Sjögren's syndrome with juvenile onset
Aim to analyze demographic data, clinical features and results of laboratory and instrumental examinations in children with primary and secondary Sjögren syndrome (SS). Materials and methods. The study included all consequently patients, who hospitalized to the pediatric department of V.A. Nasonova...
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | Russian |
| Published: |
"Consilium Medicum" Publishing house
2019-05-01
|
| Series: | Терапевтический архив |
| Subjects: | |
| Online Access: | https://ter-arkhiv.ru/0040-3660/article/viewFile/33601/pdf |
| _version_ | 1818577621912387584 |
|---|---|
| author | M I Kaleda I P Nikishina A N Latypova |
| author_facet | M I Kaleda I P Nikishina A N Latypova |
| author_sort | M I Kaleda |
| collection | DOAJ |
| description | Aim to analyze demographic data, clinical features and results of laboratory and instrumental examinations in children with primary and secondary Sjögren syndrome (SS). Materials and methods. The study included all consequently patients, who hospitalized to the pediatric department of V.A. Nasonova Scientific and Research Institute of Rheumatology from January 2013 to December 2018, which verified the diagnosis of the SS. Results. The diagnosis of SS was established in 30 patients, among whom there were only 5 (16.7%) boys, the ratio of boys and girls was 1:5. According to the results of the examination, the following diagnoses were verified: 4 - primary SS, 9 - systemic lupus erythematosus with SS, 10 - juvenile rheumatoid arthritis with SS, 3 - mixed connective tissue disease, 3 - overlap syndrome, 1 - systemic sclerosis with SS. The median age of rheumatic disease onset was 10.4 (7.0; 13.75) years. The median of disease duration at the time of SS verification - 3.0 (0.85; 4.4) years. Recurrent parotitis were observed in 8 patients. 24 pts had isolated involvement of salivary glands, 6 - combined with lacrimal glands. Sicca syndrome was occurred in 8 patients. All patients had systemic manifestations: constitutional abnormalities - 50%, polyarthritis - 83.3%, lymphadenopathy - 73.3%, cutaneous involvement - 60%, pulmonary involvement - 23.3%. Of the hematological disorders, leuko/lymphopenia was more often recorded - in 30%, polyclonal hypergammaglobulinemia - in 26.7% of patients. ANA were detected in all cases, anti-Ro antibodies - 60%, a positive rheumatoid factor - 56.7% of patients. The most common combination of immunological disorders was the presence of ANA, RF and anti-Ro antibodies (40% of patients). The treatment for each patient was justified by the main manifestations and activity of rheumatic disease: 66.7% received nonsteroidal anti - inflammatory drugs, 80% - glucocorticoids, 46.7% - methotrexate, 20% - azathioprine, 43.3% - hydroxychloroquine, 10% - mycophenolatis mofetilum, 3.4% - cyclophosphamide. 66.7% of patients received the treatment of Biologics. Conclusions. Early diagnosis of SS in children with rheumatic diseases significantly affects to the choice of treatment and prognosis. In children the SS has no clinical manifestations for a long time. Such symptoms as a hypergammaglobulinemia, positive RF without persistant arthritis, nonspecific skin lesions, recurrent parotid swelling may help to diagnosis of SS. |
| first_indexed | 2024-12-16T06:32:50Z |
| format | Article |
| id | doaj.art-3b6e3d4d246e4752b42d85634e0c40b3 |
| institution | Directory Open Access Journal |
| issn | 0040-3660 2309-5342 |
| language | Russian |
| last_indexed | 2024-12-16T06:32:50Z |
| publishDate | 2019-05-01 |
| publisher | "Consilium Medicum" Publishing house |
| record_format | Article |
| series | Терапевтический архив |
