digital ulceration in systemic sclerosis

Last edited 05/2021 and last reviewed 07/2021

Digital ulceration (DU) affects in systemic sclerosis (SSc)

• most common vascular manifestation of SSc is Raynaud's phenomenon due to excessive vasoconstriction
• however, more marked vascular involvement,resulting in digital ulceration,occurs at some point in up to 55% of SSc patients (1)
• DUs are painful, impair hand/foot function and result in significant physical and psychological impact observed in both the limited cutaneous and diffuse cutaneous subsets of the disease and cause significant morbidity and impairment of function
• severe ulceration can lead to complications such as infection (including osteomyelitis), gangrene and amputation

SSc patients with severe Raynaud's are managedinitially with standard medical treatment such ascalcium channel blockers, ACE inhibitors, losartan and/or fuoxetine.

When standard medical treatment is ineffective and DUs develop or progress

• IV prostanoids (iloprost or epoprostenol) are used
• despite also being unlicensed for use in this indication, evidence supports the use of IV prostanoids for SSc-DU
• IV prostanoids frequently succeed where initial treatment fails and are also relatively inexpensive but they require administration on a day case basis, usually on 3-5 consecutive days, and this adds considerably tothe overall cost of treatment

An intermediate treatment before IV prostanoids has been suggested (1):

• treating adult patients with SSc who have active DU with oral agents, sildenafil and bosentan

Notes:

• Sildenafil, a phosphodiesterase type 5 inhibitor (PDE5i), is also a potent vasodilator which can be used instead of, or in combination with IV prostanoids
• Bosentan, an endothelin receptor antagonist, is licensed for use in DUs and has been proven to reduce the incidence of new DU formation in patients with active DU

Reference:

• NHS England (May 2021). 210302P - Clinical Commissioning Policy: Sildenafil and Bosentan for the Treatment of Digital Ulceration in Systemic Sclerosis in adults.