nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.24884/1561-6274-2018-22-4-9-17nefr-488Research ArticleПЕРЕДОВАЯ СТАТЬЯLEADING ARTICLEЭТИОЛОГИЯ И КЛИНИКО-МОРФОЛОГИЧЕСКАЯ ПРЕЗЕНТАЦИЯ МЕМБРАНОПРОЛИФЕРАТИВНОГО ГЛОМЕРУЛОНЕФРИТА В РОССИЙСКОЙ ПОПУЛЯЦИИETIOLOGY AND CLINICO-MORPHOLOGICAL PRESENTATION OF MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS IN RUSSIAN POPULATIONhttps://orcid.org/0000-0002-7179-5520ДобронравовВ. А.DobronravovV. A.

197022, Санкт-Петербург, ул. Л. Толстого, д. 17

Проф. Добронравов Владимир Александрович, д-р мед. наук Кафедра  пропедевтики внутренних болезней, заместитель директора НИИ нефрологии по  научной работе. Тел.: +7(812)338-69-16

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

dobronravov@nephrolog.ruСмирновА. В.SmirnovA. V.

197022, Санкт-Петербург, ул. Л. Толстого, д. 17

Проф. Смирнов Алексей Владимирович, д-р мед. наук Заведующий кафедрой  пропедевтики внутренних болезней, директор НИИ нефрологии. Тел.: +7(812)338-69-01

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

Научно-исследовательский институт нефрологии Первого Санкт-Петербургского государственного медицинского университета им. акад. И.П. ПавловаРоссияResearch Institute of Nephrology, First Pavlov Saint-Petersburg State Medical UniversityRussian Federation201821072018224917Copyright © Добронравов В.А., Смирнов А.В., 20182018Добронравов В.А., Смирнов А.В.Dobronravov V.A., Smirnov A.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephrolog.ru/jour/article/view/488ЦЕЛЬ

ЦЕЛЬ. Анализ этиологических факторов и клинико-морфологических проявлений синдрома мембранопролиферативного гломерулонефрита (МПГН).

ПАЦИЕНТЫ И МЕТОДЫ

ПАЦИЕНТЫ И МЕТОДЫ. Ретроспективный анализ случаев МПГН, выявленных в период  2000–2017 гг. в контексте этиологии, основной клинической симптоматики и  гистологических изменений [по данным светооптической и электронной микроскопии,  иммунофлюоресцентного исследования депозитов иммуноглобулинов (Ig) и С3-фракции комплемента (С3)].

РЕЗУЛЬТАТЫ

РЕЗУЛЬТАТЫ. В исследование включено 214 случаев МПГН. Средний возраст пациентов –  44±16 лет. Нефротический синдром выявлен у 72% больных; в 58,4% случаев рСКФ была <60 мл/мин/1,73 м2. Распространенность случаев МПГН среди морфологически  подтвержденных гломерулопатий составила 9,3%. Идиопатический (иМПГН) выявлен в  30,4% случаев, вторичный МПГН (вМПГН) – в 69,6% (аутоиммунные заболевания – 34,1%, инфекционные – 16,4%, моноклональные гаммапатии – 9,3%, комплемент-опосредованныеповреждения – 9,8%). Ig+C3+МПГН чаще выявляли на фоне аутоиммунных заболеваний и инфекций, вместе с тем, 26,6% таких случаев остались «идиопатическими». В большинстве  случаев Ig–C3+МПГН был установлен диагноз С3-гломерулопатии или тромботической микроангиопатии. Этиология Ig–C3-/Ig+C3–МПГН была гетерогенна.

ЗАКЛЮЧЕНИЕ

ЗАКЛЮЧЕНИЕ. Синдром МПГН представляет собой тяжелый вариант повреждения клубочков с гетерогенной этиологической структурой. Целенаправленная клинико- морфологическая диагностика позволяет идентифицировать причину МПГН в большинстве  случаев, что является основой для выбора адекватного лечения и улучшения исходов.  Дальнейшее совершенствавание диагностических и классификационных подходов при  иМПГН зависит от прогресса в понимании молекулярных механизмов этиопатогенеза болезни.

THE AIM

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

PATIENTS AND METHODS

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

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.

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The authors declare that there are no conflicts of interest present.