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

Abstract

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>