Service d'anatomie et cytologie pathologiques, Hôpital Trousseau, CHRU Tours, Tours, France.
Université de Tours, PRES Centre-Val de Loire, Tours, France.
Diagn Pathol. 2020 May 27;15(1):62. doi: 10.1186/s13000-020-00980-6.
Infection-related glomerulonephritis with IgA deposits (IRGN-IgA) is a rare disease but it is increasingly reported in the literature. Data regarding epidemiology and outcome are lacking, especially in Europe. We aimed to assess the clinical, pathologic and outcome data of IRGN-IgA.
Clinical and outcome data from patients from 11 French centers over the 2007-2017 period were collected retrospectively. We reviewed pathologic patterns and immunofluorescence of renal biopsies and evaluated C4d expression in IRGN-IgA. We analyzed the correlation between histological presentation and outcome.
Twenty-seven patients (23 men, mean age: 62 ± 15 years) were included. Twenty-one (78%) had Staphylococcus aureus infection and twelve (44%) were diabetic. At the time of biopsy, 95.2% had haematuria, 48.1% had a serum creatinine level of > 4 mg/dL, and 16% had hypocomplementemia. The most common pathologic presentation included mesangial (88.9%) and endocapillary proliferative glomerulonephritis (88.9%) with interstitial fibrosis and tubular atrophy (IF/TA) (85.1%). Diffuse and global glomerular C4d expression was found in 17.8%, mostly in biopsies with acute or subacute patterns, and was associated with a short delay between infection and renal biopsy compared to segmental and focal staining. After median follow-up of 13.2 months, 23.1% died, 46.2% had persistent renal dysfunction and 15.4% reached end-stage renal disease. Renal outcome was correlated to IF/TA severity.
Infection-related glomerulonephritis with IgA deposits is usually associated with Staphylococcus infections and mainly affects adult men. This entity has a poor prognosis which is correlated to interstitial fibrosis and tubular atrophy severity.
伴有 IgA 沉积的感染相关性肾小球肾炎（IRGN-IgA）是一种罕见疾病，但在文献中报道越来越多。缺乏关于流行病学和结局的数据，特别是在欧洲。我们旨在评估 IRGN-IgA 的临床、病理和结局数据。
回顾性收集了 2007 年至 2017 年期间来自法国 11 个中心的患者的临床和结局数据。我们复习了肾活检的病理模式和免疫荧光，并评估了 IRGN-IgA 中 C4d 的表达。我们分析了组织学表现与结局之间的相关性。
共纳入 27 例患者（23 例男性，平均年龄 62±15 岁）。21 例（78%）有金黄色葡萄球菌感染，12 例（44%）为糖尿病患者。在肾活检时，95.2%有血尿，48.1%的血清肌酐水平>4mg/dL，16%有低补体血症。最常见的病理表现包括系膜（88.9%）和内皮下增生性肾小球肾炎（88.9%），伴有间质纤维化和肾小管萎缩（IF/TA）（85.1%）。弥漫性和全球性肾小球 C4d 表达见于 17.8%，主要见于急性或亚急性模式的活检，与感染和肾活检之间的时间间隔较短有关，而与节段性和局灶性染色相比。在中位数为 13.2 个月的随访中，23.1%的患者死亡，46.2%的患者持续存在肾功能障碍，15.4%的患者达到终末期肾病。肾脏结局与 IF/TA 严重程度相关。
伴有 IgA 沉积的感染相关性肾小球肾炎通常与金黄色葡萄球菌感染有关，主要影响成年男性。这种疾病的预后较差，与间质纤维化和肾小管萎缩的严重程度相关。
