Pulmonary hypertension in MCTD: Report of two cases with anticardiolipin antibody |
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Authors: | M Miyata MD S Kida T Kanno K Suzuki H Watanabe S Kaise T Nishimaki Y Hosoda R Kasukawa |
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Institution: | (1) Department of Pathology and Clinical Laboratories, Keio University School of Medicine, Tokyo, Japan;(2) Second Department of Internal Medicine, Fukushima Medical College, 1 Hikarigaoka, 960-12 Fukushima, Japan |
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Abstract: | Summary We report on 2 patients with well-documented mixed connective tissue disease (MCTD) accompanied by severe pulmonary hypertension (PH) due to thrombosis or thromboembolism. In a previous report we indicated (1) that patients with MCTD complicated by PH have a significantly worse prognosis than patients with other connective tissue disease (CTD) complicated by PH. Both our patients had anticardiolipin antibody (a-CL) in the initial stages of the disease. We also studied the relationship of a-CL to PH in patients with other CTD. Patients of either MCTD or SLE with high levels of a-CL had significantly higher values of mean pulmonary arterial pressure than patients without a-CL. Several factors were suggested for the pathogenesis of PH such as vasospasm, arteritis, platelet dysfunction, and thrombosis or thromboembolism. The presence of a-CL may be one of important factors in development of PH among patients with MCTD with recurrent pulmonary thrombosis or thromboembolism. |
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Keywords: | Pulmonary Hypertension Anticardiolipin Antibody Mixed Connective Tissue Disease |
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