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- Title
DISTINCT FINDINGS IN PRIMARY SJÖGREN SYNDROME WITH POSITIVE ANTI-CENTROMERE ANTIBODIES.
- Authors
Cobilinschi, Claudia; Saulescu, Ioana; Constantinescu, Cosmin; busuioc, Constantin-Ioan; Ionescu, Ruxandra
- 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 patient had bilateral massive enlargement of her parotid glands, while the second had a 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.
- Subjects
IMMUNOGLOBULINS; CORNEAL ulcer; HEMATOLOGIC malignancies; PAROTID glands; SYMPTOMS; HEART block; LYMPHOPENIA
- Publication
Romanian Journal of Rheumatology / Revista Romana de Reumatologie, 2020, Vol 29, Issue 3, p132
- ISSN
1843-0791
- Publication type
Article
- DOI
10.37897/RJR.2020.3.5