Discordant Phenotypes of Nephritis in Patients with X-linked Agammaglobulinemia.

IF 7.2 2区医学 Q1 IMMUNOLOGY

Journal of Clinical Immunology Pub Date : 2024-07-25 DOI:10.1007/s10875-024-01766-x

Toru Kanamori, Tomohiro Udagawa, Takayuki Fujii, Hiroyoshi Matsukura, Yuka Iwaya, Motoshi Sonoda, Keisuke Sugimoto, Masahiro Takeguchi, Atsunori Yoshino, I-Feng Wang, Daw-Yang Hwang, Harry W Schroeder, Masaki Shimizu, Hans D Ochs, Tomohiro Morio, Hirokazu Kanegane

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Abstract

Purpose: To define the clinical and histological characteristics of nephritis in patients with X-linked agammaglobulinemia (XLA) and their immunological profiles.

Methods: The clinical, immunological, and histological findings of nine patients with XLA and nephritis were retrospectively analyzed.

Results: Based on kidney histological findings, patients with XLA and nephritis could be divided into two groups, viz., chronic glomerulonephritis (CGN) and tubulointerstitial nephritis (TIN). The two groups showed different immunological profiles. Patients in the CGN group exhibited an atypical immunological profile of XLA, with pathogenic leaky B cells producing immunoglobulins that may play a role in forming immune complexes and causing immune-mediated glomerulonephritis. In contrast, patients in the TIN group exhibited a typical immunological profile of XLA, suggesting that antibody-independent/other BTK-dependent mechanisms, or immunoglobulin replacement therapy (IgRT)-related immune/nonimmune-mediated nephrotoxicity causes TIN.

Conclusion: Nephritis occurring in patients with XLA could have links between their renal pathology and immunological status. Careful observation is recommended to detect kidney pathology in patients with XLA on IgRT.

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X-连锁阿加球蛋白血症患者肾炎的不和谐表型。

目的：明确X-连锁丙种球蛋白血症（XLA）患者肾炎的临床和组织学特征及其免疫学特征：方法：回顾性分析9名XLA肾炎患者的临床、免疫学和组织学检查结果：结果：根据肾脏组织学检查结果，XLA合并肾炎患者可分为两组，即慢性肾小球肾炎（CGN）和肾小管间质性肾炎（TIN）。两组患者的免疫学特征各不相同。CGN 组患者的 XLA 免疫学特征不典型，致病性漏出 B 细胞产生的免疫球蛋白可能在形成免疫复合物和导致免疫介导的肾小球肾炎中发挥作用。与此相反，TIN 组患者表现出典型的 XLA 免疫学特征，表明抗体依赖性/其他 BTK 依赖性机制或免疫球蛋白替代疗法（IgRT）相关的免疫/非免疫介导的肾毒性导致了 TIN：结论：XLA 患者出现肾炎可能与肾脏病理和免疫状态有关。建议对接受 IgRT 的 XLA 患者进行仔细观察，以发现肾脏病变。

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来源期刊

Journal of Clinical Immunology 医学-免疫学

CiteScore

12.20

自引率

9.90%

发文量

218

审稿时长

2 months

期刊介绍： The Journal of Clinical Immunology publishes impactful papers in the realm of human immunology, delving into the diagnosis, pathogenesis, prognosis, or treatment of human diseases. The journal places particular emphasis on primary immunodeficiencies and related diseases, encompassing inborn errors of immunity in a broad sense, their underlying genotypes, and diverse phenotypes. These phenotypes include infection, malignancy, allergy, auto-inflammation, and autoimmunity. We welcome a broad spectrum of studies in this domain, spanning genetic discovery, clinical description, immunologic assessment, diagnostic approaches, prognosis evaluation, and treatment interventions. Case reports are considered if they are genuinely original and accompanied by a concise review of the relevant medical literature, illustrating how the novel case study advances the field. The instructions to authors provide detailed guidance on the four categories of papers accepted by the journal.