This study aimed to investigate the efficacy of
bosentan in the treatment of severe
pulmonary hypertension in a young female patient with
mixed connective tissue disease (
MCTD) associated with
antiphospholipid syndrome. A 27-year-old woman presented with sudden onset of
dyspnea. She had not experienced any
dyspnea before this period, and she was known to have
MCTD. Laboratory tests showed positive results for
antinuclear antibodies, anti-RNP
antibodies, anticardiolipin antibodies, beta(2)-glycoprotein I, and
lupus anticoagulant. A complete echocardiographic study was performed demonstrating a pulmonary artery systolic pressure (PAPs) of 85 mmHg. Treatment with
bosentan was initiated. After 12 days, the patient improved clinically. After 6 months of
therapy, the follow-up echocardiography showed a near-normalization of PAPs. Patients who develop PAH secondary to an underlying systemic disease often have a poor survival rate. In this report, we describe a correlation between
anticardiolipin antibodies and rapidly progressive
pulmonary hypertension. Indeed, the patient in this study very likely improved secondary to the effect of
bosentan which produces systemic and pulmonary vasodilatation associated with pulmonary
vascular remodeling as well as possible antifibrotic, anti-inflammatory and antiatherothrombotic effects on cells of lungs damaged by an aPL-antibody mediated mechanism.