Interstitial Lung Disease Viva

Long Case Viva Defence

Candidate's opening statement (model answer)

"Mr Thompson is a 70-year-old retired electrician who presents with 10 months of progressive exertional dyspnoea and dry cough. He is breathless walking 80 metres on flat ground and has recently noticed his fingers changing shape. ^[6]

"His past history includes hypertension and GERD. He is a former smoker with a 45 pack-year history, having quit 8 years ago. He has no known occupational lung disease exposures and takes no drugs associated with ILD. ^[6]

"On examination he has digital clubbing and bilateral basal fine Velcro-like crackles. He is not cyanosed. His oxygen saturation is 94 percent on room air at rest, desaturating to 87 percent on exertion. There is no evidence of cor pulmonale. ^[6]

"His HRCT shows basal and subpleural reticulation with honeycombing and traction bronchiectasis, without features suggesting an alternative diagnosis. Martinez UIP is bilateral, peripheral, basal reticular change with traction bronchiectasis and clusters of subpleural cystic airspaces; identify UIP usually on HRCT. His PFTs confirm a restrictive defect with FVC 56 percent predicted, TLC 60 percent predicted, DLCO 34 percent predicted. His autoimmune screen is negative. His echocardiogram shows estimated RVSP 45 mmHg with normal right ventricular function. ^[6]

"His main problems are:

1. Idiopathic pulmonary fibrosis — UIP-pattern HRCT after exclusion of other ILDs, progressive physiology. Ley: overall poor prognosis. GAP uses gender, age, FVC and DLCO; 1-year mortality 6 / 16 / 39 percent. Mapping this man to “GAP Stage II, 11 / 29 percent” is exam convention, not the Ley abstract.
2. Exertional desaturation requiring oxygen assessment — named PaO2 / SpO2 bands are service convention
3. Possible pulmonary hypertension (RVSP 45 mmHg) — a sourced RVSP cut-off is not in the cited abstracts
4. GERD — comorbidity. ATS 2022 is conditionally against antacid medication and antireflux surgery for treating IPF
5. Former heavy smoker
6. Progressive functional decline despite no prior therapy" ^[6][8][1]

Examiner probing questions and model answers

Q1: "How confident are you in the diagnosis of IPF, and would you biopsy?" ^[1][6]

"I am reasonably confident after MDT review. Martinez: diagnosis excludes other ILDs or overlapping conditions and depends on identifying the UIP pattern, usually with HRCT; lung biopsy might be required in some patients. ATS 2022 updated radiological and histopathological criteria by consensus. The four-level 2018 “definite UIP, no biopsy” table is exam convention, not a 2022 abstract. His HRCT matches Martinez UIP — bilateral, peripheral, basal reticular changes with traction bronchiectasis and clusters of subpleural cystic airspaces. Autoimmune screen, drugs, occupation and antigen history are negative. ATS 2022 conditionally regards transbronchial lung cryobiopsy as an acceptable alternative to surgical lung biopsy in centres with appropriate expertise — I would not mandate a surgical biopsy here, and I would not invent a sourced FVC/DLCO biopsy-contraindication cut-off from these abstracts. The MDT should confirm." ^[1][6]

Q2: "What is your first pharmacological priority?" ^[2][3][6]

"Initiating an antifibrotic. Martinez: pirfenidone and nintedanib decrease physiological progression and likely improve progression-free survival. I would offer nintedanib 150 mg twice daily (INPULSIS) or pirfenidone 2403 mg per day (ASCEND, 555 patients, 52 weeks). Do not quote a uniform “50 percent FVC reduction”. INPULSIS (1066 patients, 3:2): annual FVC −114.7 versus −239.9 mL (trial 1) and −113.6 versus −207.3 mL (trial 2). Acute-exacerbation HR 1.15 NS versus 0.38 — not a uniform exacerbation claim. Diarrhoea about 62 versus 18 percent; discontinuation less than 5 percent. A 100 mg BID reduction is tolerability convention, not an INPULSIS arm. ASCEND: 47.9 percent relative reduction in a 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. CAPACITY used 801 mg or 399 mg three times a day; week-by-week 267/534/801 titration is exam convention. Choice is tolerability — there is no head-to-head superiority trial. ATS 2022 is conditionally against antacids and antireflux surgery for treating IPF." ^[2][3][1][6]

Q3: "Would you use corticosteroids?" ^[7]

"No. Combination immunosuppression is contraindicated in IPF. PANTHER-IPF: 77 versus 78; 8 versus 1 deaths and 23 versus 7 hospitalisations; the DSMB stopped the combination arm at a mean 32 weeks. ATS 2022’s abstract asked cryobiopsy, genomic classifier, antacids and antireflux — not steroid monotherapy — so I do not cite 2022 as the steroid paper. The sourced harm signal is PANTHER." ^[7][1]

Q4: "His FVC declines by 12 percent over the next 6 months despite nintedanib. What do you do?" ^[1][4]

"First exclude a reversible cause of decline — new infection, volume overload, or an acute exacerbation (Kishaba: heart failure and volume overload). Repeat HRCT. If this is progression of fibrosis: (1) confirm he is on nintedanib 150 mg twice daily, or consider switching to pirfenidone 2403 mg per day if diarrhoea is limiting — do not escalate nintedanib to 300 mg BID; (2) refer early for transplant assessment — named FVC less-than-80 / DLCO less-than-40 cut-offs are exam convention, not numbers in these abstracts; (3) oxygen for documented hypoxaemia; (4) advance-care planning. ATS 2022 PPF is at least two of three criteria within the past year in an ILD other than IPF — he already has IPF, so do not relabel him PPF. INBUILD’s 24-month window is eligibility, not the ATS PPF definition." ^[1][4][9]

Q5: "He presents acutely with worsening dyspnoea over 5 days, fever, and new bilateral ground-glass on HRCT. What is this and how do you manage it?" ^[5][9]

"This may be an acute exacerbation of IPF. Collard 2016: an acute, clinically significant respiratory deterioration of unidentifiable cause; the working group proposed a revised definition and diagnostic criteria. The four-item 1-month / new-GGO checklist that circulates in exams is not enumerated in that abstract. Kishaba: the 2016 International Working Group divides AE-IPF into triggered and idiopathic; heart failure and volume overload are key differentials — echo and volume status first, then infection. Pulse methylprednisolone 0.5–1 g for 3 days, in-hospital mortality greater than 50 percent, and a mechanical-ventilation mortality figure are not in the Collard or Kishaba abstracts and are not taught as sourced numbers. Management is supportive oxygen, treat identified infection, continue the antifibrotic unless drug toxicity is suspected, and discuss goals of care. Do not start PANTHER combination immunosuppression." ^[5][9][7]

Q6: "When would you refer him for lung transplantation?" ^[1][8]

"Now — early, not when he is in end-stage failure. Transplantation can replace the fibrotic lung. Named FVC / DLCO referral cut-offs, a 5-year 50–60 percent survival, BMI 35, and an age-70 ceiling are not in the cited abstracts. Ley: overall poor prognosis; GAP 1-year 6 / 16 / 39 percent. The workup takes months; late referral is the common failure." ^[1][8]