nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.36485/1561-6274-2020-24-4-110-121nefr-1866Research ArticleНАБЛЮДЕНИЯ ИЗ ПРАКТИКИPRACTICAL NOTESКлинический случай мембранозной нефропатии у больного с IgG4-ассоциированным заболеваниемClinical case of membranous nephropathy in a patient with IgG4-related diseasehttps://orcid.org/0000-0002-5893-3191ПятченковМ. О.PyatchenkovM. O.

Пятченков Михаил Олегович, канд. мед. наук, помощник начальника клиники нефрологии и эфферентной терапии по лечебной работе

194044, Санкт-Петербург, ул. Академика Лебедева, д. 6

Тел.: +7 (812) 5424314

Mikhail O. Pyatchenkov, PhD, Department of nephrology and blood purifi cation

194044, St. Petersburg,

Phone: +7 (812) 5424314

vmeda_12@mil.ruhttps://orcid.org/0000-0002-6946-6816ВоробьеваО. А.VorobyevaO. A.

Воробьева Ольга Алексеевна, канд. мед. наук

192283, Санкт-Петербург, ул. Олеко Дундича, д. 8, корп. 2.

Тел.: +7 (812) 244-02-50

Olga A. Vorobyeva, PhD

8 Oleko Dundich Str, Build 2, Saint Petersburg 192283

Phone: +7 (812) 244-02-50

olvorob70@gmail.comhttps://orcid.org/0000-0002-0421-3797БельскихА. Н.BelskykhA. N.

Бельских Андрей Николаевич, член-корреспондент РАН, профессор, заведующий кафедрой нефрологии и эфферентной терапии

194044, Санкт-Петербург, ул. Академика Лебедева, д. 6

Тел.: +7 (812) 5424314

Andrei N. Belskykh, professor, Corresponding Member, Russian Academy of Sciences, Department of nephrology and blood purifi cation

194044, St-Petersburg,

Phone: +7 (812) 5424314

vmeda_12@mil.ruhttps://orcid.org/0000-0001-6549-3991ЗахаровМ. В.ZakharovM. V.

Захаров Михаил Владимирович, канд. мед. наук, заместитель начальника клиники нефрологии и эфферентной терапии

194044, Санкт-Петербург, ул. Академика Лебедева, д. 6

Тел.: +7 (812) 5424314

Mikhail. V. Zakharov, PhD, Department of nephrology and blood purifi cation

194044, St-Petersburg,

Phone: +7 (812) 5424314

vmeda_12@mil.ruhttps://orcid.org/0000-0002-7068-7093ДендриковаМ. Ю.DendrikovaM. Y.

Дендрикова Марина Юрьевна, заведующая терапевтическим отделением клиники нефрологии и эфферентной терапии

194044, Санкт-Петербург, ул. Академика Лебедева, д. 6

Тел.: +7 (812) 5424314

Marina Y. Dendrikova, Department of nephrology and blood purifi cation

194044, St-Petersburg

Phone: +7 (812) 5424314

vmeda_12@mil.ruВоенно-медицинская академия им. С.М. КироваРоссияMilitary Medical AcademyRussian FederationНациональный центр клинической морфологической диагностикиРоссияNational Center of Clinical Morphological DiagnosticsRussian Federation202026062020244110121Copyright © Пятченков М.О., Воробьева О.А., Бельских А.Н., Захаров М.В., Дендрикова М.Ю., 20202020Пятченков М.О., Воробьева О.А., Бельских А.Н., Захаров М.В., Дендрикова М.Ю.Pyatchenkov M.O., Vorobyeva O.A., Belskykh A.N., Zakharov M.V., Dendrikova M.Y.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/1866

В настоящее время IgG4-ассоциированная болезнь (IgG4-АБ) рассматривается как хроническое мультисистемное иммуноопосредованное состояние неуточненной этиологии, естественное течение которого может имитировать онкологические, инфекционные, ревматологические и другие заболевания. При IgG4-АБ в патологический процесс синхронно или последовательно может быть вовлечен практически любой орган, однако наиболее часто поражаются поджелудочная железа, гепатобилиарный тракт, параорбитальные структуры, слюнные железы, почки, лимфатические узлы. IgG4-ассоциированный тубулоинтерстициальный нефрит является наиболее распространенным почечным проявлением IgG4-АБ. Среди гломерулярных поражений преобладает мембранозная нефропатия. Независимо от органной локализации для больных с IgG4-АБ характерно повышение сывороточного уровня IgG4, однако данный показатель не является специфичным и может быть изменен при других заболеваниях. Во всех случаях подозрения на IgG4-АБ диагноз должен быть подтвержден при гистологическом исследовании. К характерным гистопатологическим изменениям относятся диффузная или очаговая лимфоплазмоцитарная инфильтрация с IgG4-положительными плазматическими клетками, сториформный фиброз и облитерирующий флебит. Больные с IgG4-АБ обычно хорошо отвечают на лечение глюкокортикоидами, однако нередко отмечаются рецидивы заболевания, что может потребовать дополнительного использования иммуносупрессантов или ритуксимаба. В связи с невысокой встречаемостью и широкой вариабельностью клинических проявлений заболевание часто остается вовремя недиагностированным. В данной статье описан случай наблюдения молодого мужчины с хроническим рецидивирующим панкреатитом, течение которого осложнилось развитием нефротического синдрома. Морфологическое исследование нефробиоптата позволило не только уточнить генез гломерулярного поражения, но и диагностировать у пациента IgG4-АБ с поражением поджелудочной железы, почек, легких, лимфатических узлов. На фоне монотерапии глюкокортикостероидами достигнута частичная ремиссия заболевания. Представленный случай наглядно демонстрирует трудности диагностики и лечения IgG4-АБ. IgG4- ассоциированная мембранозная нефропатия должна быть включена в дифференциальный диагностический перечень в каждом случае нефротического синдрома, сопровождающегося мультиорганными дисфункциями.

IgG4-related disease (IgG4-RD) currently is considered as a chronic fibroinflammatory immune-mediated multisystemic condition of unidentified etiology, which can imitate a wide range of malignant, infectious, rheumatologic, and other diseases. It can affect almost any organ system in the body synchronously or sequentially, but the most often affected are the pancreas, hepatobiliary tract, periorbital structures, salivary glands, kidneys, and lymph nodes. The most frequent renal manifestations of IgG4-RD is IgG4-related tubulointerstitial nephritis. Membranous nephropathy is the most common glomerular disease accompanied by IgG4-RD. Regardless of the organ localization, patients with IgG4-RD are characterized by elevated serum IgG4, but this laboratory abnormality is not specific and can be changed in other diseases. In all suspected cases of IgG4-RD the diagnosis should be confirmed by histological examination. Characteristic pathologic features include diffuse or focal lymphoplasmacytic infiltration with prominent IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis. Patients with IgG4- RD usually have an excellent clinical response to glucocorticoids, but relapse rates after steroid withdrawal are high, which may require additional use of immunosuppressants or rituximab. Due to the low prevalence and multitude of clinical manifestations the disease often remains underdiagnosed on time. This case report describes middle-aged patients with a history of chronic recurrent pancreatitis complicated by the nephrotic syndrome. Kidney biopsy showed membranous nephropathy and diagnosis IgG4-RD with multiorgan involvement was made. Partial remission was achieved on corticosteroid therapy. The presented case clearly demonstrates the difficulties of diagnosis and treatment of IgG4-RD. IgG4-related membranous nephropathy should be included in the differential diagnosis for patients with nephrotic syndrome accompanied by multiorgan dysfunction.

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