Distinct findings in primary Sjögren syndrome with positive anti-centromere antibodies

Abstract

Primary Sjögren’s syndrome (pSS) includes a rarer subtype with positive anti-centromere antibodies, which appears to be linked with scleroderma features and at higher risk of developing lymphoma. Two female patients were evaluated in our department for severe dryness of the mouth and eyes. The younger history of recurrent corneal ulcers. They both described Raynaud’s phenomenon and either had sclerodactily or facial teleangiectasias. The patients shared lymphopenia, negative anti-Ro and anti-La antibodies, positive high titer anti-centromere antibodies (ACA). The capillaroscopy exam revealed a scleroderma-like pattern. Minor gland biopsy reported a massive inflammatory lymphoplasmacytic infiltrate. One of the patients developed pulmonary lymphoma. The ACA positive pSS is associated with more severe sicca symptoms but can also fulfill criteria for systemic sclerosis. More research is necessary to link this subtype to the risk of developing hematological malignancies.

Keywords

• primary sjögren syndrome
• anticentromere antibodies
• lymphoma
• scleroderma features