Evans Rhys D R, Cargill Tamsin, Goodchild George, Oliveira Ben, Rodriguez-Justo Manuel, Pepper Ruth, Connolly John, Salama Alan, Webster George, Barnes Eleanor, Culver Emma L
University College London Centre for Nephrology, Royal Free Hospital, London, UK.
Translational Gastroenterology Unit, John Radcliffe Hospital, and Nuffield Department of Medicine, University of Oxford, Oxford, UK.
Kidney Int Rep. 2018 Sep 1;4(1):48-58. doi: 10.1016/j.ekir.2018.08.011. eCollection 2019 Jan.
IgG4-related disease (IgG4-RD) is a relapsing multisystem fibro-inflammatory disease, which may involve the kidney (IgG4-related kidney disease [IgG4-RKD]) and retroperitoneum (IgG4-related retroperitoneal fibrosis [IgG4-RPF]). The aim of this study was to describe IgG4-RKD and IgG4-RPF in the United Kingdom.
We conducted a retrospective observational study of patients with IgG4-RKD and IgG4-RPF in a multicenter IgG4-RD cohort. Data were collected through review of medical records. We describe clinical parameters at baseline, histological and radiological findings, treatment, and patient outcomes.
Of 154 patients with IgG4-RD, 14 (9.1%) had IgG4-RKD, 10 (6.5%) had IgG4-RPF, and 4 (2.6%) had both. Patients were aged 58.2 ± 14.2 years, and 26 (92.9%) were male. Creatinine at presentation was worse in those with intrinsic renal disease (229 μmol/l vs. 110 μmol/l; = 0.0076). Serum IgG4 was elevated in the majority of patients (87.5%), and hypocomplementemia was present in half of those with IgG4-RKD. Fifteen patients underwent renal biopsy; tubulointerstitial nephritis with abundant IgG4+ plasma cells was the most common finding (n = 14; 93.3%), and 4 (26.7%) patients had membranous nephropathy. Most patients (89.3%) were treated with corticosteroids, and 4 (16.0%) with additional azathioprine as initial management. Thirteen patients (46.4%) relapsed over 60 ± 48 months of follow-up, at median 18 (12-36) months after renal/RPF diagnosis; 61.5% of relapses were in the kidney. Renal function deteriorated in 5 patients (20.8%), including 2 (8.3%) who reached end-stage renal disease (ESRD).
IgG4-RKD and IgG4-RPF represent major organ manifestations of IgG4-RD, and should be identified early with prompt treatment to prevent progression to ESRD.
IgG4相关性疾病（IgG4-RD）是一种复发性多系统纤维炎性疾病，可累及肾脏（IgG4相关性肾病[IgG4-RKD]）和腹膜后（IgG4相关性腹膜后纤维化[IgG4-RPF]）。本研究旨在描述英国的IgG4-RKD和IgG4-RPF。
我们对一个多中心IgG4-RD队列中的IgG4-RKD和IgG4-RPF患者进行了一项回顾性观察研究。通过查阅病历收集数据。我们描述了基线时的临床参数、组织学和影像学发现、治疗情况及患者预后。
在154例IgG4-RD患者中，14例（9.1%）患有IgG4-RKD，10例（6.5%）患有IgG4-RPF，4例（2.6%）两者均有。患者年龄为58.2±14.2岁，26例（92.9%）为男性。原发性肾病患者就诊时的肌酐水平更高（229μmol/l对110μmol/l；P = 0.0076）。大多数患者（87.5%）血清IgG4升高，IgG4-RKD患者中有一半存在低补体血症。15例患者接受了肾活检；最常见的发现是伴有大量IgG4+浆细胞的肾小管间质性肾炎（n = 14；93.3%），4例（26.7%）患者患有膜性肾病。大多数患者（89.
