Digital Ulceration or Critical Digital Ischaemia in Connective Tissue Disease When Bosentan Has Not Reduced New Ulcers
This protocol applies to secondary Raynaud's phenomenon that has progressed to digital ulceration or critical digital ischaemia in patients with connective tissue disease, where a prior course of Bosentan for prevention of recurrent digital ulcers did not achieve sufficient reduction in new ulcer formation.
Secondary Raynaud's phenomenon progresses to tissue damage — digital ulceration or critical digital ischaemia — when it arises from an underlying connective tissue disease. Because digital ulceration and critical ischaemia share closely aligned management principles, they are addressed together in this protocol.
The previous treatment line — Bosentan, used to prevent recurrent digital ulcers — did not meet the target of reducing the number of new digital ulcers. This protocol defines the structured next step taken after that failure.
When escalation is required, the approach moves toward procedural interventions targeting the affected hand. This includes injection-based approaches and, in severe digital vasculopathy, surgical options — the full structured regimen specifies the applicable pathways and their sequence.
References
DOI: 10.1177/1759720X17740074
RP only progresses to tissue damage when secondary to an underlying cause, which for the rheumatologists usually means a connective tissue disease.
These principles of management are very similar for both digital ulceration and critical ischaemia, and so will be considered together.
These have attracted increasing interest in recent years, recent studies including a prospective study of 20 patients with SSc reporting improvement in hand function after 8 weeks, and a case series of 10 patients with SSc (5 had digital ulcers) reporting benefit in terms of RP, pain, skin temperature recovery after cold water immersion and digital ulcer healing.
Autologous fat grafting or injection of adipose tissue derived stromal or stem cells is now attracting substantial interest in SSc-related digital vasculopathy and at least one controlled trial is underway.
A number of different surgical procedures have been advocated for severe SSc-related digital vasculopathy, including surgical debridement, digital sympathectomy and amputation.
A recent retrospective study of 17 patients with SSc (26 hands operated on) reported symptomatic improvement in pain in 92.3% of hands and ulcer healing in all patients.
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