Pulmonary hypertension in MCTD: report of two cases with anticardiolipin antibody.
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.
Miyata
M
M
Second Department of Internal Medicine, Fukushima Medical College, Japan.
Kida
S
S
Kanno
T
T
Suzuki
K
K
Watanabe
H
H
Kaise
S
S
Nishimaki
T
T
Hosoda
Y
Y
Kasukawa
R
R
D000328:Adult; D000368:Aged; D000906:Antibodies / Q000032:analysis*; D002308:Cardiolipins / Q000276:immunology*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000276:immunology; D008947:Mixed Connective Tissue Disease / Q000150:complications* / Q000276:immunology
D000906:Antibodies; D002308:Cardiolipins