An unexpected diagnosis in a dyspnoeic patient with primary Sjogren syndrome

We describe a case of a 50-year-old lady with newly diagnosed primary Sjogren syndrome who presented with severe pulmonary artery hypertension and pericardial effusion. She was managed by immunosuppressive agents and a combination of standard therapy for pulmonary hypertension. Our patient had a clinically significant involvement of cardio-pulmonary system that is atypical of this disease. Prompt recognition and management of this condition are extremely crucial as untreated cases carry a grave prognosis. However, the ideal treatment strategy is yet to be defined for this condition.