Clinical and pathological faces of IgM deposition in children with idiopathic nephrotic syndrome: A single-center experience
Saudi Journal of Kidney Diseases and Transplantation, cilt.37, sa.2, ss.94-103, 2026 (ESCI, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 37 Sayı: 2
- Basım Tarihi: 2026
- Doi Numarası: 10.4103/sjkdt.sjkdt_512_21
- Dergi Adı: Saudi Journal of Kidney Diseases and Transplantation
- Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus, Arab World Research Source, EMBASE, MEDLINE, Directory of Open Access Journals, Biomedical Reference Collection: Corporate Edition (EBSCO), Health Research Premium Collection (ProQuest), Pharma Collection (ProQuest)
- Sayfa Sayıları: ss.94-103
- Anahtar Kelimeler: C3 co-deposition, children, immunoglobulin M intensity, immunoglobulin M nephropathy, mesangial proliferative glomerulonephritis, steroid-dependent nephrotic syndrome
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
Background: Immunoglobulin M nephropathy (IgMN) is a pathological term defining glomerulonephritis with immunoglobulin M (IgM) deposition. The clinical significance is still a matter of debate. The aim was to evaluate children with IgM nephropathy (IgMN) in terms of clinical and pathological features, along with treatment responses and outcomes. Materials and Methods: The children with idiopathic nephrotic syndrome (INS) who underwent kidney biopsy at our center (n = 41) were evaluated retrospectively. Twenty-one children with IgMN were included in the study. Results: The female-to-male ratio (f was 0.9, the median age was 3.5 years in the study group. The mean disease duration and follow-up periods were 11.8 and 11.3 years, respectively. At admission, 14% of the patients had hypertension, and 19% had microscopic hematuria. Steroid-dependent nephrotic syndrome (SDNS) was observed in 62% of the patients at admission and 81% at last visit. The patients with IgM (≥2+) depositions had more SDNS than those with IgM (1+). The most common light microscopic diagnosis was mesangial proliferative glomerulonephritis (MesPGN) (47.6%). Focal segmental glomerulosclerosis (FSGS) elevated significantly from 14% at initial biopsy to 57% at follow-up biopsies. Patients who progressed to FSGS mostly had C3 co-deposition, high IgM intensity (≥2+), diagnosis of MesPGN, and SDNS clinic. The most frequently used adjuvant agent was cyclosporine-A (n = 19) with a mean duration of 68 months. It provided lower relapse rates. Rituximab (n = 4) showed 75% remission rate. None of the patients had needed renal replacement treatment. Two patients who were steroid-resistant at admission had FSGS in their first biopsies, acted as multidrug resistance at follow-up, and ended up in Stage-2 chronic kidney disease (CKD). Conclusion: This study shows IgMN is mainly presented with SDNS clinic and MesPGN pathology. Evolution to FSGS may be related to steroid resistance, MesPGN, high IgM intensity, and C3 co-deposition.