If the diagnosis is still unclear, do a punch biopsy. Histology in the early phase will show mild cellular infiltrates around dermal blood vessels and at the dermal subcutaneous inter-phase, while the later phase will show thickening of dermis with broadening of collagen fibers and hyalinization of blood vessel walls.6 If lung, kidney, heart, or gastrointestinal involvement is suspected based on symptoms or physical exam, you’ll need to do an organ function work-up.
Treatment focuses on organ-specific problems
Currently, there are no guidelines for the treatment of scleroderma, given that the exact pathogenesis remains unclear and the disease course varies from patient to patient. Pharmacologic therapy is focused on symptoms and organ-specific problems (TABLE 2). Prazosin 1 to 3 mg TID is moderately effective in treating Raynaud’s phenomenon secondary to scleroderma7 (SOR: A). Losartan has been reported to reduce the frequency and severity of Raynaud’s phenomenon compared with a low dose of nifedepine,8 in a nonblinded randomized controlled trial (SOR: B). For interstitial lung disease, cyclophosphamide has been found to modestly reduce dyspnea while improving lung function, but it requires close monitoring9 (SOR: A). Bosentan is approved for symptomatic pulmonary hypertension and has been shown to decrease the occurrence of digital ulcers secondary to Raynaud’s phenomenon10 (SOR: A). (Bosentan is available in the United States under a special restricted distribution program called the Tracleer Access Program.)
Nonpharmacologic treatments should also be considered in the management of scleroderma. Advise patients that Raynaud’s phenomenon may be improved by avoiding exposure to the cold and by not smoking (SOR: C). Cutaneous ulcers can be protected with an occlusive dressing and treated with antibiotics if infected (SOR: C). To remove painful calcinotic nodules or release contractures secondary to sclerosis that may limit movement, you may want to consider surgery for your patient11 (SOR: C). If aesthetically unappealing, telangiectasias may be removed with electrosurgery or laser therapy (SOR: C).
Prognosis for scleroderma varies, depending on whether it is diffuse or limited. One large study found that patients with lSSc had a 10-year survival rate of 71%, compared with 21% for patients with dSSc (SOR: B).4 Patients with systemic sclerosis should be monitored for interstitial lung disease, pulmonary hypertension, renal failure, and cardiomyopathy (SOR: C). Prognosis worsens with renal, pulmonary, or cardiac involvement; pulmonary disease is the leading cause of death in dSSc.1
TABLE 2
Medical treatment of systemic scleroderma5-9
| Organ-specific problem/symptom | Treatment |
|---|---|
| Raynaud’s phenomenon | Nifedipine, verapamil, losartan, iloprost, prazosin, bosentan* |
| Pulmonary hypertension | Bosentan,* iloprost, captopril, enalapril, sildenafil |
| Interstitial lung disease | Cyclophosphamide, prednisone |
| Cardiomyopathy or arrhythmias | Antiarrhythmics, diuretics, digoxin, pacemaker, transplant |
| Renal failure or crisis | Captopril, kidney dialysis, transplant |
| Skin fibrosis | Methotrexate, cyclosporine, D-penicillamine |
| Arthralgias | Ibuprofen, acetaminophen |
| GERD, gastroparesis, diarrhea | H2 blockers, proton pump inhibitors, prokinetics, antibiotics |
| Pruritus | Antihistamines, low-dose topical steroids |
| GERD, gastroesophageal reflux disease. | |
| *Restricted access in the United States. | |