Pulmonary Involvement in Scleroderma: A Clinical Study of the Safety and Efficacy of Mycophenolate Mofetil in Scleroderma Patients With Lung Involvement

Inclusion Criteria: * To participate in this study, patients must first undergo a BAL and HRCT. To be eligible to undergo HRCT and BAL (under the purview of this trial), prospective patients must meet the following criteria: * Aged 21-70. * Negative pregnancy test (with a sensitivity of at least 50 mIU/mL) for females of child-bearing potential * All patients must fulfill the criteria for SSc by American College of Rheumatology (ACR) criteria (Subcommittee for Scleroderma Criteria 1980). * FVC \< 85% of predicted. * SSc for no more than 7 years with onset defined as the date of the first non-Raynaud manifestation. * Patients may have limited (cutaneous thickening distal but not proximal to elbows and knees, with or without facial involvement) or diffuse (cutaneous thickening proximal to elbows and knees, often involving the chest or abdomen) cutaneous SSc (Medsger 1995). * Abnormal DLCO and abnormalities on the plain chest radiograph are not required, although a normal DLCO would be unusual in the face of significant ventilatory restriction due to SSc lung disease. * To be eligible to take study medication, the patient must meet not only the criteria above, but also must have ≥ 3.0% neutrophils or ≥ 2.0% eosinophils in screening BAL fluid and/or ground glass opacification on HRCT. * Women of childbearing potential should have a negative serum or urine pregnancy test with a sensitivity of at least 50 mIU/mL within 1 week before beginning therapy. CellCept therapy will not be initiated until a report of a negative pregnancy test has been obtained. * Effective contraception must be used before beginning CellCept therapy, during therapy, and for 6 weeks following discontinuation of therapy, even where there has been a history of infertility, unless due to hysterectomy. Two reliable forms of contraception must be used simultaneously unless abstinence is the chosen method. If pregnancy does occur during treatment, the physician and patient should discuss the desirability of continuing the pregnancy. Exclusion Criteria: * FVC \< 45% of predicted or DLCO (corrected for hemoglobin \[Hgb\] but not for alveolar volume) \< 35% of predicted (suggestive of severe, probably irreparable, disease). * Leukopenia (white blood cell count \< 4000) or thrombocytopenia (platelet count \< 100,000). * Serum creatinine ≥ 2.0 mg/dl. * Pregnancy, breast feeding, unreliability, drug abuse, or chronic debilitating disease. * Uncontrolled congestive heart failure. * Active infection of the lung, or elsewhere, whose management would be compromised by mycophenolate mofetil. * Prior treatment for alveolitis with mycophenolate mofetil or prior or current treatment for alveolitis with: D-penicillamine, methotrexate, colchicine, Potaba, or azathioprine. * Other serious concomitant medical illness (e.g., cancer). * Forced expiratory volume in 1 second (FEV1)/FVC ratio \< 65%. * If of childbearing potential, failure regularly to be employing two reliable means of contraception (i.e., condom, abstinence, intrauterine device (IUD), tubal ligation, vasectomy) * Pulmonary hypertension (defined as an estimated systolic blood pressure (SBP) ≥ 35 mmHg measured by echocardiogram). * Smoking of cigars, pipes, or cigarettes during the past 6 months. * Clinically significant abnormalities on chest x-ray or HRCT scan other than interstitial lung disease (e.g., lung mass, evidence of active pulmonary infection). * Use of prednisone (or equivalent) in doses \> 10 mg per day. * Does not have ≥ 3.0% neutrophils or ≥ 2.0% eosinophils on screening BAL fluid and does not have ground glass opacification on HRCT. * Unable to take oral medication. * Not able to comply with study procedures in the opinion of the investigator.