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

Autor:	Sokol EV; V.A. Nasonova Scientific and Research Institute of Rheumatology., Vasilyev VI; V.A. Nasonova Scientific and Research Institute of Rheumatology., Palshina SG; V.A. Nasonova Scientific and Research Institute of Rheumatology., Kokosadze NV; N.N. Blokhin National Medical Research Centre of Oncology' of the Health Ministry of Russia., Probatova NA; N.N. Blokhin National Medical Research Centre of Oncology' of the Health Ministry of Russia., Kovrigina AM; National Research Center for Hematology., Safonova TN; Research Institute of eye diseases., Rodionova EB; V.A. Nasonova Scientific and Research Institute of Rheumatology., Gaiduk IV; A.I. Yevdokimov Moscow State University of Medicine and Dentistry., Selifanova EI; I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University).
Jazyk:	ruština
Zdroj:	Terapevticheskii arkhiv [Ter Arkh] 2019 May 15; Vol. 91 (5), pp. 40-48. Date of Electronic Publication: 2019 May 15.
DOI:	10.26442/00403660.2019.05.000236
Abstrakt:	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.
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu