Clinical and laboratory characteristics of IgG4-realated disease and its diagnostic algorithm


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Abstract

Aim: to propose diagnostic algorithm of IgG4-related disease (IgG4-RD). Materials and methods. One center retrospective research. 52 pts with IgG4-RD were included. The diagnosis was proved histologically and immunohistochemically. 48 out of 52 pts received treatment. Treatment included one of the following schemes (along with low oral glucocorticoids): rituximab monotherapy, cyclophosphamide monotherapy or their combination. Results. The mean age was 47.4±5.9 years, the mean age of the disease onset was 43.9±16.0 years. Median time before the diagnosis was 24 months. The most often sites of IgG4-RD were lacrimal (63.5%), salivary (46.2%) glands, lungs (48%), lymph nodes (34.6%) and retroperitoneum (17.3%). In clinical picture the leading complain was organ enlargement, but not its dysfunction. Pain was characteristic for retroperitoneum localization. In 56.8% of pts with IgG4-related syalo - and/or dacryoadenitis there was association with ear - nose - throat organs affection. In 4 pts (7.7%) IgG4-RD was combined with some malignant disease, including MALT-lymphoma of lacrimal glands. Irreversible organ damage as an IgG4-RD outcome had 15.4% of pts. The main laboratory markers of IgG4-RD were ESR elevation (38.5%), blood eosinophilia (9.6%), immunological disturbances (serum total IgG and IgG4 elevation, IgE elevation, antinuclear antibodies, rheumatoid factor detection, hypocomplementemia). Serum IgG4 level >1.35 g/l was elevated in 88% of pts and correlated with the number of affected organs (Spearman correlation coefficient 0.39, Student’s test, р=0.0056). Monoclonal serum secretion and B-cell clonality in the tissue was detected in 4 (23.5%) out of 17 pts, but not all of them had both signs. Conclusion. Based on the analysis of clinical and laboratory characteristics of IgG4-RD a diagnostic algorithm was proposed that enhances the detection and examination of the patients with suspected IgG4-RD.

About the authors

E V Sokol

V.A. Nasonova Scientific and Research Institute of Rheumatology

Email: name.sokol@gmail.com
к.м.н., н.с. лаб. интенсивных методов терапии ревматических заболеваний ФГБНУ «НИИР им. В.А. Насоновой», тел.: 8(926)563-83-93, e-mail: name.sokol@gmail.com; ORCID: 0000-0002-2191-9361 Москва, Россия

V I Vasilyev

V.A. Nasonova Scientific and Research Institute of Rheumatology

м.н., проф., в.н.с. лаб. интенсивных методов терапии ревматических заболеваний ФГБНУ «НИИР им. В.А. Насоновой» Москва, Россия

S G Palshina

V.A. Nasonova Scientific and Research Institute of Rheumatology

к.м.н., м.н.с. лаб. интенсивных методов терапии ревматических заболеваний ФГБНУ «НИИР им. В.А. Насоновой» Москва, Россия

N V Kokosadze

N.N. Blokhin National Medical Research Centre of Oncology” of the Health Ministry of Russia

к.м.н., врач патологоанатомического отд-ния ФГБНУ «РОНЦ им. Н.Н. Блохина» Минздрава России Москва, Россия

N A Probatova

N.N. Blokhin National Medical Research Centre of Oncology” of the Health Ministry of Russia

д.б.н., в.н.с. отд. патологической анатомии опухолей ФГБНУ «РОНЦ им. Н.Н. Блохина» Минздрава России Москва, Россия

A M Kovrigina

National Research Center for Hematology

д.б.н., проф., зав. патологоанатомического отд-ния ФГБНУ «ГНЦ» Минздрава России Москва, Россия

T N Safonova

Research Institute of eye diseases

к.м.н., в.н.с. отд-ния терапевтической офтальмологии и офтальмофармакологии ФГБНУ «НИИ глазных болезней» Москва, Россия

E B Rodionova

V.A. Nasonova Scientific and Research Institute of Rheumatology

к.м.н., врач-стоматолог ФГБНУ «НИИР им. В.А. Насоновой» Москва, Россия

I V Gaiduk

A.I. Yevdokimov Moscow State University of Medicine and Dentistry

к.м.н., доцент каф. хирургии полости рта ГБОУ ВПО «МГСМУ им. А.И. Евдокимова» Москва, Россия

E I Selifanova

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University)

к.м.н., врач-стоматолог, доцент каф. терапевтической стоматологии ФГАОУ ВО «Первый МГМУ им. И.М. Сеченова» Минздрава России (Сеченовский Университет) Москва, Россия

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