Anti-RNA polymerase III antibody positive limited cutaneous systemic sclerosis with cryoglobulin-induced digital gangrene.
Anti-RNA polymerase III antibody positive limited cutaneous systemic sclerosis with cryoglobulin-induced digital gangrene.
复制标题
抗 RNA 聚合酶 III 抗体阳性，局限性皮肤系统性硬化症伴冷球蛋白诱导的指坏疽。
DOI：
10.1111/ced.12968
复制
发表时间：
2017
期刊：
影响因子：
--
通讯作者：
Katayama I.
中科院分区：
文献类型：
--
作者：
Deguchi A;Yamaoka T;Komurasaki Y;Hayashi M;Kiyohara E;Murota H;Katayama I.
系统性硬化症（SSc）是一种风湿病，其特征是皮肤和各种内脏器官的过度纤维化和微血管损伤。SSc的发病机制尚不清楚，但系统性自身免疫是该疾病的主要特征之一。90%的SSc患者检测到抗核抗体（ANAs）。1基于ANA类型对SSc患者进行亚组可用于诊断、预测器官受累和预后。2据报道，抗rna聚合酶（抗rnap） III抗体阳性的患者弥漫性皮肤受累、硬皮病、肾危象和癌症的风险增加。3,4然而，在抗rnap III抗体阳性的SSc患者中，低患病率指性坏疽的发病机制尚未得到充分研究。我们报告一例抗rnap III抗体阳性的SSc合并由冷球蛋白血症引起的指坏疽。一位77岁的日本女性，表现为1年的手指水肿性红斑病史，但仅在冬季。(a)(c)(d)(b)她在1年前首次向她的初级保健医生提出这种情况。
Systemic sclerosis (SSc) is a rheumatological disorder characterized by excessive fibrosis and microvascular damage of the skin and various internal organs. The pathogenesis of SSc remains unknown, but systemic autoimmunity is one of the central features of the disease. Antinuclear antibodies (ANAs) are detected in> 90% of patients with SSc. 1 Subgrouping patients with SSc based on ANA type can be useful for diagnosis, and for predicting organ involvement and prognosis. 2 Patients who are positive for anti-RNA polymerase (anti-RNAP) III antibodies have been reported to have an increased risk of diffuse cutaneous involvement, scleroderma renal crisis and cancer. 3, 4 However, the pathogenesis of low-prevalence digital gangrene in anti-RNAP III antibody-positive patients with SSc has not been fully investigated. We report a case of anti-RNAP III antibody-positive SSc complicated by digital gangrene induced by cryoglobulinaemia. A 77-year-old Japanese woman presented with 1-year history of oedematous erythema of the fingers, but only during the winter months. She had first presented to her primary care doctor with the condition 1 year previously,(a)(c)(d)(b)