C3 glomerulopathy: experience of a pediatric nephrology center

ABSTRACT

Background: C3 glomerulopathy (C3G) is an uncommon disease characterized by the deposition of complement factors in the glomeruli due to overactivation and dysregulation of the alternative pathway of complement.

Objectives: This study aimed to describe the clinicopathological features, laboratory testing, clinical course, treatment, and outcomes of pediatric patients with C3G.

Patients and Methods: We reviewed retrospectively the laboratory testing, kidney biopsy reports, and clinical features of 18 patients at our hospital from 2007 to 2019.

Results: There were 18 cases, and the majority of the patients were girls (61.1%). The mean age at diagnosis was 11.3 ± 3.7 (5–17) years, and nephritic–nephrotic syndrome presentation in patients was more common (11 cases, 61.1%). Hematuria was found in 66.7% of the patients, of which the majority had microscopic hematuria (58.3%). Hypertension was observed in 10 (55.6%) patients. The mean glomerular filtration rate (eGFR) was 95.7 ± 47.3 mL/min/1.73 m², and 24-h urinary protein excretion was 76.2 ± 48.6 mg/m²/h. Sixteen patients (88.9%) received renin–angiotensin–aldosterone system blockers (RASB), and two of them were taking RASB only. The majority of patients (83.3%) were treated with immunosuppressive therapy. Eculizumab was also given to one of them. At the last follow-up, two patients had levels of less than 60 mL/min/1.73 m² for eGFR. Seven patients with immunosuppressive treatment achieved complete remission.

Conclusion: C3G shows a variable clinical presentation and response to immunosuppressive therapy. In the present study, we observed that the most common presentation was nephritic and/or nephrotic syndrome and partially responded to treatment to RASB and immunosuppressants.

KEYWORDS:

• Children
• C3 glomerulopathy
• alternate complement pathway
• eculizumab
• immunosuppressive agents
• kidney biopsy

Disclosure statement

No potential conflict of interest was reported by the authors.

Contributions of authors are added as follows

Study conception and design: Yazılıtaş

Acquisition of data: contribution of all authors

Analysis and interpretation of data: Yazılıtaş, Çakıcı, and Şükür

Drafting of manuscript: Yazılıtaş and Çakıcı

Critical revision: contribution of all authors

Kidney biopsies were evaluated by Orhan

Ethical approval

The study protocol was approved by the Ankara Keçiören Training and Research Hospital at the University of Health Sciences Ethics Committee.

As our analysis was retrospective, we did not receive written approval from the participants.