Acral manifestations of
systemic sclerosis include Raynaud's phenomenon,
calcinosis cutis, and sclerodactyly. In the later stages of the disease,
contractures of the skin and joints as well as obliterative vasculopathy leading to
digital ulcers and necrotic lesions may occur. Patients with acral manifestations of
systemic sclerosis are ideally treated by a team that includes a rheumatologist, dermatologist, hand surgeon, physiotherapist, and, eventually, a psychologist.
Calcium channel antagonists, alpha(1)-adrenergic blockade with
prazosin, and
prostacyclin analogs were proven to be effective in the treatment of scleroderma-related Raynaud's phenomenon.
Losartan, an
angiotensin II receptor inhibitor, and
fluoxetine, a
selective serotonin reuptake inhibitor, have been beneficial for
systemic sclerosis-associated Raynaud's phenomenon in pilot studies. Parenteral
prostacyclin analogs, e. g.,
iloprost, can be recommended as first-line treatment of ischemic
digital ulcers. When
prostacyclin analogs fail, the
phosphodiesterase type 5 inhibitor sildenafil can be tried to improve
ulcer healing.
Bosentan, an
endothelin receptor antagonist, may prevent new
digital ulcers. At present, there are no medical agents agreed to be generally effective in the reduction of calcinotic deposits or cutaneous
fibrosis, although some drugs have been identified as potentially beneficial. Surgical treatment of acral manifestations consists of excision or
curettage of symptomatic calcific deposits, digital
sympathectomy, arterial reconstruction, and
amputation in rare cases. Flexion
contractures of the proximal interphalangeal joints, with secondary hyperextension of the metacarpophalangeal joints, can be treated by
arthrodesis of the proximal interphalangeal joints and resection
arthroplasty or
prostheses at the metacarpophalangeal joints to improve hand function.