背景:指坏疽是系统性红斑狼疮(SLE)的一种罕见但严重的并发症。它通常发生在疾病持续时间较长的中年患者中。案例:在此,我们报告了一个56岁男子的案例(没有历史暗示雷诺现象,糖尿病,吸烟,创伤,感染,或化学暴露),出现SLE和数字坏疽的人是最初的迹象。他有一个月的关节痛史,脱发,光敏性,口腔溃疡,Malar皮疹,呼吸困难,数字疼痛体格检查显示四肢和背部疼痛和弥漫性红斑皮肤病变,还有手指上的发紫.我们注意到淋巴细胞减少症(600个细胞/mm3),和实验室测试中C反应蛋白升高(15.1mg/l)。抗核抗体(ANA)的免疫学测试呈阳性,标题1:400。肺计算机断层扫描显示肺纤维化,肺功能检查显示限制性肺病。保留了SLE肺部受累的诊断。在寻找有利于患者皮肤病变血管起源的元素时,免疫学评估为阴性。用200mg/天的羟氯喹开始治疗。对于皮肤和肺部受累,静脉注射(IV)脉冲治疗与甲基强的松龙(1,000mg/d,连续3天,每月)和环磷酰胺(1g/月)联用.还规定了钙阻断剂。然而,病变没有改善。患者在14天的间隔内两次输注利妥昔单抗(1克),大多数血管炎病变明显改善,和呼吸困难的部分改善。结论:指坏疽是晚发性SLE的罕见并发症,尤其是作为主要表现。
Background: Digital gangrene is a rare but serious complication of systemic lupus erythematosus (SLE). It occurs usually in middle-aged patients with longer disease duration.
Case: Herein we report the
case of a 56-year-old man (with no history suggestive of Raynaud\'s phenomenon, diabetes mellitus, smoking, trauma, infection, or chemical exposure), who presented with SLE and digital gangrene was among the first signs. He presented with a one-month history of joint pain, hair loss, photosensitivity, mouth ulcers, malar rash, dyspnea, and digital pain. Physical examination revealed painful and diffuse erythematous skin lesions in the extremities and back, as well as cyanosis in the fingers. We noted lymphocytopenia (600 cells/mm 3), and an elevated C-reactive protein (15.1 mg/l) on laboratory tests. Immunological tests were positive for antinuclear antibodies (ANA) with Title 1:400. Pulmonary computed tomography revealed pulmonary fibrosis, and pulmonary function tests revealed the restrictive pulmonary disease. Diagnosis of SLE with lung involvement was retained. The immunological assessment in search of elements in favor of a vascular origin of the patient\'s skin lesions was negative. Treatment was initiated with 200 mg/day hydroxychloroquine. For dermal and pulmonary involvement, intravenous (IV) pulse therapy was used with methylprednisolone (1,000 mg/d for three consecutive days monthly) and cyclophosphamide (1 g/month). Calcium blocking agents were also prescribed. However, the lesions did not improve. The patient was given two infusions of rituximab (1 g) at a 14-day interval with a marked improvement ofthe majority of vasculitis lesions, and a partial improvement of dyspnea. Conclusions: Digital gangrene is a rare complication of late-onset SLE, especially as a primary manifestation.