A case of Sjögren's syndrome (SS) with pulmonary hypertension (PH)

Abstract

A 44-year-old female developed Raynaud's phenomenon and polyarthralgia in 1969. Xerostomia developed in 1974, and the patient was diagnosed as having SS, based on sicca symptoms and positive findings of Schirmer's tear test, Rose-Bengal staining, sialography and labial salivary gland biopsy. One month before admission palpitation on exertion and peripheral edema developed. In May 1981, she was admitted to this hospital because of exertional dyspnea.
The temperature was 37.0°C, and the pulse 68. The blood pressure was 140/98mmHg, and the peripheral venous pressure was 18.0cmH₂O. Physical examination revealed facial edema, distended jugular veins and lymphadenopathy. The second heart sound was increased at the pulmonary area and a Grade 3 holosystolic murmur was audible at the left sternal border. No rales were heard. Hepatosplenomegaly was noted, but no ascites was detected.
Laboratory data revealed a hematocrit of 33% and a white blood cell count of 3, 700 cells/mm³. The erythrocyte sedimentation rate was 16mm/hr. The unine was normal. Serological studies revealed hypergammaglobulinemia, 1+CRP, negative rheumatoid factor and LE cells, positive fluorescent anti-nuclear antibody and anti-DNA antibody (12.8 units/ml, RIA). Anti-RNP, anti-Sm, anti-SS-A and anti-SS-B antibodies were positive in immunodiffusion test. Serum complement level was normal. X-ray films of the chest revealed cardiomegaly with prominent pulmonary arteries and clear lung fields. Pulmonary function studies showed a normal vital capacity and a decreased diffusion capacity. The echocardiography demonstrated a dilated right atrium and ventricle. There was no pericardial effusion. Right cardiac catheterization and angiography showed precapillary pulmonary hypertension, 64/25mmHg (mean 38mmHg), with no evidence of either shunt disease or pulmonary embolism. The right heart failure disappeared by treatment with furosemide by the seventh hospital day.
This patient had a sicca symptom and twelve years' history of Raynaud's phenomenon and polyarthritis. No other symptoms of SLE or PSS developed during this period without corticosteroid therapy. Therefore, although anti-Sm antibody was positive, SLE was unlikely, and the patient was diagnosed as sicca syndrome alone with PH.
Collagen disease with PH and primary pulmonary hypertension with Raynaud's phenomenon have several clinical features in common, such as Raynaud's phenomenon, polyarthritis, leukopenia, hypergammaglobulinemia and positive rheumatoid factor. These similarities are also often found in SS. It is, therefore, suggested that subclinical SS might underlie the former two diseases, or that the three disorders had some common etiopathogenic processes which might contribute to the development of PH.