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ETIOLOGY AND CLINICO-MORPHOLOGICAL PRESENTATION OF MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS IN RUSSIAN POPULATION

https://doi.org/10.24884/1561-6274-2018-22-4-9-17

Abstract

THE AIM. Analysis of etiology, clinical and morphological manifestations, of membranoproliferative glomerulonephritis  (MPGN).

PATIENTS AND METHODS. Retrospective analysis focused on etiology, clinical presentation, light and electron microscopy, immunoglobulins (Ig) and C3 complement component  (C3) deposits was done in the cohort of MPGN cases identified in the period 2000-2017. RESULTS. Two hundred and fourteen cases of  MPGN were included in the study (mean age of 44 ± 16 years). Most patients had nephrotic syndrome and significant hematuria. In  58.4% of cases, eGFR was <60 mL/min/1.73 m2, and every fifth  patient had CKD stages 4 or 5. The prevalence of MPGN among all  biopsy-confirmed glomerulopathies was 9.3%. Idiopathic MPGN  (iMPGN) was diagnosed in 30.4% of cases, while the proportion of  iMPGN cases significantly reduced along the study period. Secondary  MPGN (sMPGN) was identified in 69.6% of cases  (autoimmune diseases – 34.1%, infectious diseases – 16.4%,  monoclonal gammopathies – 9.3%, complement-mediated damage – 9.8%). Ig+C3+MPGN was mainly associated with autoimmune  diseases and infections, however 26,6% of such cases remained  “idiopathic”. C3-glomerulopathy or thrombotic microangiopathy were most often causes of Ig-C3+MPGN. Ig-C3-/Ig+C3-MPGN had  heterogeneous etiology.

 

CONCLUSION. MPGN is a severe variant of glomerular damage with a heterogeneous etiological structure. Targeted routine clinical and  morphological diagnostics of MPGN allows identifying the cause of  the disease in most cases. This approach is reliable for the adequate  treatment choice and improvement of outcomes in sMPGN. Further  improvement in diagnostic and classification approaches in idiopathic MPGN relies on progress in understanding of molecular etiopathogenesis of the disease.

About the Authors

V. A. Dobronravov
Research Institute of Nephrology, First Pavlov Saint-Petersburg State Medical University
Russian Federation

197022 Russia, St. Petersburg, L. Tolstoy st., 17 build. 54

Prof. Vladimir A. Dobronravov, MD, PhD, DMedSci, Pavlov First Saint Petersburg State Medical University Institute of Nephrology, Vice Director, Phone (812) 338-69-01



A. V. Smirnov
Research Institute of Nephrology, First Pavlov Saint-Petersburg State Medical University
Russian Federation

197022 Russia, St. Petersburg, L. Tolstoy st., 17 build. 54

Prof. Alexey V. Smirnov MD, PhD, DMedSci., First Pavlov St-Petersburg State Medical University, Research Institute of Nephrology, director. Phone: (812) 338-69-01



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Review

For citations:


Dobronravov V.A., Smirnov A.V. ETIOLOGY AND CLINICO-MORPHOLOGICAL PRESENTATION OF MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS IN RUSSIAN POPULATION. Nephrology (Saint-Petersburg). 2018;22(4):9-17. (In Russ.) https://doi.org/10.24884/1561-6274-2018-22-4-9-17

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