Diagnostic and Prognostic Comparison of Immune-Complex-Mediated Membranoproliferative Glomerulonephritis and C3 Glomerulopathy

Author:

Kovala Marja1ORCID,Seppälä Minna2,Räisänen-Sokolowski Anne1ORCID,Meri Seppo3ORCID,Honkanen Eero2,Kaartinen Kati2

Affiliation:

1. Department of Pathology, University of Helsinki and Helsinki University Hospital, 00290 Helsinki, Finland

2. Department of Nephrology, University of Helsinki and Helsinki University Hospital, 00290 Helsinki, Finland

3. Translational Immunology Research Program TRIMM, Department of Bacteriology and Immunology, University of Helsinki and Helsinki University Hospital, 00290 Helsinki, Finland

Abstract

Membranoproliferative glomerulonephritis (MPGN) is subdivided into immune-complex-mediated glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G). Classically, MPGN has a membranoproliferative-type pattern, but other morphologies have also been described depending on the time course and phase of the disease. Our aim was to explore whether the two diseases are truly different, or merely represent the same disease process. All 60 eligible adult MPGN patients diagnosed between 2006 and 2017 in the Helsinki University Hospital district, Finland, were reviewed retrospectively and asked for a follow-up outpatient visit for extensive laboratory analyses. Thirty-seven (62%) had IC-MPGN and 23 (38%) C3G (including one patient with dense deposit disease, DDD). EGFR was below normal (≤60 mL/min/1.73 m2) in 67% of the entire study population, 58% had nephrotic range proteinuria, and a significant proportion had paraproteins in their serum or urine. A classical MPGN-type pattern was seen in only 34% of the whole study population and histological features were similarly distributed. Treatments at baseline or during follow-up did not differ between the groups, nor were there significant differences observed in complement activity or component levels at the follow-up visit. The risk of end-stage kidney disease and survival probability were similar in the groups. IC-MPGN and C3G have surprisingly similar characteristics, kidney and overall survival, which suggests that the current subdivision of MPGN does not add substantial clinical value to the assessment of renal prognosis. The high proportion of paraproteins in patient sera or in urine suggests their involvement in disease development.

Funder

Helsinki University Hospital Diagnostic center

Alexion

Publisher

MDPI AG

Subject

General Medicine

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