Raynaud Phenomenon. The most effective method of preventing Raynaud phenomenon is avoidance of cold exposure. Patients should wear warm protective clothing and avoid tobacco use. Conventional vasodilators, such as long-acting dihydropyridine calcium channel blockers (nifedipine, amlodipine, felodipine), are effective in some patients and are relatively safe. Other vasodilators such as nitrates and prazosin are used alone or in combination with calcium channel blockers in patients who fail to respond adequately to calcium channel blockers alone. One baby aspirin (81 mg) is recommended to inhibit platelet activation and microvascular occlusion. More expensive second-line agents, such as phosphodiesterase-5 inhibitors (sildenafil or tadalafil), endothelin receptor antagonists (bosentan), and intravenous prostanoids (epoprostenol or alprostadil), are reserved for refractory cases with critical digital ischemia leading to ulceration or gangrene. Selective digital sympathectomy has been successful in cases that are not responsive to medical management. Oral antibiotics with good staphylococcal coverage are indicated if lesions become infected. Deeper soft tissue infections or osteomyelitis require treatment with intravenous antibiotics, debridement of devitalized tissue, and, if necessary, amputation.

Gastrointestinal Disease. Esophageal symptoms can be minimized with small, frequent meals, elevation of the head end of the bed, and use of proton pump inhibitors. Patients with persistent symptoms require upper gastrointestinal endoscopy to exclude esophageal stricture and Barrett metaplasia. Small bowel dysmotility symptoms can be managed by increasing dietary fiber, avoiding drugs that affect motility (narcotics), and administering empirical antibiotic therapy cyclically for small intestinal bacterial overgrowth. Octreotide has been used as a small bowel prokinetic agent with variable results. In refractory disease with severe malnutrition and weight loss, parenteral hyperalimentation may be necessary.

Pulmonary Hypertension. Endothelin-1 receptor antagonists (bosentan and ambrisentan) and phosphodiesterase-5 inhibitors (sildenafil and tadalafil) have been approved for treatment of pulmonary hypertension in scleroderma. Inhaled, intravenous or subcutaneous administration of prostanoids is indicated in more advanced cases. Combination therapy is sometimes necessary.

Interstitial Lung Disease. There have been few randomized controlled trials in pulmonary fibrosis in SSc. In double-blind placebo-controlled studies, patients with active alveolitis had stabilization of lung function when treated with oral or monthly intravenous cyclophosphamide for 6 to 12 months. However, the clinical relevance of the rather small changes in the forced vital capacity is still in question, and it is debatable whether such a small measurable benefit after 1 year of oral cyclophosphamide is worth the long-term cumulative risk of exposure to this alkylating agent. Mycophenolate mofetil may be effective for interstitial lung disease in scleroderma, and federally funded studies are currently ongoing.

Resting or exertional hypoxia is an indication for supplemental oxygen. Lung transplantation may represent a viable therapeutic option for selected patients.

Renal Disease. Scleroderma renal crisis is a medical emergency. Aggressive antihypertensive therapy with angiotensin-converting enzyme (ACE) inhibitors has considerably improved survival. A rapid-acting ACE inhibitor, such as captopril, should be titrated to normalize blood pressure promptly. Some patients may not respond and progress to renal failure requiring dialysis. However, a subset of patients requiring dialysis may eventually recover renal function after 12 to 18 months if ACE inhibitors are continued.

Cardiac Disease. At present, treatment of symptomatic scleroderma cardiomyopathy is essentially empirical and is similar to the medical treatment of idiopathic dilated cardiomyopathy. Diuretics, ACE inhibitors, β-adrenergic blockers, and vasodilators are routinely used as indicated for cardiac failure. For symptomatic cardiac conduction defects or ventricular arrhythmias, cardiac pacemakers or implantable defibrillators may be required. Cardiac transplant may be a viable option in suitable candidates.

Musculoskeletal Disease. NSAIDs may be used for arthralgias. A regular exercise program can improve joint range of motion and prevent muscle wasting and contractures. Active myositis is treated with methotrexate, azathioprine, or other immunosuppressive agents. Corticosteroid therapy should be avoided or used in precipitating low doses if required, because of the increased risk for scleroderma renal crisis.

Although no cure has been found for scleroderma, the disease is often slowly progressive and manageable, and people who have it can sometimes lead healthy and productive lives. Like many other conditions, education about scleroderma and local support groups can be the greatest tools for managing the disease and reducing the risk of further complications.