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Pulmonary-renal syndrome — RACP Paediatrics MCQ

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HardRespiratoryPulmonary-renal syndromeRACP Paediatrics

A 16-year-old has diffuse alveolar haemorrhage, rapidly rising creatinine, circulating anti-GBM antibodies and red-cell casts. Kidney biopsy is scheduled tomorrow, but the respiratory status is worsening. What treatment strategy is most appropriate now?

Educational content. Not a substitute for clinical judgement or local policy.

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Correct answer: C — Begin plasma exchange, glucocorticoids and cyclophosphamide before biopsy confirmation

Explanation lettering: E = shown as B · B = shown as E

C is correct because suspected anti-GBM disease with pulmonary haemorrhage and rapidly progressive glomerulonephritis requires immediate antibody removal and suppression of further antibody production; treatment should not await biopsy. D omits standard initial components. E confuses anti-GBM disease with an ANCA pathway. A forfeits salvageable lung and kidney tissue. B does not treat the immune mechanism. Biopsy remains valuable, but it proceeds without delaying therapy.

Reference: Royal Children’s Hospital Melbourne, Haematuria: https://www.rch.org.au/clinicalguide/guideline_index/Haematuria/; CARI Guidelines Australian commentary on KDIGO glomerular disease guidance: https://onlinelibrary.wiley.com/doi/10.1111/nep.70119; KDIGO 2021 Glomerular Diseases Guideline: https://kdigo.org/wp-content/uploads/2021/10/KDIGO-2021-Guideline-for-the-Management-of-Glomerular-Diseases.pdf