PDF(Pubmed)
Abstract：
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease and there is a distinct differentiation of clinical manifestations. Lupus nephritis (LN) is clinically apparent in approximately half of patients. A kidney biopsy is essential to define the kidney injury, exclude other injurious causes, and determine the histopathologic subtypes. Autoantibodies are crucial to the pathogenesis and the deposition of immune complexes in glomeruli is a hallmark of LN. The histopathology of LN is quite varied. Despite pauci-immune LN being an unexpected condition in SLE, it has been observed rarely with the presence of antineutrophil cytoplasmic autoantibodies (ANCA). We present a young male who was admitted to the emergency with syncope. The brain imaging revealed small infarct areas and signs of cerebral vasculitis. Also, he had elevated inflammatory markers, moderate proteinuria, and preserved kidney function. Anti-nuclear antibodies and anti-dsDNA were positive. Pauci-immune crescentic glomerulonephritis (PICGN) was observed in a kidney biopsy, however, ANCA was negative. SLE diagnosis was established by neurological manifestation, specific antibodies, proteinuria, and kidney biopsy findings. We administered a combination induction regimen, including pulse steroid and parenteral cyclophosphamide. The proteinuria was resolved in the follow-up. Our case highlights that SLE-associated ANCA-negative PICGN can be the initial presentation in the absence of typical manifestations. LN exhibits various pathological mechanisms in the kidney. As a consequence, SLE should be considered in the differential diagnosis of all forms of kidney injury.
摘要：
系统性红斑狼疮（SLE）是一种慢性自身免疫性疾病，临床表现有明显差异。狼疮性肾炎（LN）在大约一半的患者中临床上很明显。肾活检对于确定肾损伤至关重要，排除其他有害原因，并确定组织病理学亚型。自身抗体对发病机理至关重要，免疫复合物在肾小球中的沉积是LN的标志。LN的组织病理学差异很大。尽管缺乏免疫LN是SLE的一种意外情况，很少观察到存在抗中性粒细胞胞浆自身抗体（ANCA）。我们介绍了一名年轻男性，他因晕厥而入院。脑成像显示小梗死区和脑血管炎的征象。此外，他的炎症标志物升高，中度蛋白尿,保留肾功能。抗核抗体和抗dsDNA为阳性。在肾活检中观察到Pauci免疫性新月体肾小球肾炎（PICGN），然而,ANCA是阴性的。SLE诊断是根据神经系统表现确定的，特异性抗体，蛋白尿,和肾活检结果。我们采用联合诱导方案，包括脉冲类固醇和肠胃外环磷酰胺。蛋白尿在随访中得到解决。我们的病例强调，在没有典型表现的情况下，SLE相关的ANCA阴性PICGN可能是最初的表现。LN在肾脏中表现出各种病理机制。因此,在所有形式的肾损伤的鉴别诊断中应考虑SLE。
