Mycophenolate mofetil as first-line treatment improves clinically evident early scleroderma lung disease.
OBJECTIVE
To find an effective, safe immunosuppressive regimen as an alternative to cyclophosphamide (Cy) for the treatment of clinically evident diffuse scleroderma (dSSc)-associated alveolitis of recent onset.


METHODS
Five consecutive patients with dSSc and recent-onset alveolitis were enrolled and treated with mycophenolate mofetil (MMF) and small (< or =10 mg/day) doses of predinisolone in this open-label trial. One patient with long-standing fibrosing alveolitis was later added to our cohort. Pulmonary function tests [carbon monoxide diffusing capacity (DLCO) and forced vital capacity (FVC)], pulmonary high-resolution computed tomography (HRCT) scans and clinical assessment were performed before and at specified time-points after enrolment. Cases of significant infections, leucopenia and abdominal pain were recorded.


RESULTS
After 4-6 months of MMF therapy, DLCO improved significantly compared with pre-treatment (mean DLCO 75.4% vs 64.2% of predicted value, respectively, P = 0.033). Values of FVC also improved, with the difference almost reaching levels of statistical significance (mean FVC 76.2% vs 65.6% of predicted value, P = 0.057). Ground glass opacities cleared in three of four patients with recent-onset alveolitis and were reduced in one patient after 6-8 months of treatment. Breathlessness and cough improved by 3 months. A possible treatment failure was seen in one patient. However, in five patients functional and clinical improvement was sustained during the study period. No adverse events were recorded in this ongoing clinical trial.


CONCLUSIONS
Our preliminary data suggest that in patients with dSSc and recent, clinically apparent alveolitis, early treatment with MMF and small doses of corticosteroids (CS) may represent an effective, well-tolerated and safe alternative therapy.
Division of Rheumatology, Department of Internal Medicine, Patras University Hospital, 26504 Rion, Patras, Greece. sliossis@med.upatras.gr
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Liossis
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Bounas
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Andonopoulos
D000293:Adolescent; D000328:Adult; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000009:adverse effects / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D011446:Prospective Studies; D011653:Pulmonary Diffusing Capacity / Q000187:drug effects; D011658:Pulmonary Fibrosis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D045743:Scleroderma, Diffuse / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D017211:Treatment Failure; D014797:Vital Capacity / Q000187:drug effects
D000894:Anti-Inflammatory Agents, Non-Steroidal; D005938:Glucocorticoids; D011239:Prednisolone; D009173:Mycophenolic Acid