There is no specific therapy for this other than avoidance of excessive sun exposure. There are generally two forms of lung involvement in scleroderma patients. One is pulmonary interstitial disease which may be mild and not progressive. Occasionally an inflammatory alveolitis can be seen in patients with pulmonary interstitial disease which can be treated more aggressively with steroids and cyclophosphamide. The other kind of involvement is pulmonary arterial hypertension without any significant interstitial fibrosis but which has a worse prognosis.
Mortality is high and the aim of therapy is to dilate the pulmonary arteries.
The use of intravenous prostacyclin has been studied and is currently used under careful observation. A. One common reason for the muscles to hurt is joint and tendon involvement with associated diffuse myalgias or muscle aches. These generally respond well to the use of a nonsteroidal anti-inflammatory medication in short courses. Care must be exercised with use of these medications so as not to aggravate the gastrointestinal tract. Patients with weakness of the muscles without evidence of inflammation may have a fibrotic myopathy which can be treated with physical therapy.