Use of bosentan for digital ulcers related to systemic sclerosis: a real-life retrospective French study of 89 patients treated since specific approval.

OBJECTIVES: Ischaemic digital ulcers (DUs) are a common complication of systemic sclerosis (SSc). This study aimed to characterize patients with SSc and ongoing DUs treated with the endothelin receptor antagonist bosentan in clinical practice in France. METHOD: An observational, retrospective, longitudinal study was conducted in 10 French expert centres. Medical records from randomly selected adult SSc patients who received treatment with bosentan for DU prevention from March 2007 to December 2010 were analysed. The primary objective was to determine the profile of patients at treatment initiation. Secondary objectives were to monitor bosentan dosing, treatment schedule, and reasons for treatment termination. RESULTS: The study included 89 patients (mean age 52 years, 69% female, 44% diffuse cutaneous SSc). At bosentan treatment initiation, the mean duration of Raynaud's phenomenon was 15 ± 12 years, and the mean time since first episode with DU was 6.5 ± 7 years. Most patients had a history of at least two episodes with DUs, separated by < 12 months (61%), and had received intravenous iloprost (63%). Previous DU complications included auto-amputation (8%), surgical amputation (6%), osteitis (6%), and gangrene (4.5%). Active smokers (25%) had a history of significantly more surgical amputation (p = 0.004) and osteitis (p = 0.004) than non-smokers. At least one active DU at bosentan initiation was detected in 82% of patients. Bosentan was used according to prescription guidelines and was well tolerated; six patients (7%) withdrew from treatment because of raised liver enzymes. CONCLUSIONS: Patients treated with bosentan for DU prevention in France have severe, refractory, ongoing ulcerative disease. Active smoking was correlated to a history of DU complications. Tolerance of bosentan was comparable to previous studies.

Mondor's disease: what's new since 1939?

Mondor's disease (MD) is a rare and self-limited benign disease first described in 1939. Originally its clinical presentation was a superficial vein thrombosis (SVT) without contiguous skin inflammation of the chest wall veins. Over time its definition has evolved and now also includes subcutaneous thrombosis of the dorsal vein of the penis but also retractile scarring of the fascia after breast surgery without concomitant SVT. In all cases clinical examination constitutes the first step of diagnostic management. It is followed by an ultrasound exploration (US) to search for a thrombus. In about half of all cases the disease is considered as idiopathic and cancer is rare. Whatever the location considered, the follow-up is usually uneventful with low rates of recurrence and of subsequent cancer. Treatment is debated and ranges from therapeutic abstention to anticoagulants or even surgery. It is likely that the new locations and mechanisms (without thrombosis) of the MD have lead to the constitution of a heterogeneous entity precluding from a consensual mode of care.