Systemic sclerosis-associated digital vasculopathy — SCE Rheumatology MCQ

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ModerateConnective Tissue DiseasesSystemic sclerosis-associated digital vasculopathySCE Rheumatology

A 49-year-old woman with diffuse cutaneous systemic sclerosis has recurrent digital vasculopathy. She takes nifedipine MR 40 mg twice daily and sildenafil 25 mg three times daily. Despite this, she has developed three exquisitely painful fingertip ulcers over 3 weeks; two have failed to reduce in size. There is no purulence, fever, exposed bone or clinical evidence of large-vessel occlusion. What is the most appropriate next pharmacological treatment to promote healing of her current digital ulcers?

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Correct answer: D — Give an intravenous iloprost infusion

This is refractory active systemic-sclerosis digital ulceration despite maximised oral vasodilator treatment with a dihydropyridine calcium-channel blocker and a PDE5 inhibitor. Intravenous iloprost is the best next treatment because prostanoid therapy is recommended for active digital ulcers and is used to promote ulcer healing, particularly where oral therapy has been insufficient. There are no features suggesting infection, osteomyelitis or an alternative large-vessel process that would instead require antimicrobial, surgical or vascular intervention. Bosentan is an important near-miss: it reduces the occurrence of new digital ulcers in systemic sclerosis but has not been shown to heal established ulcers. Increasing nifedipine is inappropriate because she is already receiving a high dose. Tadalafil is another PDE5 inhibitor and may be used for Raynaud’s/digital vasculopathy, but changing from sildenafil after inadequate response is not the preferred escalation for persistent active ulcers. Ambrisentan is an endothelin-receptor antagonist used in pulmonary arterial hypertension, but the evidence-based digital-ulcer prevention recommendation is specific to bosentan and does not establish a role for ambrisentan in healing active ulcers.

Reference: Digital ulcers: sildenafil — Full evidence summary (24 March 2015) — https://www.nice.org.uk/advice/esuom42/chapter/full-evidence-summary Updated: Recommendations for systemic sclerosis (2024) — https://www.eular.org/document/download/1021/cb11a479-9d7a-4b7e-8020-b8cc1dc69bb8/932