Immune cytopenias as the presenting finding in primary Sjogren's syndrome

A diagnostic delay of several years in primary Sjogren's syndrome is common , even in patients who present with sicca symptoms. It is much more likely in cases with prominent symptomatic extraglandular involvement. We report o n three such patients who presented as Coomb's positive haemolytic anaemia, systemic symptoms with agranulocytosis and gingival bleeding due to immune thrombocytopenia, to alert clinicians to the fact that primary Sjogren's s yndrome may present as clinically significant immune-mediated cytopenia in the absence of sicca symptoms. Sjogren's syndrome, a common autoimmune diso rder, should be considered in the differential diagnosis of apparently 'idi opathic' cytopenias and actively sought by directed history, Schirmer test and autoantibody screening.