nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное общество10.28996/1680-4422-2018-1-92-99nid-327Research ArticleНАБЛЮДЕНИЯ ИЗ ПРАКТИКИCASE REPORTSПоражение почек при IgG4-ассоциированной болезни (Клиническое наблюдение)A clinical case of IgG4-releated diseaseВолошиноваЕ. В.VoloshinovaE. V.voloshinovaelena@mail.ruВоробьеваО. А.VorobyevaO. A.noemail@neicon.ruЦатуроваК. Н.TsaturovaK. N.noemail@neicon.ruКафедра госпитальной терапии ФГБОУ ВО "Саратовский государственный медицинский университет имени В. И. Разумовского" Минздрава РоссииРоссияChair of hospital therapy, V.I. Razumovsky Saratov State Medical UniversityRussian FederationООО "Национальный центр клинической морфологической диагностики"РоссияNational Center of Clinical Morphological DiagnosticsRussian Federation2018120820242019299Copyright © Волошинова Е.В., Воробьева О.А., Цатурова К.Н., 20242024Волошинова Е.В., Воробьева О.А., Цатурова К.Н.Voloshinova E.V., Vorobyeva O.A., Tsaturova K.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/327

IgG4-ассоциированная болезнь (IgG4-АБ) - системное заболевание, которое характеризуется воспалением и фиброзом и поражает практически все системы и органы. В патофизиологии IgG4-АБ играет роль гуморальный и клеточный иммунитет, но механизмы этого процесса на настоящий момент до конца не установлены. Самыми частыми "мишенями" являются органы билиодигестивной зоны и почки. IgG4-ассоциированный тубуло-интерстициальный нефрит (ТИН) - самый частый вариант поражения почек при данном заболевании. В статье представлен случай IgG4-АБ у молодого пациента, дебютировавшей клиникой сахарного диабета и острого почечного повреждения. Диагноз IgG4-АБ был верифицирован морфологически: в биоптате почки определялась характерная густая интерстициальная инфильтрация, представленная плазматическими клетками с увеличением количества IgG4-позитивных клеток. С учетом полученных данных у пациента было констатировано развитие IgG4-ассоциированного поражения почек в виде острого ТИН и поджелудочной железы в виде аутоиммуного панкреатита с формированием вторичного сахарного диабета. Согласно международным стандартам терапии IgG4-АБ пациенту были назначены системные глюкокортикоиды. На этом фоне отмечалось полное восстановление почечной функции, однако нормализации инкреторной функции поджелудочной железы не произошло.

IgG4-associated disease (IgG4-AD) is a systemic disease characterized by inflammation and fibrosis that affects almost all systems and organs. Humoral and cellular immunity plays some role in the pathophysiology of IgG4-AD, but the mechanism of this process is currently not fully understood. The most frequent targets are the organs of the biliodigestive zone and kidneys. IgG4-associated tubulo-interstitial nephritis (TIN) is the most common variant of kidney damage associated with this disease. The article presents a case of IgG4-AD in a young patient which debuted with diabetes mellitus and acute renal injury. The diagnosis of IgG4-AD was verified morphologically: the characteristic dense interstitial infiltration represented by plasma cells with an increase in the number of IgG4-positive cells were detected in kidney biopsy. Taking into account the obtained data, the patient was diagnosed with the development of IgG4-associated renal lesions in the form of acute TIN and pancreatic gland in the form of autoimmune pancreatitis with the formation of secondary diabetes mellitus. According to the international standards of IgG4-AD therapy, systemic glucocorticoids were prescribed. As the result, there was a complete restoration of renal function, but a normalization of endocrine function of the pancreas was no achieved.

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