Complications
Necrosis may occur in secondary RP but not in primary RP. This complication results from the progression of digital ulcers and ischaemia and may lead to auto-amputation or require surgical amputation. In digital ulcers with scleroderma, only 60% of ulcers heal by 6 months, with 1% to 2% requiring amputation.[71][90]
May result from severe digital ischaemia and tissue necrosis.
The likelihood of developing digital ulcers increases with disease duration, probably due to worsening obliterative vasculopathy. The presence of digital ulceration is generally thought to exclude a diagnosis of primary RP, so a secondary cause should be sought if ulceration occurs. Ischaemic digital ulcers are found on the digit tips. Treatment of digital ulcers includes prostacyclins and phosphodiesterase-5 (PDE-5) inhibitors.[58][64] Bosentan may be used for prevention of digital ulcers.[68]
Traumatic ulcers (particularly in scleroderma) are often located on the extensor surfaces of the fingers. Creased areas with little underlying subcutaneous tissue (such as the extensor surfaces of the proximal interphalangeal joints) are particularly vulnerable.
Treating traumatic ulcers with vasodilator therapy is not appropriate. Treatment should be targeted at protecting the digits from further trauma and treating superimposed infection as it arises; analgesia is often required.
Moderate risk in secondary RP; does not occur in primary RP. Ischaemia causes loss of tissue pulp and should be treated with prostacyclins and PDE-5 inhibitors.
Patients with severe ischaemia should be treated with intravenous prostacyclins such as iloprost or epoprostenol, and sildenafil or other PDE-5 inhibitors such as tadalafil and vardenafil. If necessary, a sympathetic block should be considered. Techniques available include stellate ganglion blockage, proximal cervical sympathectomies via endoscopic surgery, and local palmar and/or digital sympathectomies. Such techniques may not be available in all centres.
Fibrotic plugs due to ischaemia occur in RP and should be treated with prostacyclins and PDE-5 inhibitors.
Digital ulcers can become infected and may require topical or oral antibiotics and appropriate wound care. If osteomyelitis is suspected, seek consultant advice from a microbiologist.
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