Abstract:
Cryoglobulinemia is a rare entity which frequently occurs in the setting of an underlying disease, with chronic hepatitis C virus infections and primary Sjogren syndrome being the most common underlying conditions. Cryoglobulinemic vasculitis (CV) is an immune-mediated type of small-vessel vasculitis with a broad spectrum of specific organ involvement, including renal, pulmonary, peripheral nerve, and cutaneous involvement and variable manifestations that can be life threatening. Moreover, a strong relationship between cryoglobulinemia and CV, with the future development of lymphoma, is well established. We present the case of a 72-year-old Caucasian male referred for nephrotic syndrome and acute renal injury. A diagnosis of Sjogren syndrome-associated CV was made, and he was successfully treated with rituximab and plasma exchange sessions.
摘要:
冷球蛋白血症是一种罕见的实体,经常发生在潜在疾病的背景下,慢性丙型肝炎病毒感染和原发性干燥综合征是最常见的基础疾病。冷球蛋白性血管炎(CV)是一种免疫介导的小血管血管炎,具有广泛的特定器官受累,包括肾,肺,周围神经,皮肤受累和各种可能危及生命的表现。此外,冷球蛋白血症和CV之间有很强的关系,随着淋巴瘤的未来发展,建立得很好。我们介绍了一名72岁的高加索男性,因肾病综合征和急性肾损伤而转诊。诊断为干燥综合征相关的CV,利妥昔单抗和血浆置换治疗成功.