Diagnosing of Small Vessel Vasculitis Might be a Challenge – A Rare Case Report

A. Shahin, Mohammad Mamun Khan, Swikriti Shrestha, S. J. Abdal, A. Islam, M. Choudhury
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Abstract

The term vasculitis refers to the inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple organ failure. Most patients with small vessel vasculitis present with constitutional symptoms like fever, malaise, weakness, fatigue and weight loss. To diagnose small vessel vasculitis, serology like ANCA, serum cryoglobulin and biopsy play an important role. Despite the serology and biopsy, diagnosing small vessel vasculitis occasionally remains challenging in resource constraint countries. Here we are reporting a case of a 26-year- old female who presented with purpura and neuropathy. The patient lacks clinical features like constitutional symptoms, renal involvement, upper airway involvement and her ANCA was negative. Depending on biopsy finding and skin and neurologic involvement, we diagnosed her as a case of small vessel vasculitis (unclassified). The patient improved with IV methylprednisolone followed by oral glucocorticoid treatment along with methotrexate. Although small vessel vasculitis has some typical features, diagnosis may often remain challenging even after biopsy. Bangladesh Med J. 2020 May; 49(2) : 41-43
小血管炎的诊断可能是一个挑战-一个罕见的病例报告
血管炎是指血管壁的炎症。它的严重程度可以从单个器官的自限性疾病到由于多个器官衰竭而危及生命的疾病。大多数小血管炎患者表现为发热、不适、虚弱、疲劳和体重减轻等体质症状。对于小血管炎的诊断,血清学如ANCA、血清冷球蛋白和活检具有重要作用。尽管有血清学和活检,但在资源有限的国家,诊断小血管炎偶尔仍然具有挑战性。我们在此报告一位二十六岁的女性,以紫癜及神经病变为主要表现。患者缺乏体质症状、肾脏受累、上呼吸道受累等临床特征,ANCA为阴性。根据活检结果和皮肤及神经系统受累情况,我们诊断她为小血管炎(未分类)。患者在静脉注射甲基强的松龙后口服糖皮质激素和甲氨蝶呤治疗后病情好转。虽然小血管炎有一些典型的特征,但即使在活检后诊断仍然具有挑战性。2020年5月;49(2): 41-43
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