Severe Pulmonary Hypertension Associated with Primary Sjoegren's Syndrome
Open Access
- 1 January 2003
- journal article
- case report
- Published by Japanese Society of Internal Medicine in Internal Medicine
- Vol. 42 (12) , 1248-1252
- https://doi.org/10.2169/internalmedicine.42.1248
Abstract
Severe pulmonary hypertension is one of the fetal complications in various connective tissue diseases. We report a case of severe pulmonary hypertension associated with primary Sjögren's syndrome. In a lung biopsy specimen, there were findings of intimal and medial hypertrophy with narrowing vessel lumina and plexiform lesions. Moreover, deposits of immunoglobulin M, immunoglobulin A and complement protein Clq were found in the pulmonary arterial walls. Although pulmonary hypertension was refractory to oral prostacyclin, steroid therapy improved the clinical and hemodynamic conditions. In the present case, the immunological etiology may be related to the mechanisms of pulmonary hypertension associated with Sjögren's syndrome.(Internal Medicine 42: 1248-1252, 2003)Keywords
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