Background/Purpose: Primary Sjögren syndrome (pSS) is a systemic autoimmune disease involving primarily the exocrine glandular system. pSS in men is uncommon. Data of pSS in male are controversial. Objectives: The aim of our study was to describe the epidemiological, clinical and serological features of male patients with pSS in a spanish pSS cohort (SJOGRENSER Registry)

Methods: This is a multicenter descriptive transversal study of pSS patients fulfilling European/American consensus criteria, from thirty-three Rheumatology departments. Patients were included by randomisation from an anonymized list provided by every department. Data were collected by reviewing clinical records and interviewing the patients. Informed consent was obtained and local ethics committees approved the study. Variables were analyzed by descriptive statistical methods, using means, medians, and rates, with their deviations and interquartile ranges (p25-p75).

Results: Four hundred and thirty-seven patients with pSS were included. Twenty-one men were identified. The ratio between women and men was 20:1. Median age at the time of the first symptoms was 53 years for men compared with 46 years for women (p=0.026). Median age at the time at pSS diagnosis was 56 years for men and 50 years for women (p=0.041). Twenty nine percent of men had a family history of autoimmune disease, most commonly pSS (40%). All male patients had ocular and oral dry symptoms.The most frecuent extraglandular manifestations in men were hematological involvement (48%) followed by upper airways involvement (38%), asthenia (38%), salivary gland enlargement (33%) and articular involvement (33%). A significant difference between men and women was found in the prevalence of asthenia, 38% for men and 64% for women (p=0.015), and genitourinary manifestations, 5% for men vs 51% for women (p=0.001). Systemic features, salivary gland enlargement, lymphadenopathy, vasculitis, peripheral neuropathy, pulmonary, cardiovascular, central nervous system and pancreatic manifestations tended to be more frequent in male patients, without statistical significance. The most frequent antibodies in male were ANA (95%), Anti-Ro/SS-A (95%), anti-La/SS-B (67%) and RF (76%). No significant differences in serological characteristics were found between men and women.Median score for the EULAR Sjogren´s Syndrome Disease Activity Index (ESSDAI) was 6.5, for Sjogren´s Syndrome Disease Activity Index (SSDAI) was 1.76, for EULAR Sjogren´s Syndrome Patient Reported Index (ESSPRI) was 4.35, and for Sjogren´s Syndrome Disease Damage Index (SSDDI) was 2.67.ESSDAI and SSDDI tended to be more frequent in males patients compared with women. Morbidities such as hypertension (33%) and diabetes mellitus (10%) tended to be more frequent in male patients compared with women.

Conclusion: The median age at onset and diagnosis of pSS is higher in men. All pSS male patients showed ocular and oral dry symptoms. The most frecuent extraglandular manifestations in men were hematological involvement, upper airways involvement, asthenia, salivary gland enlargement and articular involvement. Most men had ANA and antiRo antibodies. The disease activity and damage indexes were higher in males.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-and-immunological-characteristics-of-primary-sjogrens-syndrome-in-men/