmonale — poor prognostic overlay when present.
• Lung cancer may coexist; a sourced 3–7-fold relative risk is not in the cited abstracts.
• Respiratory failure is the usual mode of death.^[14][10]

Q5: Distinguish IPF from chronic hypersensitivity pneumonitis and NSIP on imaging (2 min)

• IPF (UIP) — bilateral, peripheral, basal reticular changes, traction bronchiectasis, clusters of subpleural cystic airspaces (Martinez).
• NSIP — originally a pathology diagnosis, now multidisciplinary (Hino). Subacute/chronic HP can mimic NSIP and UIP.
• HP — repeated organic-particle exposure; acute/subacute/chronic forms overlap. HRCT: ground-glass and poorly defined nodules with patchy air trapping in acute/subacute disease; reticular opacities, volume loss and traction bronchiectasis in chronic disease. Diagnosis needs a known antigen plus the assemblage of findings. Avoidance is the key; steroids are generally used, long-term efficacy unproven. BAL CD4/CD8 cut-offs are not in the sourced review.^[6][9][13]

Q6: Name four drugs that cause ILD and one characteristic of each (1 min)

• Amiodarone, bleomycin, methotrexate, nitrofurantoin — classical exam causes; stop the drug. Sourced amiodarone 33 percent / bleomycin 3–25 percent mortality figures are not in the Naraoka review.
• Molecular targeted agents, immune checkpoint inhibitors, and antibody-drug conjugates — modern DIILD; toxicity can significantly affect outcomes (Naraoka).
• Diagnosis — systematic exclusion with multidisciplinary team consensus; vigilant monitoring and early detection.
• Checkpoint inhibitors — immune-related pneumonitis; MDT exclusion diagnosis, not a BAL-ratio diagnosis.^[12]

References14ShowHide

1. [1]Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline Am J Respir Crit Care Med, 2022.PMID 35486072
2. [2]Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis N Engl J Med, 2014.PMID 24836310
3. [3]King TE Jr, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis N Engl J Med, 2014.PMID 24836312
4. [4]Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases N Engl J Med, 2019.PMID 31566307
5. [5]Distler O, Highland KB, Gahlemann M, et al. Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease N Engl J Med, 2019.PMID 31112379
6. [6]Martinez FJ, Collard HR, Pardo A, et al. Idiopathic pulmonary fibrosis Nat Rev Dis Primers, 2017.PMID 29052582
7. [7]Raghu G, Anstrom KJ, King TE Jr Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis N Engl J Med, 2012.PMID 22607134
8. [8]Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial Lancet Respir Med, 2016.PMID 27469583
9. [9]Selman M, Pardo A, King TE Jr Hypersensitivity pneumonitis: insights in diagnosis and pathobiology Am J Respir Crit Care Med, 2012.PMID 22679012
10. [10]Kishaba T Acute Exacerbation of Idiopathic Pulmonary Fibrosis Medicina (Kaunas), 2019.PMID 30884853
11. [11]Sakthivel MK, Hazelton TR, Askin FB, et al. Organizing Pneumonia Phenotype Semin Roentgenol, 2026.PMID 41513514
12. [12]Naraoka T, Sumi T Management of drug-induced interstitial lung disease in the era of evolving lung cancer therapies: A mini-review Respir Investig, 2026.PMID 42520532
13. [13]Hino T, Lee KS, Yoo H, et al. Interstitial lung abnormality (ILA) and nonspecific interstitial pneumonia (NSIP) Eur J Radiol Open, 2021.PMID 33796637
14. [14]Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis Ann Intern Med, 2012.PMID 22586007