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

A diagnostic delay of several years in primary Sjögren'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 on 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 Sjögren's syndrome may present as clinically significant immune-mediated cytopenia in the absence of sicca symptoms. Sjögren's syndrome, a common autoimmune disorder, should be considered in the differential diagnosis of apparently 'idiopathic' cytopenias and actively sought by directed history, Schirmer test and autoantibody screening.