Ma Rui, Wu Dengyan, He Zhiqin, Chang Qian, Yang Yonghong
Department of Pediatric Nephrology, Lanzhou University Second Hospital, Lanzhou, China.
Department of Nephrology, Gansu Children's Hospital, Lanzhou, China.
Front Pediatr. 2021 Feb 4;8:568773. doi: 10.3389/fped.2020.568773. eCollection 2020.
C1q nephropathy is a glomerulopathy that is characterized by large amount of C1q deposits in the glomerular mesangium. It is a diagnosis of exclusion after ruling out systemic lupus erythematosus and membranoproliferative glomerulonephritis by systemic and serological examination. The pathogenesis of C1q nephropathy is unclear. In addition, there is very little generalizability in the treatment and prognosis for pediatric C1q nephropathy due to diversities in clinical manifestations and pathological types. Rituximab is a human/mouse chimeric monoclonal antibody against CD20, which is primarily used for treating lymphomas and, most recently, has been used to treat certain kidney diseases including C1q nephropathy. In this report, we used one quarter of the typical dose of rituximab for lymphoma treatment to achieve complete remission in a C1q nephropathy patient, significantly reducing deposition of immune complexes and glomerular damage. This case indicates that dosage reconsiderations may be necessary for rituximab in treatment of pediatric C1q nephropathy.
C1q肾病是一种肾小球疾病,其特征是肾小球系膜中有大量C1q沉积。它是在通过全身和血清学检查排除系统性红斑狼疮和膜增生性肾小球肾炎后作出的排除性诊断。C1q肾病的发病机制尚不清楚。此外,由于临床表现和病理类型的多样性,儿童C1q肾病的治疗和预后几乎没有普遍适用性。利妥昔单抗是一种针对CD20的人/鼠嵌合单克隆抗体,主要用于治疗淋巴瘤,最近也被用于治疗包括C1q肾病在内的某些肾脏疾病。在本报告中,我们使用了治疗淋巴瘤典型剂量四分之一的利妥昔单抗,使一名C1q肾病患者完全缓解,显著减少了免疫复合物的沉积和肾小球损伤。该病例表明,在治疗儿童C1q肾病时,可能有必要重新考虑利妥昔单抗的剂量。