Abstract

JIMENEZ, Karen V.; MOSQUERA, Catalina  and  QUINTERO, Edna Margarita. Bladder involvement in a paediatric patient with polyarteritis nodosa: First description. Rev.Colomb.Reumatol. [online]. 2020, vol.27, n.2, pp.141-146.  Epub Apr 24, 2021. ISSN 0121-8123.  https://doi.org/10.1016/j.rcreu.2019.05.004.

Polyarteritis nodosa (PAN) is a necrotising vasculitis, rare in childhood, and characterized by the inflammation of small and medium vessels and multiple organ involvement. The case is presented of a 4 year old girl with prolonged febrile syndrome, chronic abdominal pain, disabling myalgia, and skin involvement. After 2years of symptoms, she met clinical criteria for PAN. She received treatment with oral and intravenous systemic corticosteroids, 6 months of intravenous cyclophosphamide and maintenance with conventional immuno-suppressants without an adequate response. However, she showed clinical improvement with oral cyclophosphamide and long-term corticosteroids. She had several relapses during follow-up visits due to irregular treatment requiring a high cumulative dose of cyclophosphamide. Five years later she presented with macroscopic haematuria, and was assessed for, among other causes, cyclophosphamide toxicity and disease activity. The workup included cystoscopy and bladder biopsy with findingof necrotising vasculitis of bladder wall. Bladder vasculitis is rarely reported in the literature (3-5 cases in adults) and in that consulted there are no reports in children. To our knowledge, this is the first case of bladder involvement associated with systemic vasculitis reported in the paediatric age.

Keywords : Haematuria; Necrotising vasculitis; Polyarteritis nodosa; Cyclophosphamide; Bladder.

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