Hughes, M; Allanore, Y; El Aoufy, K; Denton, CP; Khanna, D; Krieg, T; Matucci-Cerinic, M; (2021) A Practical Approach to the Management of Digital Ulcers in Patients With Systemic Sclerosis: A Narrative Review. JAMA Dermatology 10.1001/jamadermatol.2021.1463. (In press).

Text Hughes-JAMA Dermatology DU review 2021-for UCL upload.pdf - Accepted Version Access restricted to UCL open access staff Download (935kB)

Abstract

IMPORTANCE: Digital ulcers (DUs) occurring on the fingers in patients with systemic sclerosis (SSc) are associated with substantial pain and disability and are often challenging to treat. However, careful clinical assessment and prompt intervention (wound bed management and systemic pharmacologic treatment) may modify the clinical course. OBJECTIVES: To provide a practical approach to the assessment and management of SSc-DUs and highlight unmet needs and research priorities. EVIDENCE REVIEW: A narrative review of the extant literature was undertaken to provide a broad overview of current knowledge and augmented by expert opinion. FINDINGS: Half of the patients with SSc have a history of DUs, and there is a point of prevalence of approximately 10%. Digital ulcers are often very painful and affect all aspects of physical, social, and family life as well as occupation. Digital ulcers are associated with a severe disease course. Systemic sclerosis DUs, particularly those occurring on the fingertips, represent a vascular ischemic complication, although other etiopathogenic factors play an important role. To guide management, a structured clinical approach is required, including DU definition, classification, and categorization. Digital ulcers require a multidisciplinary approach with close cooperation between physicians and specialist nursing and other allied health professionals to guarantee the appropriate treatment and provide patient education. Local wound bed management is necessary for all DUs and is combined with systemic (pharmacologic) treatments. When treating a DU, the clinician should actively review the therapeutic strategy to prevent further DUs, including the level of systemic disease control, and monitor closely for the development of DU complications, including infection and progression to gangrene. Despite a wide available therapeutic armory, a number of unmet needs and challenges remain that that require resolution to optimize DU management. CONCLUSIONS AND RELEVANCE: A practical approach to DU management, including local wound bed management and systemic treatments, is useful. Digital ulcers are of interest to a broad range of dermatologists, rheumatologists, and other physicians providing care for patients with SSc. Careful clinical assessment and prompt intervention can substantially modify the clinical course of DUs in SSc.

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