title: Granulomatosis with polyangiitis mimicking infective endocarditis in an adolescent male.
abstract: Granulomatosis with polyangiitis (GPA) is a rare but serious small vessel vasculitis with heterogeneous clinical presentation ranging from mainly localised disease with a chronic course, to a florid, acute small vessel vasculitic form characterised by severe pulmonary haemorrhage and/or rapidly progressive vasculitis or other severe systemic vasculitic manifestations. Cardiac involvement is, however, uncommon in the paediatric population. We report a case of a 16-year-old male who presented with peripheral gangrene and vegetation with unusual location on the supporting apparatus of the tricuspid valve, initially considered to have infective endocarditis but ultimately diagnosed with GPA. We provide an overview of the limited literature relating to cardiac involvement in GPA, and the diagnostic challenge relating to infective endocarditis in this context, especially focusing on the interpretation of the antineutrophil cytoplasmic antibody (ANCA) and the characteristic clinical features to identify in order to promptly recognise GPA, since timely diagnosis and treatment are essential for this potentially life-threatening condition.
authors.affiliation: Department of Rheumatology, Institute of Child Health, Great Ormond Street Hospital, 30 Guilford Street, London, WC1N 1EH, UK. giuliavarnier@yahoo.it.
authors.forename: Giulia Camilla
authors.initials: GC
authors.lastname: Varnier
authors.affiliation: UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, UK.
authors.forename: Neil
authors.initials: N
authors.lastname: Sebire
authors.affiliation: UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, UK.
authors.forename: Georgi
authors.initials: G
authors.lastname: Christov
authors.affiliation: UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, UK.
authors.forename: Despina
authors.initials: D
authors.lastname: Eleftheriou
authors.affiliation: UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, UK.
authors.forename: Paul A
authors.initials: PA
authors.lastname: Brogan
mesh_terms: D000293:Adolescent; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D004696:Endocarditis / Q000175:diagnosis* / Q000276:immunology; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D053146:Myeloblastin / Q000276:immunology; D010951:Plasma Exchange; D011241:Prednisone / Q000627:therapeutic use; D000069283:Rituximab / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D000069283:Rituximab; D053146:Myeloblastin; D011241:Prednisone; D008775:Methylprednisolone
keywords: ANCA-associated vasculitis; Adolescent; Granulomatosis with polyangiitis; Infective endocarditis