Abstract
PAH and digital ulcers are the refractive pathological conditions of collagen diseases. In this study, we treated four cases as the subjects for studying therapeutic effects of ERA (bosentan).
［Subjects］ Case one was a 65 years old, female MCTD＋RA patient. Case two was 70 years old female(dcSSc), case three was 68 years old female(lcSSc), case four was 63 years old female(dcSSc).
［Results］ Case one patient showed marked improvement of symptoms caused by PAH after day four of ERA administration，thereafter PAP gradually decreased from 70 mmHg to 27 mmHg...Case two showed improvement in six-minute walk test from 115 m to 140 m and, coldness of fingers improved after one week of administration. Her night-time cough markedly decreased and she could get a sound sleep after three years absence.
From day 10 of administration, case three began to show improvement in digital ulcer, which was refractory. But liver damage appeared four weeks later and gradually aggravated, therefore, eighteen weeks after we resigned the treatment.
Case four terminated administrations due to vertigo occurred on day five of administration. But, the digital ulcer deteriorated, therefore, we started the treatment again five months later. Thereafter, the digital ulcer became in healing condition.
［Conclusion］ The effects of ERA were identified at NYHA III stage of PAH therapies, and its effectiveness is promising as new therapeutics for digital ulcers due to peripheral circulation failure.
