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Paeds Casesrheumatology-musculoskeletal-and-sports

Paeds Cases · rheumatology-musculoskeletal-and-sports

Counsel a family on CVID arthritis and the need for a dual plan — OSCE

OSCE communication and shared-planning station: explaining to a family that their daughter's arthritis and bruising are rheumatic fingerprints of a common variable immunodeficiency rather than ordinary juvenile idiopathic arthritis, that the immune defect must be confirmed before any biologic is started, that immunoglobulin replacement will control infection susceptibility but not the arthritis, and that the plan is to treat both the immune defect and the rheumatic manifestation in parallel with shared care between immunology and rheumatology.

osce communication and shared decision-making1 min readVerification in progress

Target exams

MRCPCH ClinicalRACP DCERCPSC Pediatrics
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Target exams

MRCPCH ClinicalRACP DCERCPSC Pediatrics
Prompt
The parents of a six-year-old girl have been told her three-month wrist and knee arthritis is juvenile idiopathic arthritis and that she will start a tumour-necrosis-factor inhibitor next week. They are worried because she has also had two pneumonias, chronic diarrhoea, and unexplained bruising, and her immunoglobulins have just come back low. They are frightened the new medicine will make her sicker and confused about why her joints and her immune system seem linked. Counsel them.

Candidate brief

You have eight minutes to counsel the parents of a six-year-old girl in whom a rheumatic-immune overlap — common variable immunodeficiency with arthritis, chronic diarrhoea, and autoimmune thrombocytopenia — is suspected alongside a plan to start a biologic for presumed juvenile idiopathic arthritis. Use a structured, honest, empathic approach. [3] [8]

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References4ShowHide
  1. [1]Bousfiha A, Moundir A, Tangye SG, et al. The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity. J Clin Immunol, 2022.PMID 36198931
  2. [3]Köstel Bal S, Pazmandi J, Boztug K, et al. Rheumatological manifestations in inborn errors of immunity. Pediatr Res, 2020.PMID 31581173
  3. [7]Odnoletkova I, Kindle G, Quinti I, et al. The burden of common variable immunodeficiency disorders: a retrospective analysis of the European Society for Immunodeficiency (ESID) registry data. Orphanet J Rare Dis, 2018.PMID 30419968
  4. [8]Ameratunga R, Gillis D, Steele R. Diagnostic criteria for common variable immunodeficiency disorders. J Allergy Clin Immunol Pract, 2016.PMID 27587325
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