ANCA Vasculitis — SCE Rheumatology MCQ
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Correct answer: E — Diffuse alveolar haemorrhage causing acute hypoxaemic respiratory failure
The immediate threat is diffuse alveolar haemorrhage causing severe acute hypoxaemic respiratory failure. The haemoptysis, falling haemoglobin, bilateral infiltrates and profound hypoxaemia establish pulmonary capillaritis with life-threatening alveolar bleeding, requiring immediate critical-care respiratory support and urgent AAV remission induction. The rapidly progressive glomerulonephritis is also organ-threatening, but creatinine alone is not an indication for emergency renal replacement therapy; potassium, pH and bicarbonate are acceptable, with no refractory pulmonary oedema or uraemic pericarditis. Subglottic stenosis and orbital inflammation can become emergencies, but neither is present here. Saddle-nose deformity reflects accumulated structural damage rather than acute disease. Current UK guidance supports high-dose glucocorticoids with rituximab or cyclophosphamide; combined rituximab and cyclophosphamide may be considered in selected life-threatening disease but is not universally required.
Reference: Fussner LA et al. Alveolar Hemorrhage in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Results of an International Randomized Controlled Trial (PEXIVAS). American Journal of Respiratory and Critical Care Medicine. 2024;209:1141-1151. https://pubmed.ncbi.nlm.nih.gov/38346237/