Abstract：
OBJECTIVE: 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.
CONCLUSIONS: 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.
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.
CONCLUSIONS: 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.
摘要：
目的：明确X-连锁无丙种球蛋白血症（XLA）患者肾炎的临床和组织学特征及其免疫学特征。
方法：临床，免疫学，回顾性分析9例XLA合并肾炎患者的组织学表现。
结果：根据肾脏组织学发现，XLA和肾炎患者可分为两组，viz.,慢性肾小球肾炎（CGN）和肾小管间质性肾炎（TIN）。两组表现出不同的免疫学特征。CGN组患者表现出XLA的非典型免疫学特征，与致病性渗漏B细胞产生的免疫球蛋白可能在形成免疫复合物和引起免疫介导的肾小球肾炎中起作用。相比之下,TIN组患者表现出典型的XLA免疫学特征，表明抗体非依赖性/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TIN。
结论：XLA患者发生的肾炎可能与肾脏病理和免疫状态有关。建议仔细观察以检测IgRT上XLA患者的肾脏病理。
方法：临床，免疫学，回顾性分析9例XLA合并肾炎患者的组织学表现。
结果：根据肾脏组织学发现，XLA和肾炎患者可分为两组，viz.,慢性肾小球肾炎（CGN）和肾小管间质性肾炎（TIN）。两组表现出不同的免疫学特征。CGN组患者表现出XLA的非典型免疫学特征，与致病性渗漏B细胞产生的免疫球蛋白可能在形成免疫复合物和引起免疫介导的肾小球肾炎中起作用。相比之下,TIN组患者表现出典型的XLA免疫学特征，表明抗体非依赖性/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TIN。
结论：XLA患者发生的肾炎可能与肾脏病理和免疫状态有关。建议仔细观察以检测IgRT上XLA患者的肾脏病理。
