Features Of Sjögren's Syndrome Research Articles

Glandular symptoms always not the presenting features of Sjogrens syndrome: Report of 2 cases of sjogrens syndrome with interesting extra glandular presentations

Glandular symptoms always not the presenting features of Sjogrens syndrome: Report of 2 cases of sjogrens syndrome with interesting extra glandular presentations

Role of complement and B lymphocytes in Sjögren's syndrome-like autoimmune exocrinopathy of NOD.B10- H2b mice

Sjögren's syndrome (SjS) is a human autoimmune disease characterized by the loss of exocrine function as a result of a chronic immune attack directed primarily against the salivary and lacrimal glands leading to xerostomia (dry mouth) and xerophthalmia (dry eyes). NOD.B10- H2 b mice manifest many features of SjS, exhibiting exocrine gland dysfunction concomitant with leukocyte infiltration of the salivary and lacrimal glands. Recent studies have shown that both SjS patients and NOD.B10- H2 b mice exhibit increased B lymphocyte survival, B cell hyper-reactivity and hyper-gammaglobulinemia with high production of autoantibodies. To study the possible influence of complement on the development and expression of SjS-like disease of the NOD.B10- H2 b , we have utilized a prophylactic treatment with CVF known to interfere with the action of complement C3 factor. NOD.B10- H2 b mice, injected with CVF starting at 10 weeks of age, a time when leukocyte infiltration is expected to begin, failed to develop salivary dysfunction out to 24 weeks of age, a time when reduced salivary flow rates are known to occur in non-treated animals. Concomitant with retention of salivary exocrine function, CVF-treated mice showed reduced levels of leukocytic infiltration, reduction of anti-nuclear autoantibodies and major alterations in the B lymphocyte profiles while maintaining general pathophysiological measures of disease. These data suggest that C3 plays a significant role in development and onset of SjS-like disease, yet additional studies need to be carried out to identify the precise mode of action.

Haematological manifestations of primary sjogren’s syndrome: A clinopathological study

Clinically significant cytopenias are thought to be uncommon in primary Sjogren's Syndrome (SjS) with only a few case reports of patients with immuno thrombocytopenia, haemolytic anaemia and neutropenia. During the past three years, we have identified haematological abnormalities in ten of the 26 patients with SjS, the latter diagnosis was based on the Copenhagen criteria - keratoconjunctivitis sicca: Schirmer's test Tear Breakup Time, Rose Bengal Staining; Xerostomia: lower lip biopsy; exclusion of other chronic inflammatory connective tissue disease. All the 26 patients had ANA, ENA, SS-A, SS-B tests performed. Three patients had immune thrombocytopenia; three had a positive coombs test (IgG only), one with features of positive haemolysis; two had lideroblastic anaemia, one with a positive coombs test; one each with pure red cell aplasia, aplastic anaemia and neutropenia. Response to therapy of immune cytopenias was comparable to that seen in patients with idiopathic immune cytopenias. Sicca symptoms were present in eight patients out of ten; in five, it preceded and in three it followed the haematological abnormality. <h3>Conclusions</h3> <ul><li>1.Haematological manifestations are common (40%) in patients with SjS.</li><li>2.Features of SjS should be looked for in patients with immune cytopenias and, ANA as well as ENA should be performed to rule in/out SjS.</li></ul>