Clinical Investigation
Experience of Mycophenolate Mofetil in 10 Patients With Autoimmune-Related Interstitial Lung Disease Demonstrates Promising Effects

https://doi.org/10.1097/MAJ.0b013e31818d094bGet rights and content

Abstract

Background

Interstitial lung disease (ILD) is a frequent manifestation of connective tissue disease (CTD), especially systemic sclerosis (SSc), polymyositis-dermatomyositis, and rheumatoid arthritis. ILD related to CTDs heralds a poor prognosis and is associated with high mortality and 60% of patients have evidence of ILD. Cyclophosphamide (CYC) is extensively used in SSc ILD with moderate initial response but a poor long-term outcome, and is associated with significant toxicity.

Results

Mycophenolate mofetil (MMF) was administered to 10 patients with autoimmune-related ILD: 4 with SSc, 3 with rheumatoid arthritis, 2 with polymyositis, and 1 with systemic lupus erythematosus and Sjögren syndrome. Five patients received remote CYC infusion. Ten patients had improvement in alveolitis, symptoms (cough, dyspnea, and chest discomfort), perceived quality of life and activity levels. Four of 5 patients discontinued oxygen. Two of 8 repeat high-resolution computed tomography improved, 6 stabilized, none worsened. Pulmonary function testing in 1 of 9 patients showed worsening, 3 with improvement and 5 stabilized. Serial echocardiograms revealed no new pulmonary arterial hypertension and no worsening of preexisting pulmonary arterial hypertension. Very importantly, averaged prednisone dose decreased from 58 to 1.4 mg without worsening.

Conclusion

MMF is safe, well tolerated, and allows reduction or discontinuation of prednisone without worsening of symptoms or objective progression of disease. MMF is less toxic and its targeted antifibrotic properties make it a potentially more effective agent than CYC that may supplant it as a first-line agent or provide sensible post-CYC maintenance or synergistic strategy in the treatment of CTD-ILD.

Section snippets

MATERIALS AND METHODS

This is a case series collating and reporting retrospective and longitudinal observations on the effects of MMF on clinical, radiological, and pulmonary findings of CTD-ILD in 10 of our clinic patients. Upon decision, to publish these findings, the Louisiana State University institutional review board deemed these findings as “anecdotal communications” not requiring formal approval.

Patients at the Louisiana State University Rheumatology clinics receive a diagnosis of ILD based on evaluation of

RESULTS

The series was comprised of 10 patients (9 women and 1 man) who elected management with MMF; no patients opted for other management strategies. Disease-specific distribution was 4 patients with SSc of whom 2 had established pulmonary hypertension, 3 patients with RA of whom 2 had ILD on initial presentation and were naïve to methotrexate and biologic treatment, 2 patients with PM, and 1 patient with systemic lupus erythematosus (SLE) and Sjögren syndrome (see Table 1). All 4 patients with SSc

DISCUSSION

The natural history of AIR-ILD is not well established, but there is documented evidence in the literature that supports a role for autoimmune phenomena, particularly, in SSc, SLE, PM-DM, and RA, as an important mechanism responsible for alveolar injury leading to pulmonary fibrosis.13 Dedicated efforts to elucidate the natural history of ILD are necessary to provide clinicians and researchers with a platform for which to establish appropriate screening guidelines, develop validated instruments

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    Presented at the American College of Rheumatology 2007 Scientific Meeting in Boston and at the meeting of the Southern Society for Clinical Investigation February 2008 in New Orleans, where this presentation received the Centocor Scholar in Rheumatology Award.

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