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Paeds Casesrheumatology-musculoskeletal-and-sports

Paeds Cases · rheumatology-musculoskeletal-and-sports

Explain a new diagnosis of granulomatosis with polyangiitis and the long-term plan — OSCE

OSCE communication station: explaining a new diagnosis of granulomatosis with polyangiitis to a 14-year-old boy and his parents after a pulmonary-renal presentation, addressing the diagnosis, the mechanism, the treatment plan, the side effects of the immunosuppression, the relapse risk, and the long-term surveillance and transition.

osce communication and explanation3 min readVerification in progress

Target exams

MRCPCH ClinicalRACP DCERCPSC Pediatrics
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Study tools

Target exams

MRCPCH ClinicalRACP DCERCPSC Pediatrics
Prompt
A 14-year-old boy and his parents are in the paediatric ward two days after admission for bloody nasal discharge, a septal ulcer, a cough, and cola-coloured urine. The PR3 (c-ANCA) is positive and the renal biopsy shows a crescentic pauci-immune glomerulonephritis. The diagnosis of granulomatosis with polyangiitis has been made, and the induction therapy with rituximab and glucocorticoids is about to start. The parents have read online that the disease causes kidney failure and is lifelong, and the boy is worried about the side effects of the steroids. Explain the diagnosis, the treatment plan, the side effects and the long-term outlook.

Candidate brief

You have eight minutes to explain a new diagnosis of granulomatosis with polyangiitis to a 14-year-old boy and his parents. He presented with three weeks of bloody nasal discharge, a septal ulcer, a cough and cola-coloured urine. The PR3 (c-ANCA) is positive and the renal biopsy shows a crescentic pauci-immune glomerulonephritis. The induction therapy with rituximab and glucocorticoids is about to start. The parents have read online that the disease causes kidney failure and is lifelong, and the boy is worried about the steroid side effects. Use a structured, honest, empathic approach that names the diagnosis, explains the mechanism in plain language, addresses the online fears and the steroid worry, and builds a clear treatment and surveillance plan. [7][6]

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References4ShowHide
  1. [3]Jennette JC, Falk RJ, Bacon PA, et al 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum, 2013.PMID 23045170
  2. [6]de Graeff N, Groot N, Brogan P, et al European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides - the SHARE initiative. Rheumatology (Oxford), 2019.PMID 30535249
  3. [7]Cabral DA, Canter DL, Muscal E, et al Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study. Arthritis Rheumatol, 2016.PMID 27111558
  4. [10]Pop AA, Bot Rachisan AL, Botan E, et al Renal Involvement in Pediatric Small-Vessel Vasculitis: A Comprehensive Review of Clinical Impact, Diagnosis, and Management. Med Sci (Basel), 2026.PMID 42346872
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