Pulmonary vasculitis flashcards
15 high-yield MRCP Part 1 cards from the Intellect Medicos Respiratory Medicine course. Try to answer each question before you open it — then study them properly with spaced repetition, which brings each card back just before you would forget it.
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Which ANCA pattern and target antigen are typical of granulomatosis with polyangiitis (GPA)?
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c-ANCA directed against proteinase 3 (PR3).
Why? Most patients with active generalised GPA (roughly 80–90%) are c-ANCA/PR3 positive.
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Which three organ systems make up the classic triad of GPA?
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Upper respiratory tract (ENT), lungs and kidneys.
Why? ENT: sinusitis, crusting, epistaxis, saddle-nose deformity. Lungs: cavitating nodules. Kidneys: rapidly progressive (pauci-immune) GN.
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What is the induction treatment for organ-threatening GPA?
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High-dose corticosteroids plus rituximab or cyclophosphamide.
Why? This combination induces remission. Maintenance uses azathioprine, methotrexate or rituximab.
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In ANCA vasculitis, in which presentations may plasma exchange be added to induction therapy?
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Severe, organ-threatening disease: rapidly progressive renal failure (e.g. creatinine > 300 µmol/L) or diffuse alveolar haemorrhage with hypoxaemia.
Why? Plasma exchange removes circulating antibodies quickly. After the PEXIVAS trial it is no longer routine and is considered case by case.
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What is the mechanism of action of rituximab?
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Monoclonal antibody against CD20 on B cells (depletes B cells).
Why? Removing B cells reduces ANCA production; it is as effective as cyclophosphamide for remission induction.
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Which bladder complication does cyclophosphamide cause, and which drug reduces the risk?
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Haemorrhagic cystitis; mesna (plus good hydration) reduces the risk.
Why? The metabolite acrolein irritates the bladder; mesna binds it. Long-term risk includes bladder cancer.
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What is the gold-standard investigation to confirm GPA?
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Tissue biopsy (e.g. renal or nasal), showing necrotising granulomatous vasculitis.
Why? Renal biopsy shows pauci-immune crescentic glomerulonephritis.
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Which clinical feature is present in almost all patients with eosinophilic granulomatosis with polyangiitis (EGPA) and usually comes first?
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Asthma (usually adult-onset or worsening).
Why? EGPA = asthma + marked eosinophilia + vasculitis (e.g. mononeuritis multiplex). p-ANCA (MPO) is positive in only about 40%.
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Which asthma drug class has been linked with unmasking EGPA?
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Leukotriene receptor antagonists (e.g. montelukast).
Why? EGPA may appear when montelukast allows oral steroids to be tapered, unmasking the vasculitis.
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Which biologic is used for relapsing or steroid-dependent EGPA?
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Mepolizumab (anti-IL-5).
Why? Blocking IL-5 reduces eosinophils, allowing steroid sparing.
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Which organ involvement is the main poor prognostic marker in EGPA?
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Cardiac involvement (myocarditis, cardiomyopathy).
Why? Heart involvement is the leading cause of death in EGPA.
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How does microscopic polyangiitis (MPA) differ from GPA?
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MPA is p-ANCA (MPO) positive with NO granulomas and no destructive ENT disease or cavitating nodules.
Why? It is a pulmonary–renal syndrome: alveolar haemorrhage plus pauci-immune crescentic GN.
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What is the target antigen in anti-GBM (Goodpasture's) disease?
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The alpha-3 chain of type IV collagen (in glomerular and alveolar basement membranes).
Why? Antibodies attack both kidney and lung, causing rapidly progressive GN and pulmonary haemorrhage. Smoking is a key trigger.
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What does renal biopsy immunofluorescence show in anti-GBM disease?
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Linear IgG deposition along the glomerular basement membrane.
Why? ANCA vasculitis, by contrast, is pauci-immune (little or no immune deposit).
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What is the treatment for anti-GBM disease?
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Plasma exchange plus high-dose corticosteroids plus cyclophosphamide.
Why? Plasma exchange removes the pathogenic antibody quickly. Prognosis is poor if dialysis-dependent or creatinine > 500 µmol/L at presentation.