[Systemic manifestations of primary Gougerot-Sjögren syndrome. Nature and incidence apropos of 34 cases]. / Manifestations systémiques du syndrome de Gougerot-Sjögren primaire. Nature et fréquence à propos de 34 cas.

ABSTRACT

OBJECTIVES: Assess the systemic manifestations observed in patients with primary Sjörgen's syndrome and the correlation between clinical findings and paraclinical data. METHODS: This retrospective study included patients who met the preliminary European criteria established in 1993. RESULTS: Among the 34 patients with primary Sjögren's syndrome (32 women, 2 men, mean age 54.9 years), systemic manifestations were observed as the inaugural sign in 25 (73.5%) and 31 cases (91%) during a mean 43-month follow-up. Clinical signs in 23 patients were neuropsychiatric disorders including depressive syndromes (n = 17) and peripheral neuropathies (n = 13), joint and muscle disorders (n = 16), Raynaud's phenomenon (n = 8), pulmonary disorders (n = 8), digestive disorders (n = 8) including two cases of chronic autoimmune hepatitis, skin disease (n = 7), and renal (n = 3) and thyroid (n = 8) disorders. Antinuclear antibodies were found in 20 cases and anti-SSA antibodies in 5 associated with anti-SSB in 4. Eight patients had hematologic disorders. There was a correlation between the number of systemic signs (at least 2) and positive Rose-Bengal test (p = 0.045). DISCUSSION: Different systemic manifestations were found at percentages comparable with data in the literature except for Raynaud's syndrome which was less frequent and neurological disorders which were more frequent.