Clinical and laboratory characteristics of IgG4-realated disease and its diagnostic algorithm
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| 1. | Title | Title of document | Clinical and laboratory characteristics of IgG4-realated disease and its diagnostic algorithm |
| 2. | Creator | Author's name, affiliation, country | E V Sokol; V.A. Nasonova Scientific and Research Institute of Rheumatology |
| 2. | Creator | Author's name, affiliation, country | V I Vasilyev; V.A. Nasonova Scientific and Research Institute of Rheumatology |
| 2. | Creator | Author's name, affiliation, country | S G Palshina; V.A. Nasonova Scientific and Research Institute of Rheumatology |
| 2. | Creator | Author's name, affiliation, country | N V Kokosadze; N.N. Blokhin National Medical Research Centre of Oncology” of the Health Ministry of Russia |
| 2. | Creator | Author's name, affiliation, country | N A Probatova; N.N. Blokhin National Medical Research Centre of Oncology” of the Health Ministry of Russia |
| 2. | Creator | Author's name, affiliation, country | A M Kovrigina; National Research Center for Hematology |
| 2. | Creator | Author's name, affiliation, country | T N Safonova; Research Institute of eye diseases |
| 2. | Creator | Author's name, affiliation, country | E B Rodionova; V.A. Nasonova Scientific and Research Institute of Rheumatology |
| 2. | Creator | Author's name, affiliation, country | I V Gaiduk; A.I. Yevdokimov Moscow State University of Medicine and Dentistry |
| 2. | Creator | Author's name, affiliation, country | E I Selifanova; I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University) |
| 3. | Subject | Discipline(s) | |
| 3. | Subject | Keyword(s) | IgG4-related disease; IgG4-related sialoadenitis; IgG4-related dacryoadenitis; retroperitoneal fibrosis; diagnostic algorithm; monoclonal secretion; MALT-lymphoma |
| 4. | Description | 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. |
| 5. | Publisher | Organizing agency, location | LLC Obyedinennaya Redaktsiya |
| 6. | Contributor | Sponsor(s) | |
| 7. | Date | (DD-MM-YYYY) | 15.05.2019 |
| 8. | Type | Status & genre | Peer-reviewed Article |
| 8. | Type | Type | Research Article |
| 9. | Format | File format | |
| 10. | Identifier | Uniform Resource Identifier | https://ter-arkhiv.ru/0040-3660/article/view/33589 |
| 10. | Identifier | Digital Object Identifier (DOI) | 10.26442/00403660.2019.05.000236 |
| 11. | Source | Title; vol., no. (year) | Terapevticheskii arkhiv; Vol 91, No 5 (2019) |
| 12. | Language | English=en | ru |
| 13. | Relation | Supp. Files | |
| 14. | Coverage | Geo-spatial location, chronological period, research sample (gender, age, etc.) | |
| 15. | Rights | Copyright and permissions |
Copyright (c) 2019 Consilium Medicum This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License. |