Clinical relevance of membrane attack complex deposition in children with IgA nephropathy and Henoch-Schönlein purpura

• Published in: Pediatric nephrology (Berlin, West) West), 2020-05, Vol.35 (5), p.843-850
• Main Authors: Dumont, Camille, Mérouani, Aicha, Ducruet, Thierry, Benoit, Geneviève, Clermont, Marie-Josée, Lapeyraque, Anne Laure, Phan, Véronique, Patey, Natalie
• Format: Article
• Language: English
• Subjects: Adolescent; Biomarkers - analysis; Biopsy; Child; Child, Preschool; Children; Complement activation; Complement Membrane Attack Complex - analysis; Complement Membrane Attack Complex - immunology; Complement Pathway, Alternative - drug effects; Complement Pathway, Alternative - immunology; Complement Pathway, Mannose-Binding Lectin - drug effects; Complement Pathway, Mannose-Binding Lectin - immunology; Feasibility Studies; Female; Follow-Up Studies; Glomerular filtration rate; Glomerular Mesangium - immunology; Glomerular Mesangium - pathology; Glomerulonephritis, IGA - diagnosis; Glomerulonephritis, IGA - drug therapy; Glomerulonephritis, IGA - immunology; Glomerulonephritis, IGA - pathology; Humans; IgA nephropathy; Immunoglobulin A; Immunoglobulin A - immunology; Immunohistochemistry; Immunosuppressive Agents - pharmacology; Immunosuppressive Agents - therapeutic use; Male; Medicine; Medicine & Public Health; Membrane attack complex; Mesangium; Nephrology; Nephrotic syndrome; Original Article; Patients; Pediatrics; Prognosis; Proteinuria; Purpura, Schoenlein-Henoch - diagnosis; Purpura, Schoenlein-Henoch - drug therapy; Purpura, Schoenlein-Henoch - immunology; Purpura, Schoenlein-Henoch - pathology; Renal function; Retrospective Studies; Schonlein-Henoch purpura; Treatment Outcome; Urology
• ISSN: 0931-041X; 1432-198X
• DOI: 10.1007/s00467-019-04445-x

Background IgA nephropathy (IgAN) and Henoch-Schönlein purpura are common glomerular disorders in children sharing the same histopathologic pattern of IgA deposits within the mesangium, even if their physiopathology may be different. Repeated exposure to pathogens induces the production of abnormal IgA1. The immune complex deposition in the renal mesangium in IgAN or potentially in small vessels in Henoch-Schönlein purpura induces complement activation via the alternative and lectin pathways. Recent studies suggest that levels of membrane attack complex (MAC) in the urine might be a useful indicator of renal injury. Because of the emerging availability of therapies that selectively block complement activation, the aim of the present study is to investigate whether MAC immunostaining might be a useful marker of IgA-mediated renal injury. Methods We conducted immunohistochemistry analysis of the MAC on renal biopsies from 67 pediatric patients with IgAN and Henoch-Schönlein purpura. We classified their renal biopsies according to the Oxford classification, retrieved symptoms, biological parameters, treatment, and follow-up. Results We found MAC expression was significantly related to impaired renal function and patients whose clinical course required therapy. MAC deposits tend to be more abundant in patients with decreased glomerular filtration rate ( p  = 0.02), patients with proteinuria > 0.750 g/day/1.73 m 2 , and with nephrotic syndrome. No correlation with histological alterations was observed. Conclusions We conclude that MAC deposition could be a useful additional indicator of renal injury in patients with IgAN and Henoch-Schönlein purpura, independent of other indicators.