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IgM肾病患者的临床特征与肾脏结局:一项回顾性单中心研究

Clinical Characteristics and Renal Outcomes in Patients with IgM Nephropathy: A Retrospective Single-Center Study.

作者信息

Albakr Rehab B, Sandokji Ibrahim A, Asfina Kazi N, Bakhit Amaar, Alnaeim Norah, Albacker Abdulaziz B, Husain Sufia

机构信息

Department of Medicine, Division of Nephrology, College of Medicine, King Saud University, Riyadh, Saudi Arabia.

Pediatric Department, College of Medicine, Taibah University, Madinah, Saudi Arabia.

出版信息

Int J Gen Med. 2025 Aug 29;18:4923-4939. doi: 10.2147/IJGM.S541794. eCollection 2025.

Abstract

BACKGROUND

Defining immunoglobulin M (IgM) nephropathy as a discrete clinical disorder remains controversial, with limited documentation in Saudi Arabian patients.

AIM

This study analyzes the clinical and pathological features, as well as the prognosis, of IgM nephropathy in the Saudi population.

METHODS

This study is conducted as a retrospective descriptive study at the nephrology unit of King Saud University Medical City in Riyadh. The study included individuals with biopsy-proven IgM nephropathy from 2016 to 2024. Stringent diagnostic criteria were employed, focusing on immunofluorescence positivity (IgM 1+ to 3+). Clinical data were collected from a kidney biopsy registry from the pathology department at King Saud University, and renal pathology was evaluated by a single expert renal pathologist for various features, including mesangial cell proliferation and glomerulosclerosis.

RESULTS

We analyzed a total of 15 patients with IgM nephropathy (n = 15), with a median age at diagnosis of 4 years (IQR: 2-8) and a male predominance (80%). Patients were categorized into early childhood onset (<4 years, 46.7%) and late childhood onset (≥4 years, 53.3%). Common manifestations included nephrotic syndrome (93.3%) and generalized body swelling (66.7%), with lower limb edema reported in all patients (100%). Nephrotic-range proteinuria was present in 93.3%, with preserved renal function (median eGFR: 158 mL/min/1.73m²). Biopsy results for nine patients revealed mesangial proliferation in 66.7%. The overall relapse rate was 93.3%, with early-onset patients exhibiting higher rates of steroid resistance and more frequent relapses.

CONCLUSION

This study enhances the understanding of IgM nephropathy in Saudi Arabia, indicating that early onset may be associated with a more aggressive disease course and higher relapse rates, highlighting the need for early identification of high-risk patients. Additionally, the use of rituximab in refractory cases may present a promising option for further evaluation.

摘要

背景

将免疫球蛋白M(IgM)肾病定义为一种独立的临床疾病仍存在争议,沙特阿拉伯患者中的相关文献有限。

目的

本研究分析沙特人群中IgM肾病的临床和病理特征以及预后情况。

方法

本研究在利雅得国王沙特大学医学城的肾脏病科进行回顾性描述性研究。研究纳入了2016年至2024年经活检证实为IgM肾病的患者。采用了严格的诊断标准,重点关注免疫荧光阳性(IgM 1+至3+)。临床数据从沙特国王大学病理科的肾脏活检登记处收集,肾脏病理由一名专业肾脏病理学家评估各种特征,包括系膜细胞增殖和肾小球硬化。

结果

我们共分析了15例IgM肾病患者(n = 15),诊断时的中位年龄为4岁(四分位间距:2 - 8岁),男性占优势(80%)。患者分为儿童早期发病(<4岁,46.7%)和儿童晚期发病(≥4岁,53.3%)。常见表现包括肾病综合征(93.3%)和全身肿胀(66.7%),所有患者均有下肢水肿(100%)。93.3%的患者出现肾病范围蛋白尿,肾功能保留(中位估算肾小球滤过率:158 mL/min/1.73m²)。9例患者的活检结果显示66.7%有系膜增生。总体复发率为93.3%,早期发病患者表现出更高的激素抵抗率和更频繁的复发。

结论

本研究增进了对沙特阿拉伯IgM肾病的了解,表明早期发病可能与更具侵袭性的病程和更高的复发率相关,凸显了早期识别高危患者的必要性。此外,在难治性病例中使用利妥昔单抗可能是一个有前景的进一步评估选项。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0e66/12405712/05e3e8df858e/IJGM-18-4923-g0001.jpg

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