Clinical Course, Prognosis, and Cause of Death in Primary Sjögren’s Syndrome
The aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosed with primary Sjögren’s syndrome (pSS) between 1975 and 2010. The patients were followed up for 11.4±6.2 years. We evaluated the clinical and labor...
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| Format: | Article |
| Language: | English |
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Wiley
2014-01-01
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| Series: | Journal of Immunology Research |
| Online Access: | http://dx.doi.org/10.1155/2014/647507 |
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| author | Ildiko Fanny Horvath Antonia Szanto Gabor Papp Margit Zeher |
| author_facet | Ildiko Fanny Horvath Antonia Szanto Gabor Papp Margit Zeher |
| author_sort | Ildiko Fanny Horvath |
| collection | DOAJ |
| description | The aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosed with primary Sjögren’s syndrome (pSS) between 1975 and 2010. The patients were followed up for 11.4±6.2 years. We evaluated the clinical and laboratory features, and assessed their influence on the time of diagnosis, survival, and mortality ratios, and compared them within subgroups defined by gender, glandular and extraglandular manifestations (EGMs), associated diseases, and immunoserological abnormalities. The most frequent EGMs were polyarthritis, Raynaud’s phenomenon, and vasculitis among our patients; the most common associated disease was thyroiditis. During the follow-up period, 51 patients died; the median survival time was 33.71 years. Our results revealed a negative effect of cryoglobulinemia on survival ratios; additionally, the presence of vasculitis and lymphoproliferative diseases at the time of diagnosis increased the risk of mortality. The development of vasculitis was the most powerful predictor of mortality. Mortality in the group of patients with extraglandular symptoms was two- to threefold higher than in the glandular group. Attention is drawn to the importance of close monitoring and targeted diagnostic approaches in those pSS subgroups with obviously increased mortality risk. |
| format | Article |
| id | doaj-art-b9ea81cfbd864ea4aff4b2e978b84c93 |
| institution | Kabale University |
| issn | 2314-8861 2314-7156 |
| language | English |
| publishDate | 2014-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of Immunology Research |
| spelling | doaj-art-b9ea81cfbd864ea4aff4b2e978b84c932025-02-03T06:42:04ZengWileyJournal of Immunology Research2314-88612314-71562014-01-01201410.1155/2014/647507647507Clinical Course, Prognosis, and Cause of Death in Primary Sjögren’s SyndromeIldiko Fanny Horvath0Antonia Szanto1Gabor Papp2Margit Zeher3Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zsigmond Street 22, Debrecen 4032, HungaryDivision of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zsigmond Street 22, Debrecen 4032, HungaryDivision of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zsigmond Street 22, Debrecen 4032, HungaryDivision of Clinical Immunology, Faculty of Medicine, University of Debrecen, Móricz Zsigmond Street 22, Debrecen 4032, HungaryThe aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosed with primary Sjögren’s syndrome (pSS) between 1975 and 2010. The patients were followed up for 11.4±6.2 years. We evaluated the clinical and laboratory features, and assessed their influence on the time of diagnosis, survival, and mortality ratios, and compared them within subgroups defined by gender, glandular and extraglandular manifestations (EGMs), associated diseases, and immunoserological abnormalities. The most frequent EGMs were polyarthritis, Raynaud’s phenomenon, and vasculitis among our patients; the most common associated disease was thyroiditis. During the follow-up period, 51 patients died; the median survival time was 33.71 years. Our results revealed a negative effect of cryoglobulinemia on survival ratios; additionally, the presence of vasculitis and lymphoproliferative diseases at the time of diagnosis increased the risk of mortality. The development of vasculitis was the most powerful predictor of mortality. Mortality in the group of patients with extraglandular symptoms was two- to threefold higher than in the glandular group. Attention is drawn to the importance of close monitoring and targeted diagnostic approaches in those pSS subgroups with obviously increased mortality risk.http://dx.doi.org/10.1155/2014/647507 |
| spellingShingle | Ildiko Fanny Horvath Antonia Szanto Gabor Papp Margit Zeher Clinical Course, Prognosis, and Cause of Death in Primary Sjögren’s Syndrome Journal of Immunology Research |
| title | Clinical Course, Prognosis, and Cause of Death in Primary Sjögren’s Syndrome |
| title_full | Clinical Course, Prognosis, and Cause of Death in Primary Sjögren’s Syndrome |
| title_fullStr | Clinical Course, Prognosis, and Cause of Death in Primary Sjögren’s Syndrome |
| title_full_unstemmed | Clinical Course, Prognosis, and Cause of Death in Primary Sjögren’s Syndrome |
| title_short | Clinical Course, Prognosis, and Cause of Death in Primary Sjögren’s Syndrome |
| title_sort | clinical course prognosis and cause of death in primary sjogren s syndrome |
| url | http://dx.doi.org/10.1155/2014/647507 |
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