[Sjögren's syndrome with malignant lymphoma, interstitial pneumonia, and pulmonary hypertension]

A 62-year-old woman was admitted to our hospital for further examination of cough and dyspnea on exertion. She had suffered xerostomia and xerophthalmia for 7 years. Physical examinations showed purpura on the lower extremities, Raynaud's phenomenon, and swelling of the parotid glands. Laboratory data disclosed thrombocytopenia, hypergammaglobulinemia, and high titers of anti-nuclear antibody, anti SS-A, and anti SS-B antibodies. Sicca symptoms, a positive Schirmer's tear test, and laboratory findings together yielded a diagnosis of Sjögren's syndrome. Chest X-P and computed tomographic films demonstrated diffuse reticulonodular shadows and multiple nodules in the left lower lung. Echocardiography and right cardiac catheterization revealed enlargement of the right ventricle, tricuspid regurgitation, and elevated pulmonary arterial pressure. Histologic findings from an open-lung biopsy specimen revealed accumulation of inflammatory cells and fibrosis around broncho-vascular bundles, and diffuse large cell lymphoma. Despite a favorable response to chemotherapy, the patient died of right ventricular failure.