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Interstitial Lung Disease — Written Clinical Reasoning
DCE long-case preparation: structured written reasoning for interstitial lung disease management, including problem-list synthesis, HRCT interpretation, investigation interpretation, and integrated management planning for IPF and CTD-ILD.
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SAQ 1 — Integrated Management (20 marks, 30 minutes)
Prompt: Outline your integrated management plan for this patient, including the diagnostic conclusion, pharmacological therapy, non-pharmacological measures, prognostic assessment, and surveillance plan. Justify each decision with reference to evidence. [1]
Model Answer
Diagnostic conclusion (2 marks): [1][7]
The working diagnosis is idiopathic pulmonary fibrosis (IPF). Martinez: the UIP pattern is predominantly bilateral, peripheral and basal reticular changes with traction bronchiectasis and clusters of subpleural cystic airspaces; identify UIP usually on HRCT after excluding other ILDs. ATS 2022 updates prior IPF guidelines by consensus and is an MDT diagnosis. The four-level “definite UIP, no biopsy” table is exam convention, not a 2022 abstract. [7][1]
Problem list (3 marks): [1]
- Idiopathic pulmonary fibrosis — UIP-pattern HRCT, moderate-severe physiological impairment (FVC 58 percent, DLCO 36 percent), progressive
- Exertional desaturation (6MWT desaturation to 86 percent) — oxygen assessment; named PaO2 / SpO2 bands are service convention
- Possible pulmonary hypertension (estimated RVSP 42 mmHg) — monitor; a sourced RVSP cut-off is not in the cited abstracts
- GERD — comorbidity. ATS 2022 is conditionally against antacid medication and antireflux surgery for the treatment of IPF — do not prescribe a PPI as IPF therapy
- Hypertension — cardiovascular comorbidity
- Former smoker (50 pack-years) [1]
Pharmacological management (5 marks): [2][3][6]
| Therapy | Dose | Rationale |
|---|---|---|
| Nintedanib | 150 mg twice daily (INPULSIS) | INPULSIS (1066 patients, 3:2): annual FVC −114.7 versus −239.9 mL (trial 1) and −113.6 versus −207.3 mL (trial 2). AE HR 1.15 NS versus 0.38 — do not quote a uniform exacerbation or “50 percent / 110 mL” reduction. Diarrhoea ~62 versus ~18 percent; discontinuation less than 5 percent. A 100 mg BID reduction is tolerability convention, not an INPULSIS arm. |
| OR Pirfenidone | 2403 mg per day (ASCEND; CAPACITY used 801 mg or 399 mg three times a day) | ASCEND (n=555, 52 weeks): 47.9 percent relative reduction in an absolute 10-point FVC decline or death; death not significant (P=0.10). Do not teach a pooled ASCEND+CAPACITY mortality benefit as an ASCEND result. Week-by-week 267/534/801 titration is exam convention. |
| Not PPI for IPF | — | ATS 2022 conditionally against antacids and antireflux surgery for treating IPF. |
Choice is tolerability and preference — there is no head-to-head superiority trial. Both decrease physiological progression (Martinez). [7]
Key teaching point: Combination immunosuppression is contraindicated. PANTHER-IPF: 77 versus 78; 8 versus 1 deaths and 23 versus 7 hospitalisations; stopped at a mean 32 weeks. [6]
Non-pharmacological management (4 marks): [1]
- Oxygen: correct hypoxaemia. Named PaO2 less-than-55 mmHg / SpO2 88 percent / 2–4 L/min bands are BTS/NICE service convention, not trial end-points here. Do not teach LTOT as a sourced survival end-point from these papers.
- Pulmonary rehabilitation: standard supportive care. A 6-to-8-week duration is exam convention.
- Vaccinations: influenza, pneumococcal, COVID-19 — infection can trigger AE-IPF (Kishaba: triggered versus idiopathic).
- Smoking cessation. [1]
Prognostic assessment (2 marks): [5]
Ley: IPF has an overall poor prognosis. The GAP index uses gender (G), age (A), and two lung physiology variables (P) (FVC and DLCO). Three stages had 1-year mortality of 6 percent, 16 percent and 39 percent. The point-score table that maps this man to “Stage II, 11 percent / 29 percent” is not in the Ley abstract and is labelled exam convention. Exam 3–5 year median survival is likewise not in the Martinez/Ley abstracts. [5][7]
Transplant referral (2 marks): [1]
Refer early. Named FVC less-than-80-percent or DLCO less-than-40-percent referral cut-offs and 5-year 50–60 percent survival are not in the cited abstracts. The workup takes months; late referral is the common failure. [1]
Surveillance and follow-up (2 marks): [1]
- PFTs every 3–6 months (FVC trajectory)
- Interval HRCT if decline or suspected exacerbation
- 6-minute walk test
- Echocardiography for pulmonary hypertension screening
- ILD nurse coordinator
- Early advance care planning [1]
References9ShowHide
- [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]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]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]Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases N Engl J Med, 2019.PMID 31566307
- [5]Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis Ann Intern Med, 2012.PMID 22586007
- [6]Raghu G, Anstrom KJ, King TE Jr Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis N Engl J Med, 2012.PMID 22607134
- [7]Martinez FJ, Collard HR, Pardo A, et al. Idiopathic pulmonary fibrosis Nat Rev Dis Primers, 2017.PMID 29052582
- [8]Distler O, Highland KB, Gahlemann M, et al. Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease N Engl J Med, 2019.PMID 31112379
- [9]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