Yasmin Farah, Jawaid Haris, Batra Simran, Vighio Ashfaque Ahmed, Shaikh Majid Ahmed
2nd Year Medical Student, Dow Medical College, Karachi Pakistan.
3rd Year Medical Student, Dow Medical College, Karachi Pakistan.
J Pak Med Assoc. 2020 Oct;70(10):1869-1873. doi: 10.47391/JPMA.642.
This report describes a rare case of Juvenile Systemic Lupus Erythematosus (JSLE). A young 13-year-old girl presented to the Civil Hospital Karachi on February 15, 2019 with gangrene as the only manifestation of this autoimmune disease. JSLE has several clinical manifestations such as butterfly rash, fever, joint pain, cardiac problems like pericardial infusion and neuropsychiatric disorders. However, in this case gangrene was the only presenting symptom; only laboratory investigations - anti-SSA and anti-ribosomal P protein - were suggestive of JSLE, while anti dsDNA, considered to be the most sensitive and reliable diagnostic tool for Systemic Lupus Erythematosus (SLE), was negative. Raynaud's phenomenon and gangrene have been described as rare symptoms, with gangrene occurring in only a small percentage of SLE patients. Moreover, the patient had received a blood transfusion a few months ago in Hyderabad which was suspected to be the cause of the transmission of infection which lead to polyclonal activation of lymphocytes.
本报告描述了一例罕见的青少年系统性红斑狼疮（JSLE）病例。一名13岁的年轻女孩于2019年2月15日前往卡拉奇市民医院就诊，坏疽是这种自身免疫性疾病的唯一表现。青少年系统性红斑狼疮有多种临床表现，如蝶形红斑、发热、关节疼痛、心脏问题（如心包积液）和神经精神障碍。然而，在这个病例中，坏疽是唯一出现的症状；只有实验室检查——抗SSA和抗核糖体P蛋白——提示青少年系统性红斑狼疮，而被认为是系统性红斑狼疮（SLE）最敏感和可靠诊断工具的抗双链DNA呈阴性。雷诺现象和坏疽被描述为罕见症状，坏疽仅在一小部分系统性红斑狼疮患者中出现。此外，该患者几个月前在海得拉巴接受了输血，怀疑这是导致感染传播的原因，进而导致淋巴细胞多克隆激活。
