Marleen Bouhuys1, Wineke Armbrust2, Patrick F van Rheenen1. 1. Department of Pediatric Gastroenterology, Hepatology and Nutrition, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, Netherlands. 2. Department of Pediatric Rheumatology and Immunology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, Netherlands.
Abstract
Introduction: Small-vessel vasculitis (SVV) is a rare immunological disease that affects arterioles, capillaries and venules. It causes purpura, but can also manifest in other organs, including the gastrointestinal tract. SVV and inflammatory bowel disease (IBD) co-occur more frequently than would be expected by chance. Case description: A 16-year-old girl, who had been diagnosed with ulcerative colitis (UC) 2 years earlier at a general hospital, developed purpura, progressive abdominal pain with frequent bloody diarrhea and frontotemporal headache and swelling while on azathioprine and mesalamine maintenance therapy. Serology was positive for perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) without antiprotease- or myeloperoixidase antibodies. Endoscopy revealed active left-sided UC and atypical ulcerations in the ascending colon. Biopsies of these ulcerations and of affected skin revealed leukocytoclastic vasculitis. Initially this was interpreted as an extraintestinal manifestation of UC that would subside when remission was induced, consequently infliximab was started. Over the next 3 weeks she developed severe burning pain in her right lower leg that progressed to a foot drop with numbness and the purpura progressed to bullous lesions. The diagnosis was adjusted to ANCA-associated vasculitis with involvement of skin, bowel and peripheral nerves. Infliximab was discontinued and induction treatment with high-dose prednisolone and cyclophosphamide was given until remission of SVV and UC was achieved. Subsequently, infliximab induction and maintenance was re-introduced in combination with methotrexate. Remission has been maintained successfully for over 2 years now. The foot drop only partly resolved and necessitated the use of an orthosis. Conclusion: Pediatric patients with IBD who present with purpuric skin lesions and abdominal pain should be evaluated for systemic involvement of SVV, which includes endoscopic evaluation of the gastrointestinal tract. We discuss a practical approach to the diagnosis, evaluation and management of systemic SVV with a focus on prompt recognition and early aggressive therapy to improve outcome.
Introduction: Small-vessel vasculitis (SVV) is a rare immunological disease that affects arterioles, capillaries and venules. It causes purpura, but can also manifest in other organs, including the gastrointestinal tract. SVV and inflammatory bowel disease (IBD) co-occur more frequently than would be expected by chance. Case description: A 16-year-old girl, who had been diagnosed with ulcerative colitis (UC) 2 years earlier at a general hospital, developed purpura, progressive abdominal pain with frequent bloody diarrhea and frontotemporal headache and swelling while on azathioprine and mesalamine maintenance therapy. Serology was positive for perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) without antiprotease- or myeloperoixidase antibodies. Endoscopy revealed active left-sided UC and atypical ulcerations in the ascending colon. Biopsies of these ulcerations and of affected skin revealed leukocytoclastic vasculitis. Initially this was interpreted as an extraintestinal manifestation of UC that would subside when remission was induced, consequently infliximab was started. Over the next 3 weeks she developed severe burning pain in her right lower leg that progressed to a foot drop with numbness and the purpura progressed to bullous lesions. The diagnosis was adjusted to ANCA-associated vasculitis with involvement of skin, bowel and peripheral nerves. Infliximab was discontinued and induction treatment with high-dose prednisolone and cyclophosphamide was given until remission of SVV and UC was achieved. Subsequently, infliximab induction and maintenance was re-introduced in combination with methotrexate. Remission has been maintained successfully for over 2 years now. The foot drop only partly resolved and necessitated the use of an orthosis. Conclusion: Pediatric patients with IBD who present with purpuric skin lesions and abdominal pain should be evaluated for systemic involvement of SVV, which includes endoscopic evaluation of the gastrointestinal tract. We discuss a practical approach to the diagnosis, evaluation and management of systemic SVV with a focus on prompt recognition and early aggressive therapy to improve outcome.
Authors: Nienke de Graeff; Noortje Groot; Paul Brogan; Seza Ozen; Tadej Avcin; Brigitte Bader-Meunier; Pavla Dolezalova; Brian M Feldman; Isabelle Kone-Paut; Pekka Lahdenne; Stephen D Marks; Liza McCann; Clarissa Pilkington; Angelo Ravelli; Annet van Royen; Yosef Uziel; Bas Vastert; Nico Wulffraat; Sylvia Kamphuis; Michael W Beresford Journal: Rheumatology (Oxford) Date: 2019-04-01 Impact factor: 7.580
Authors: Mahmoud H Mosli; Guangyong Zou; Sushil K Garg; Sean G Feagan; John K MacDonald; Nilesh Chande; William J Sandborn; Brian G Feagan Journal: Am J Gastroenterol Date: 2015-05-12 Impact factor: 10.864
Authors: C Mukhtyar; R Lee; D Brown; D Carruthers; B Dasgupta; S Dubey; O Flossmann; C Hall; J Hollywood; D Jayne; R Jones; P Lanyon; A Muir; D Scott; L Young; R A Luqmani Journal: Ann Rheum Dis Date: 2008-12-03 Impact factor: 19.103
Authors: Ivana Pantic; Djordje Jevtic; Charles W Nordstrom; Cristian Madrid; Tamara Milovanovic; Igor Dumic Journal: J Clin Med Date: 2022-01-29 Impact factor: 4.241
Authors: Valentina Giorgio; Elisa Blasi; Donato Rigante; Cristina Guerriero; Clara De Simone; Anna Laura Fedele; Giuseppe Stella; Antonio Gasbarrini; Franco Scaldaferri Journal: Int J Environ Res Public Health Date: 2021-06-22 Impact factor: 3.390