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Biol Sex Differ. 2017 Aug 8;8(1):25. doi: 10.1186/s13293-017-0146-6.

Long-term follow-up in primary Sjögren's syndrome reveals differences in clinical presentation between female and male patients.

Author information

1
Unit of Experimental Rheumatology, Department of Medicine, Karolinska University Hospital, Karolinska Institutet, SE-171 76, Stockholm, Sweden.
2
Division of Rheumatology, Department of Clinical Experimental Medicine, Linköping University, Linköping, Sweden.
3
Department of Rheumatology, Skåne University Hospital, Malmö, Sweden.
4
Clinical immunology unit, Department of Internal Medicine, Stavanger University Hospital, Stavanger, Norway.
5
Broegelmann Research Laboratory, Department of Clinical Science, University of Bergen, Bergen, Norway.
6
Department of Rheumatology, Haukeland University Hospital, Bergen, Norway.
7
Section for Oral and Maxillofacial Radiology, Department of Clinical Dentistry, University of Bergen, Bergen, Norway.
8
Gade Laboratory for Pathology, Department of Clinical Medicine, University of Bergen, Bergen, Norway.
9
Department of Pathology, Haukeland University Hospital, Bergen, Norway.
10
Department of Medical Sciences, Rheumatology and Science for Life Laboratory, Uppsala University, Uppsala, Sweden.
11
Department of Public Health and Clinical Medicine, Rheumatology, Umeå University, Umeå, Sweden.
12
Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.
13
Unit of Experimental Rheumatology, Department of Medicine, Karolinska University Hospital, Karolinska Institutet, SE-171 76, Stockholm, Sweden. Marie.Wahren@ki.se.

Abstract

BACKGROUND:

Despite men being less prone to develop autoimmune diseases, male sex has been associated with a more severe disease course in several systemic autoimmune diseases. In the present study, we aimed to investigate differences in the clinical presentation of primary Sjögren's syndrome (pSS) between the sexes and establish whether male sex is associated with a more severe form of long-term pSS.

METHODS:

Our study population included 967 patients with pSS (899 females and 68 males) from Scandinavian clinical centers. The mean follow-up time (years) was 8.8 ± 7.6 for women and 8.5 ± 6.2 for men (ns). Clinical data including serological and hematological parameters and glandular and extraglandular manifestations were compared between men and women.

RESULTS:

Male patient serology was characterized by more frequent positivity for anti-Ro/SSA and anti-La/SSB (p = 0.02), and ANA (p = 0.02). Further, men with pSS were more frequently diagnosed with interstitial lung disease (p = 0.008), lymphadenopathy (p = 0.04) and lymphoma (p = 0.007). Conversely, concomitant hypothyroidism was more common among female patients (p = 0.009).

CONCLUSIONS:

We observe enhanced serological responses and higher frequencies of lymphoma-related extraglandular manifestations in men with pSS. Notably, lymphoma itself was also significantly more common in men. These observations may reflect an aggravated immune activation and a more severe pathophysiological state in male patients with pSS and indicate a personalized managing of the disease due to the influence of the sex of patients with pSS.

KEYWORDS:

Autoimmunity; Disease severity; Extraglandular manifestations; Sex difference; Sjögren’s syndrome

PMID:
28789696
PMCID:
PMC5549313
DOI:
10.1186/s13293-017-0146-6
[Indexed for MEDLINE]
Free PMC Article

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