| spelling | doaj.art-3b6e3d4d246e4752b42d85634e0c40b32022-12-21T22:40:51Zrus"Consilium Medicum" Publishing houseТерапевтический архив0040-36602309-53422019-05-01915546010.26442/00403660.2019.05.00018930330Sjögren's syndrome with juvenile onsetM I Kaleda0I P Nikishina1A N Latypova2V.A. Nasonova Scientific and Research Institute of Rheumatology, Pediatric DepartmentV.A. Nasonova Scientific and Research Institute of Rheumatology, Pediatric DepartmentV.A. Nasonova Scientific and Research Institute of Rheumatology, Pediatric DepartmentAim to analyze demographic data, clinical features and results of laboratory and instrumental examinations in children with primary and secondary Sjögren syndrome (SS). Materials and methods. The study included all consequently patients, who hospitalized to the pediatric department of V.A. Nasonova Scientific and Research Institute of Rheumatology from January 2013 to December 2018, which verified the diagnosis of the SS. Results. The diagnosis of SS was established in 30 patients, among whom there were only 5 (16.7%) boys, the ratio of boys and girls was 1:5. According to the results of the examination, the following diagnoses were verified: 4 - primary SS, 9 - systemic lupus erythematosus with SS, 10 - juvenile rheumatoid arthritis with SS, 3 - mixed connective tissue disease, 3 - overlap syndrome, 1 - systemic sclerosis with SS. The median age of rheumatic disease onset was 10.4 (7.0; 13.75) years. The median of disease duration at the time of SS verification - 3.0 (0.85; 4.4) years. Recurrent parotitis were observed in 8 patients. 24 pts had isolated involvement of salivary glands, 6 - combined with lacrimal glands. Sicca syndrome was occurred in 8 patients. All patients had systemic manifestations: constitutional abnormalities - 50%, polyarthritis - 83.3%, lymphadenopathy - 73.3%, cutaneous involvement - 60%, pulmonary involvement - 23.3%. Of the hematological disorders, leuko/lymphopenia was more often recorded - in 30%, polyclonal hypergammaglobulinemia - in 26.7% of patients. ANA were detected in all cases, anti-Ro antibodies - 60%, a positive rheumatoid factor - 56.7% of patients. The most common combination of immunological disorders was the presence of ANA, RF and anti-Ro antibodies (40% of patients). The treatment for each patient was justified by the main manifestations and activity of rheumatic disease: 66.7% received nonsteroidal anti - inflammatory drugs, 80% - glucocorticoids, 46.7% - methotrexate, 20% - azathioprine, 43.3% - hydroxychloroquine, 10% - mycophenolatis mofetilum, 3.4% - cyclophosphamide. 66.7% of patients received the treatment of Biologics. Conclusions. Early diagnosis of SS in children with rheumatic diseases significantly affects to the choice of treatment and prognosis. In children the SS has no clinical manifestations for a long time. Such symptoms as a hypergammaglobulinemia, positive RF without persistant arthritis, nonspecific skin lesions, recurrent parotid swelling may help to diagnosis of SS.https://ter-arkhiv.ru/0040-3660/article/viewFile/33601/pdfchildhoodsjögren's syndrome in childrenrecurrent non-infectious parotitis |
| spellingShingle | M I Kaleda I P Nikishina A N Latypova Sjögren's syndrome with juvenile onset Терапевтический архив childhood sjögren's syndrome in children recurrent non-infectious parotitis |
| title | Sjögren's syndrome with juvenile onset |
| title_full | Sjögren's syndrome with juvenile onset |
| title_fullStr | Sjögren's syndrome with juvenile onset |
| title_full_unstemmed | Sjögren's syndrome with juvenile onset |
| title_short | Sjögren's syndrome with juvenile onset |
| title_sort | sjogren s syndrome with juvenile onset |
| topic | childhood sjögren's syndrome in children recurrent non-infectious parotitis |
| url | https://ter-arkhiv.ru/0040-3660/article/viewFile/33601/pdf |
| work_keys_str_mv | AT mikaleda sjogrenssyndromewithjuvenileonset AT ipnikishina sjogrenssyndromewithjuvenileonset AT anlatypova sjogrenssyndromewithjuvenileonset |