Michael J. Snyder, MD; Mary R. Jacobs, MD; Rafael G. Grau, MD; David S. Wilkes, MD; Kenneth S. Knox, MD
Potential Financial Conflicts of Interest: None disclosed.
Snyder M., Jacobs M., Grau R., Wilkes D., Knox K.; Resolution of Severe Digital Ulceration during a Course of Bosentan Therapy. Ann Intern Med. 2005;142:802-803. doi: 10.7326/0003-4819-142-9-200505030-00029
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Published: Ann Intern Med. 2005;142(9):802-803.
TO THE EDITOR:
Background: Bosentan, the dual endothelin-receptor antagonist, has proven efficacy for treatment of secondary pulmonary arterial hypertension in patients with systemic sclerosis (1). Preliminary observations suggest a role for endothelin antagonist therapy in patients with systemic sclerosis who experience the Raynaud phenomenon and associated digital ulceration (2).
Objective: To describe a patient with systemic sclerosis and pulmonary arterial hypertension who had complete resolution of previously refractory digital ulcerations 4 months after initiation of bosentan therapy.
Case Report: A 50-year-old man with a 10-year history of progressive systemic sclerosis developed worsening dyspnea and debilitating digital ulcers. The patient had chronic dyspnea due to long-standing pulmonary fibrosis and required oxygen therapy at rest. In addition to scleroderma-associated pulmonary fibrosis, he had severe esophageal dysfunction and skin changes that restricted his thoracic cage. His previous treatment regimens included 20 cycles of pulse cyclophosphamide over approximately 2 years, methotrexate, steroids, and penicillamine. Secondary pulmonary hypertension was diagnosed as the cause of his recent dyspnea and increasing oxygen requirements, and bosentan therapy was initiated. At that time, the patient's digital ulcers were extremely painful and had rapidly progressed over several months. Initially, his fingers were black and necrotic with bone exposed (no photograph available). After 2 weeks of treatment with bosentan, 62.5 mg twice daily, his digital ulcers initially healed and pain was substantially relieved (Figure 2,parts A and B). After 4 months of therapy, the ulcers had nearly resolved (Figure,parts C and D).
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