Khadka Nisha, Kandel Kamal, Mishra Aakash, Jha Saket
Kathmandu Medical College and Teaching Hospital.
Manipal College of Medical Sciences, Pokhara, Nepal.
Ann Med Surg (Lond). 2023 Jun 20;85(8):4067-4070. doi: 10.1097/MS9.0000000000001009. eCollection 2023 Aug.
Systemic lupus erythematosus (SLE) is a rare autoimmune condition that may affect almost every organ system and has a wide range of disease severity. It is characterized by a spectrum of clinical manifestation, a plethora of autoantibodies, and immune complex formation. The symptoms can come from any organ system, alone or in a group, and they can be of any severity, which makes diagnosis and prognosis difficult.
The authors hereby present the case of an 18-year-old female with chief complaints of fever, abdominal pain, headache, vomiting, and loss of vision. She was diagnosed with acute pancreatitis (AP) and intracerebral hemorrhage (ICH) with an etiology linked to SLE. SLICC criterion was used to diagnosed SLE while ATLANTA criteria for AP and neuro-radiological findings for ICH. Emergency temporo- parietal-occipital-osteoplastic craniotomy was done for ICH as well as started with immunosuppressive therapy for SLE. On the 18th day of admission, she was discharge with maintenance medications for SLE. While the vision took over a month to come to a premorbid state, she was clinically improved within 2 weeks of admission.
Clinical manifestation of SLE vary greatly. AP and intracranial bleeding are few of the rare presentation of SLE. Acute presentation of both conditions in an otherwise healthy individual in the initial course of disease left the clinician with a wide array of differentials. Literature shows very little evidence of co-occurrence of ICH and pancreatitis as an initial manifestation in SLE patients. The exclusive diagnosis of these potentially fatal condition is made holistically with clinical, biochemical, and radiological parameters.
SLE may present with atypical, life-threatening initial manifestations. Early diagnosis and timely intervention in therapy can lead to successful management. The treating physician must consider, SLE when a straightforward diagnosis is associated with inexplicable multiple concomitant abnormalities, especially in young women.
系统性红斑狼疮（SLE）是一种罕见的自身免疫性疾病，几乎可累及每个器官系统，疾病严重程度范围广泛。其特征为一系列临床表现、大量自身抗体以及免疫复合物形成。症状可来自任何器官系统，单独出现或成群出现，严重程度各异，这使得诊断和预后都很困难。
作者在此报告一例18岁女性病例，主要症状为发热、腹痛、头痛、呕吐及视力丧失。她被诊断为急性胰腺炎（AP）和脑出血（ICH），病因与SLE有关。采用SLICC标准诊断SLE，AP采用亚特兰大标准，ICH采用神经放射学检查结果。对ICH进行了急诊颞顶枕骨成形开颅手术，并开始对SLE进行免疫抑制治疗。入院第18天，她出院并服用SLE维持药物。虽然视力恢复到病前状态花了一个多月时间，但她在入院后2周内临床症状有所改善。
SLE的临床表现差异很大。AP和颅内出血是SLE罕见的表现形式。在疾病初始阶段，原本健康的个体同时出现这两种疾病的急性表现，给临床医生带来了众多不同的鉴别诊断。文献显示，SLE患者中很少有证据表明ICH和胰腺炎会同时作为初始表现出现。这些潜在致命疾病的明确诊断需综合临床、生化和放射学参数来做出。
SLE可能以非典型、危及生命的初始表现出现。早期诊断和及时的治疗干预可实现成功管理。当直接诊断与无法解释的多种伴随异常相关时，尤其是在年轻女性中，治疗医生必须考虑到SLE。
