iloprost and Hand-Dermatoses

Ischaemic ulcerations of the fingertips are common in SSc and a source of pain and disability. Healing has been demonstrated with intravenous iloprost and two studies with bosentan have demonstrated reduction in the occurrence of new digital ulcers (DUs) over 4-6 months of treatment. Both bosentan studies showed no benefit in healing DU and because of this, net DU burden is no different between drug and placebo and accordingly secondary measures of outcome including pain and hand functionality are inconsistently affected. While it is likely an artefact, it remains unclear that current outcome measures including the Scleroderma Health Assessment Questionnaire (SHAQ), the UK Functional Score and the Michigan Hand Questionnaire are sensitive to change in the domain of digital ischaemia. Major events including amputation and hospitalization occur too infrequently to serve as practical measures of outcome in trials. Future studies of DU therapies will benefit from development of an ulcer-specific measure of outcome.

Topics: Bosentan; Fingers; Hand Dermatoses; Humans; Iloprost; Ischemia; Scleroderma, Systemic; Severity of Illness Index; Skin Ulcer; Sulfonamides; Treatment Outcome; Vasodilator Agents

Digital ulcers (DU's) are one of the main symptoms of systemic scleroderma and occur in approximately 60% of all scleroderma patients. Due to possible complications such as infections, gangrene or amputation, they require regular medical attention and a good wound treatment by doctors and nursing staff. A definition of DU's has not yet been established. In 2009 the European League Against Rheumatism (EULAR) published guidelines for the treatment of DU's. An improvement of the healing of active ulcers has been described with Iloprost. Bosentan significantly reduced the frequency of occurrence of new DU's. In some small studies PDE-5 inhibitors appear helpful. Further studies with other therapeutic approaches will follow in the next few years.

Topics: Bosentan; Fingers; Hand Dermatoses; Humans; Iloprost; Phosphodiesterase 5 Inhibitors; Scleroderma, Systemic; Skin Ulcer; Sulfonamides; Wound Healing

Digital ulcers in systemic sclerosis are painful ischemic necrotic lesions of the acra. Optimal treatment consists of conventional wound management and medication: iloprost infusions promote primary healing of the ulcers, while the dual endothelin receptor antagonist bosentan is used for secondary prophylaxis of new ulcers. The described case illustrates the essential interdisciplinary collaboration for optimal management of these patients.

Topics: Angiography; Antihypertensive Agents; Arm; Bosentan; Cooperative Behavior; CREST Syndrome; Endothelin Receptor Antagonists; Fatal Outcome; Fingers; Follow-Up Studies; Hand Dermatoses; Humans; Iloprost; Interdisciplinary Communication; Leg; Male; Middle Aged; Scleroderma, Limited; Skin Ulcer; Sulfonamides; Vasodilator Agents