Bouhuys Marleen, Armbrust Wineke, van Rheenen Patrick F
Department of Pediatric Gastroenterology, Hepatology and Nutrition, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, Netherlands.
Department of Pediatric Rheumatology and Immunology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, Netherlands.
Front Pediatr. 2021 Feb 10;9:617312. doi: 10.3389/fped.2021.617312. eCollection 2021.
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. 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. 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.
小血管血管炎（SVV）是一种罕见的免疫性疾病，可累及小动脉、毛细血管和小静脉。它会导致紫癜，但也可能在包括胃肠道在内的其他器官中表现出来。SVV与炎症性肠病（IBD）同时出现的频率高于偶然预期。一名16岁女孩，两年前在一家综合医院被诊断为溃疡性结肠炎（UC），在接受硫唑嘌呤和美沙拉嗪维持治疗期间出现紫癜、进行性腹痛伴频繁血性腹泻以及额颞部头痛和肿胀。血清学检查显示核周抗中性粒细胞胞浆抗体（p-ANCA）呈阳性，无抗蛋白酶或髓过氧化物酶抗体。内镜检查显示左侧活动性UC以及升结肠非典型溃疡。对这些溃疡和受累皮肤进行活检显示白细胞破碎性血管炎。最初，这被解释为UC的肠外表现，在诱导缓解时会消退，因此开始使用英夫利昔单抗。在接下来的3周内，她右小腿出现严重灼痛，发展为足下垂伴麻木，紫癜进展为大疱性病变。诊断调整为ANCA相关性血管炎，累及皮肤、肠道和周围神经。停用英夫利昔单抗，给予大剂量泼尼松龙和环磷酰胺诱导治疗，直至SVV和UC缓解。随后，重新引入英夫利昔单抗诱导和维持治疗，并联合甲氨蝶呤。目前已成功维持缓解超过2年。足下垂仅部分缓解，需要使用矫形器。患有IBD且出现紫癜性皮肤病变和腹痛的儿科患者应评估是否存在SVV的全身受累，这包括对胃肠道进行内镜评估。我们讨论了一种诊断、评估和管理全身性SVV的实用方法，重点是及时识别和早期积极治疗以改善预后。
