nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.36485/1561-6274-2021-25-2-73-78nefr-1946Research ArticleНАБЛЮДЕНИЯ ИЗ ПРАКТИКИPRACTICAL NOTESАНЦА-ассоциированный васкулит и IGG4-ассоциированная болезнь - одно или два заболевания?ANСA-associated vasculitis and IGG4-associated disease - the same or different diseases?https://orcid.org/0000-0002-5160-4512ПетросянЭ. К.PetrosyanE. K.

Проф. Петросян Эдита Константиновна, д-р мед. наук

119571, Россия, Москва, Ленинский пр. д. 117, корпус 6

Тел.: (903) 2270584

Prof. Edita K. Petrosyan, MD, PhD, DMedSci

119571, Russia, Moscow, Leninsky Prospect 117/ 6 bilding

Tel : (903) 2270584

Ed3565@yandex.ruГавриловаВ. А.GavrilovaV. A.

Гаврилова Валерия Аркадьевна, д-р мед. наук, врач-нефролог

119571, Россия, Москва, Ленинский пр. д. 117, корпус 6

Teл.: 8(916) 826-36-67

Gavrilova Valeria A., MD, PhD, DMedSci, nephrologist

119571, Russia, Moscow, Leninsky prospect, 117/ 6 building

Tel.: 8(916) 826-36-67

КушнирБ. Л.KushnirB. L.

Кушнир Берта Леонидовна, врач-патологоанатом

119571, Россия, Москва, Ленинский проспект д. 117, корпус 6

Тел.: 8(910) 401-60-63

Kushnir Berta L., pathologist

119571, Russia, Moscow, Leninsky prospect, 117/ 6 building

Tel.: 8(910) 401-60-63

ПовилайтитеП. Э.PovilaititeP. E.

Повилайтите Патриция Эдмундовна, канд. мед. наук, врач-патологоанатом

344015, г. Ростов-на-Дону, Благодатная, д. 170а

Тел.:8(918) 554-98-35

Povilaitite Patricia E., Ph.D. pathologist

344015, Rostov-on-Don, Blagodatnaya, 170a

Tel.: 8 (918) 554-98-35

Кафедра госпитальной педиатрии им. В.А. Таболина. Российский Национальный исследовательский медицинский университет им. Н.И. Пирогова; Российская детская клиническая больница Российского национального исследовательского медицинского университета им. Н.И. ПироговаРоссияV.A. Tabolin’s Department of Hospital Pediatrics of N.I. Pirogov’s Russian National Research Medical University; Russian Children's Clinical Hospital of N.I. Pirogov’s Russian National Research Medical Uni-versityRussian FederationРоссийская детская клиническая больница Российского национального исследовательского медицинского университета им. Н.И. ПироговаРоссияRussian Children's Clinical Hospital of N.I. Pirogov’s Russian National Research Medical Uni-versityRussian FederationПатологоанатомическое бюро Ростовской областиРоссияPathological Bureau of Rostov regionRussian Federation2021130220212527378Copyright © Петросян Э.К., Гаврилова В.А., Кушнир Б.Л., Повилайтите П.Э., 20212021Петросян Э.К., Гаврилова В.А., Кушнир Б.Л., Повилайтите П.Э.Petrosyan E.K., Gavrilova V.A., Kushnir B.L., Povilaitite P.E.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/1946

IgG4-ассоциированная болезнь (IgG4-АБ) представляет собой фибровоспалительное состояние, характеризующееся повышением сывороточного IgG4 и инфильтрацией IgG4-позитивными плазматическими клетками ткани пораженных органов. С точки зрения клинико-морфологических особенностей, существует значительный перекрест между IgG4-АБ и антинейтрофильными цитоплазматическими антителами (АНЦА) – ассоциированными васкулитами (AAВ). Положительный анализ на АНЦА считается весьма специфичным для ААВ и исключает другие формы системных заболеваний. Однако в ряде недавних исследований было продемонстрировано сочетание IgG4-АБ с позитивными АНЦА в сыворотке крови пациентов, что предполагает пересмотр роли АНЦА как диагностического критерия ААВ. В настоящей работе мы описываем клинический случай сочетания клинико-морфологических маркеров IgG4-АБ у пациентки, в сыворотке которой определялись позитивные АНЦА. У 8-летней пациентки отмечалось сочетание поражения легких и почек. Поражение почек проявлялось в виде пиелонефрита и нефротического синдрома. Гистологически в почках было выявлено сочетание тубулоинтерстициального нефрита и мембранозной нефропатии с выраженной инфильтрацией IgG4 в клубочке. Поиск серологических маркеров системных заболеваний выявил положительный тест на наличие АНЦА к миелопероксидазе, что предполагало течение у больной ААВ. Однако наличие мембранозной нефропатии, нехарактерной для морфологических изменений при ААВ, и выраженное отложение IgG4 в ткани почек предполагает другой патогенетический механизм развития заболевания у данной пациентки.

IgG4-related disease (IgG4-AD), is a fibro-inflammatory condition characterized by an increase of serum IgG4 and infiltration of the tissue of affected organs by IgG4-positive plasma cells. There is a significant cross between IgG4-AB and antineutrophilic cytoplasmic antibodies (ANCA) – associated vasculitis (AAV) because of clinical and morphological features of both pathologies. A positive ANCA test is considered highly specific for AAV and excludes other forms of systemic diseases. However, a number of recent publications have demonstrated a combination of IgG4-AD with positive serum ANCA in patients, which suggests a revision of the role of ANCA as a diagnostic criterion for AAV. In this work, we describe a clinical case of a combination of clinical and morphological markers of IgG4-AD and positive serum ANCA in an 8-year-old girl. She had a combination of lung and kidney injury. Kidney disease was manifested as pyelonephritis and incomplete nephrotic syndrome. Histologically, it was identified a combination of tubulointerstitial nephritis and membranous nephropathy, with severe IgG4 infiltration in the glomerulus. The search for serological markers of systemic diseases revealed a positive test for myeloperoxidase ANCA, which suggested the existence of AAV in the patient. However, the presence of membranous nephropathy, which is an uncharacteristic morphological pattern of AAV, and massive deposition of IgG4 in the kidney tissue suggests a different pathogenetic mechanism in this patient.

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