Background/Purpose:

Primary Sjögren’s syndrome (pSS) and SLE are chronic autoimmune diseases that predominantly affect women, with a female:male ratio of approximately 9:1. SLE in men is more severe than in women, but studies of sex-related clinical features in pSS have given ambiguous results. In this study, we therefore investigated the clinical presentation of pSS in men and women in a cohort of pSS at time of diagnosis, all treatment naïve cases collected in a population-based manner during a 5-year period.

Methods:

Patients represent incident cases of pSS in Stockholm County from January 1, 2007 to December 31, 2011, and were diagnosed at the Department of Rheumatology at the Karolinska University Hospital, Stockholm, Sweden (women n=136, men n=10). Patients were examined according to an established protocol including clinical and laboratory investigations and all fulfilled the 2002 revised American–European Consensus Criteria (AECC). Extraglandular manifestations (EGM) were defined as those included in the EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI). Serum was sampled at the time of diagnosis, anti-Ro52 levels were measured by ELISA and a cut off at 80 AU was used for categorization into high and low levels. The Chi-square test was used for analyzing frequencies of patient subgroups and prevalence of EGM, and the Mann-Whitney U-test was used when comparing autoantibody levels and age at diagnosis between female and male patients with pSS.

Results:

Male patients were younger at the time of pSS diagnosis than female patients (48.0 ±12.2 versus 57.0 ±14.1, p=0.04). No difference was observed for frequencies of Ro and La autoantibodies, but male patients with pSS displayed a tendency towards higher Ro52-autoantibody levels (p=0.06), and more male patients had high levels (p=0.03). Notably, cutaneous vasculitis was more common in male patients with pSS (p<0.001), and pulmonary disease in terms of interstitial lung disease and alveolitis occurred more often in male patients with pSS (p<0.001 and p<0.001). No EGM was more common in female pSS patients. Further, concomitant presentation of EGM was more common in male than in female patients (p=0.02).

Conclusion:

There are differences in the clinical presentation of pSS between the sexes. Our study, based on a cohort of incident, treatment naïve cases collected in a population-based manner during a 5-year period, indicates that disease onset is earlier in men, and that the immune-activity is higher. Importantly, half of the male patients presented one or more EGM at diagnosis, supporting the conclusion that pSS in men represents a more severe form of disease, regardless of the lower risk for males to develop the disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/sjogrens-syndrome-in-male-patients-presents-with-higher-autoantibody-levels-and-more-extraglandular-manifestations/