Background: Primary Sjogren’s syndrome (PSS) is characterized as an autoimmune exocrinopathy, but this disorder also may involve extraglandular organs, giving rise to multisystem disease. Although the first case reported by Dr Mikulicz was a 42 year old man [1], there are only few reports describing the clinical picture of Sjogren’s Syndrome in males

Objectives: To determine whether there were any clinical and biological differences between male and female tunisian patients with primary Sjogren’s syndrome (PSS).

Methods: We studied 60 patients with pSS according to American-European Consensus Group (AECG), attending an Internal Medicine departement in Tunisia. The nine (15%) male patients in this cohort comprised the male group described in this study

Results: The average age of men at diagnosis was 53.2 years [31 to 73 years]. Male SSp was revealed by sicca symptoms (55.5%), by extra glandular manifestations (33.3%). Fourty-four percent of our male patients had a positive result for a minor salivary gland biopsy (grade III or IV).

Extraglandular manifestations during the course of the disease were present in 6 cases (66%) of our male patients with pSS. weight loss and raynaud’s phenomenon were more common in men. a significantly greater percentage of men had neurological involvement (66.6%vs 23,7) and and pulmonary involvement, notably a restrictive syndrome (66.6% vs 31,1%, p=0.043). The positivity of Anti-nuclear antibodies, anti-SSb and rheumatoid factor were significantly less common in males.

Anemia was significanty more common in the male patients (p=0,049). No cases of Lymphoma in the male patients in our series.

Eighty-eight percent of the men were treated with corticosteroids. In our series, the use of immunosuppressants was not necessary in male patients.

Conclusion: Although pSS is typically a disease affecting women, clinicians should be aware that it may be diagnosed in male patients. Although men were at the same risk for the development of extraglandular complications, there were significant serologic and immunogenetic differences. In Sharp contrast to women with Sjogren’s syndrome, men with Sjogren’s syndrome were more seronegative in our series.

REFERENCES:

[1]Mikulicz J. Discussion at Verein fur wissenschaftliche Heilkunde zu königsberg. Berl Klin Wocchenschr 1888; 25:759.

[2]Gondran G, Fauchais A, Lambert M, Ly K, Launay D, Queyrel V, et al. Primary Sjogren’s syndrome in men. Scandinavian journal of rheumatology. 2008;37(4):300-5.

[3]Virdee S, Greenan-Barrett J, Ciurtin C. A systematic review of primary Sjogren’s syndrome in male and paediatric populations. 2017;36(10):2225-36

Disclosure of Interests: None declared