The clinical course of a patient with pulmonary hypertension associated with Sjögren's syndrome is reported. The clinical features and outcome of this case are compared with those of two other reported cases in the Spanish and Japanese published work. Thus far, pulmonary hypertension has not been reported with primary Sjögren's syndrome in English publications. These three cases suggest that the development of pulmonary hypertension in primary Sjögren's syndrome is uncommon but should be included as part of the clinical spectrum.
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