Pauci-immune crescentic glomerulonephritis caused to dilemma in a patient with suspected systemic lupus erythematosus: a case report.

IF 1 Q4 UROLOGY & NEPHROLOGY CEN Case Reports Pub Date : 2024-06-01 Epub Date: 2023-10-14 DOI:10.1007/s13730-023-00825-3
Cihan Uysal, Sule Ketenci Ertas, Merve Civan, Hulya Akgun, Ismail Kocyigit
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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.

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一例疑似系统性红斑狼疮患者的Pauci免疫新月体肾小球肾炎陷入困境:病例报告。
系统性红斑狼疮(SLE)是一种慢性自身免疫性疾病,临床表现有明显的分化。狼疮性肾炎(LN)在大约一半的患者中具有明显的临床表现。肾活检对于确定肾损伤、排除其他损伤原因和确定组织病理学亚型至关重要。自身抗体对LN的发病机制至关重要,免疫复合物在肾小球中的沉积是LN的标志。LN的组织病理学变化很大。尽管缺乏免疫性LN是SLE的一种意外情况,但很少观察到其存在抗中性粒细胞胞浆自身抗体(ANCA)。我们介绍一位年轻男性,他因晕厥入院。脑部影像显示有小面积梗塞和脑血管炎的迹象。此外,他的炎症标志物升高,中度蛋白尿,肾功能保持。抗核抗体和抗dsDNA阳性。在肾活检中观察到Pauci免疫新月体肾小球肾炎(PICGN),但ANCA为阴性。SLE的诊断是通过神经系统表现、特异性抗体、蛋白尿和肾活检结果来确定的。我们采用了联合诱导方案,包括脉冲类固醇和肠外环磷酰胺。蛋白尿在随访中得到缓解。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在肾脏中表现出多种病理机制。因此,在鉴别诊断各种形式的肾损伤时,应考虑SLE。
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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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