Hamsho Suaad, Dumirieh Sumaya, Sleiay Mouhammed, AlBaroudi Douha, Alshekh Muhamad Ali, Alahmad Marwa
Rheumatology Department, Faculty of medicine Damascus University Damascus Syria.
Faculty of Medicine Damascus University Damascus Syria.
Clin Case Rep. 2024 Aug 5;12(8):e9258. doi: 10.1002/ccr3.9258. eCollection 2024 Aug.
Pauci-immune necrotizing glomerulonephritis (PING) is a small vessel renal vasculitis usually associated with the presence of antineutrophil cytoplasmic antibodies (ANCA) against myeloperoxidase or proteinase. A small proportion of PING patients do not have ANCA antibodies.
A condition known as Pauci-immune necrotizing glomerulonephritis, or PING for short, is a type of kidney inflammation that affects small blood vessels. This condition is typically linked with the existence of certain antibodies, specifically antineutrophil cytoplasmic antibodies or ANCA, which target myeloperoxidase or proteinase. However, it's worth noting that a minor percentage of individuals diagnosed with PING do not possess these ANCA antibodies. A 24-year-old woman with no previous medical history arrived at the ER due to various symptoms including joint pain, fever, difficulty swallowing, and shortness of breath. Despite multiple symptoms suggesting systemic lupus erythematosus (SLE), this diagnosis was ruled out based on the EULAR/ACR 2019 classification criteria and laboratory tests. Other potential diagnoses such as rheumatoid arthritis (RA) and eosinophilic garnulomatosis with polyaniitis (EGPA) were also excluded based on respective criteria. The patient was treated with a 3-day course of methylprednisolone, followed by prednisolone, which improved her creatinine levels. Subsequent tests for P-ANCA and C-ANCA were negative. A kidney biopsy confirmed necrotizing glomerulonephritis, consistent with pauci-immune vasculitis. A bronchoscopy revealed bleeding and hemorrhage in her lungs, but bacterial culture analysis was negative. The patient was given piperacillin, tazobactam, and vancomycin for septic coverage, as well as intravenous immunoglobulin (IVIg), which led to symptom improvement.
寡免疫性坏死性肾小球肾炎（PING）是一种小血管性肾血管炎，通常与抗髓过氧化物酶或蛋白酶的抗中性粒细胞胞浆抗体（ANCA）有关。一小部分PING患者没有ANCA抗体。
一种称为寡免疫性坏死性肾小球肾炎，简称PING的疾病，是一种影响小血管的肾脏炎症。这种疾病通常与某些抗体的存在有关，特别是针对髓过氧化物酶或蛋白酶的抗中性粒细胞胞浆抗体或ANCA。然而，值得注意的是，一小部分被诊断为PING的个体并不拥有这些ANCA抗体。一名既往无病史的24岁女性因关节疼痛、发热、吞咽困难和呼吸急促等各种症状来到急诊室。尽管多种症状提示系统性红斑狼疮（SLE），但根据欧洲抗风湿病联盟/美国风湿病学会2019年分类标准和实验室检查，该诊断被排除。其他潜在诊断，如类风湿关节炎（RA）和嗜酸性肉芽肿性多血管炎（EGPA）也根据各自标准被排除。该患者接受了为期3天的甲泼尼龙治疗，随后使用泼尼松龙，这改善了她的肌酐水平。随后的P-ANCA和C-ANCA检测均为阴性。肾脏活检证实为坏死性肾小球肾炎，符合寡免疫性血管炎。支气管镜检查显示她的肺部有出血，但细菌培养分析为阴性。该患者接受了哌拉西林、他唑巴坦和万古霉素以预防感染，以及静脉注射免疫球蛋白（IVIg），症状得到改善。
