Y. Guan, MD, PhD, S. Liu, MD, PhD, C.M. Hao, MD, PhD, L. Lai, MD, PhD, Division of Nephrology, Huashan Hospital, Fudan University, Shanghai, China.
J Rheumatol. 2023 Aug;50(8):1032-1038. doi: 10.3899/jrheum.2022-1100. Epub 2023 May 1.
Adult immunoglobulin A vasculitis (IgAV) is documented to be associated with more renal involvement and poorer renal outcomes compared to children, but adult IgAV nephritis (IgAV-N) data are rather limited. The present study aimed to describe the characteristics of adult IgAV-N and investigate the long-term prognostic factors.
Clinical and morphological data from 106 adult patients with biopsy-proven IgAV-N and follow-up data from 94 patients in a single Chinese center were analyzed in this retrospective study. Median follow-up time interval was 102 months.
The median age of patients with IgAV-N at biopsy was 38 (IQR 24-53) years, and 52.8% were male. The median blood pressure was 126/80 mmHg, and 25.5% of patients were hypertensive at baseline. The median initial proteinuria was 1.4 (IQR 0.7-2.2) g/day and estimated glomerular filtration rate (eGFR) was 103 (IQR 84-121) mL/min/1.73 m. The median time interval of onset to biopsy was 8 (IQR 3-40) weeks. In biopsy, the median percentage of global sclerosis was 5.9% (IQR 0.0-13.8), whereas 45.3% of patients had interstitial fibrosis and tubular atrophy. Further, during follow-up, 7.4% patients died, 4.3% patients progressed to endstage kidney disease (ESKD), and 6.4% patients developed > 30% eGFR reduction from baseline. Multivariate Cox proportional analyses revealed hypertension (HTN) history and > 10% global sclerosis at presentation were independent prognostic factors for poor outcome.
The present adult IgAV-N cohort revealed a relatively young onset age, and lower incidence of nephrotic syndrome and ESKD. Moreover, nonimmune factors such as history of HTN and renal chronic histological lesions in biopsy played a crucial role in prognosis of IgAV-N.
成人免疫球蛋白 A 血管炎（IgAV）与儿童相比，更易发生肾脏受累和肾脏预后不良，但成人 IgAV 肾炎（IgAV-N）的数据相当有限。本研究旨在描述成人 IgAV-N 的特征，并探讨其长期预后因素。
本回顾性研究分析了单中心 106 例经活检证实的 IgAV-N 成人患者的临床和形态学数据，以及 94 例患者的随访数据。中位随访时间间隔为 102 个月。
IgAV-N 患者活检时的中位年龄为 38（IQR 24-53）岁，52.8%为男性。中位血压为 126/80mmHg，基线时有 25.5%的患者为高血压。中位初始蛋白尿为 1.4（IQR 0.7-2.2）g/天，估算肾小球滤过率（eGFR）为 103（IQR 84-121）mL/min/1.73m2。发病至活检的中位时间间隔为 8（IQR 3-40）周。在活检中，全球硬化的中位数百分比为 5.9%（IQR 0.0-13.8），而 45.3%的患者有间质纤维化和肾小管萎缩。此外，在随访期间，7.4%的患者死亡，4.3%的患者进展为终末期肾病（ESKD），6.4%的患者从基线开始 eGFR 下降>30%。多变量 Cox 比例分析显示，高血压（HTN）病史和初诊时>10%的全球硬化是不良预后的独立预后因素。
本成人 IgAV-N 队列发病年龄相对较年轻，肾病综合征和 ESKD 的发病率较低。此外，非免疫因素，如高血压病史和活检中的肾慢性组织学病变，在 IgAV-N 的预后中起着关键作用。
