Cheng Ting, Zhi Huiwen, Liu Yunxiao, Zhang Shengxiao, Song Ziyi, Li Yafeng
Department of Rheumatology, The Second Hospital of Shanxi Medical University, Taiyuan 030000, China.
Key Laboratory of Cellular Physiology, Shanxi Medical University, Ministry of Education, Taiyuan 030000, China.
J Clin Med. 2022 Nov 16;11(22):6793. doi: 10.3390/jcm11226793.
Rapidly progressive glomerulonephritis (RPGN) is a life-threatening disease characterized by rapid progressive deterioration of renal function and extensive formation of crescents. Some antibodies tend to be positive, such as a perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) and anti-glomerular basement membrane (anti-GBM) antibodies, in most patients with the disease. However, cases of double positivity for the above antibodies are considered to be rare. In addition, both rheumatoid arthritis (RA) and Sjogren's syndrome (SS) are deemed to be independent immune disorders that can cause renal impairment. Nevertheless, the association between RPGN and these two diseases has not been elucidated in previous studies. Here, we provide a case of RPGN with the concurrence of RA and SS characterized by double positivity in anti-GBM antibodies and p-ANCA. After aggressive treatment with cyclophosphamide, glucocorticoids, and plasma exchange, the patient improved significantly. Despite the malignant event of arteriovenous fistula rupture and bleeding during treatment, the patient survived with renal function recovery for the rest of the follow-up period.
急进性肾小球肾炎（RPGN）是一种危及生命的疾病，其特征为肾功能迅速进行性恶化和大量新月体形成。在大多数该疾病患者中，一些抗体往往呈阳性，如核周型抗中性粒细胞胞浆抗体（p-ANCA）和抗肾小球基底膜（抗GBM）抗体。然而，上述抗体双阳性的病例被认为很罕见。此外，类风湿关节炎（RA）和干燥综合征（SS）均被视为可导致肾功能损害的独立免疫性疾病。尽管如此，先前的研究尚未阐明RPGN与这两种疾病之间的关联。在此，我们报告一例同时并发RA和SS的RPGN病例，其特征为抗GBM抗体和p-ANCA双阳性。经环磷酰胺、糖皮质激素和血浆置换积极治疗后，患者病情显著改善。尽管治疗期间发生了动静脉瘘破裂出血这一不良事件，但患者在随访期剩余时间内肾功能恢复且存活。
