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. 2013 May;40(5):640-6.
doi: 10.3899/jrheum.121043. Epub 2013 Mar 1.

Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease

Aryeh Fischer  1 , Kevin K Brown, Roland M Du Bois, Stephen K Frankel, Gregory P Cosgrove, Evans R Fernandez-Perez, Tristan J Huie, Mahalakshmi Krishnamoorthy, Richard T Meehan, Amy L Olson, Joshua J Solomon, Jeffrey J Swigris
Affiliations

Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease

Aryeh Fischer et al. J Rheumatol. 2013 May.

Abstract

Objective: Small series suggest mycophenolate mofetil (MMF) is well tolerated and may be an effective therapy for connective tissue disease-associated interstitial lung disease (CTD-ILD). We examined the tolerability and longitudinal changes in pulmonary physiology in a large and diverse cohort of patients with CTD-ILD treated with MMF.

Methods: We identified consecutive patients evaluated at our center between January 2008 and January 2011 and prescribed MMF for CTD-ILD. We assessed safety and tolerability of MMF and used longitudinal data analyses to examine changes in pulmonary physiology over time, before and after initiation of MMF.

Results: We identified 125 subjects treated with MMF for a median 897 days. MMF was discontinued in 13 subjects. MMF was associated with significant improvements in estimated percentage of predicted forced vital capacity (FVC%) from MMF initiation to 52, 104, and 156 weeks (4.9% ± 1.9%, p = 0.01; 6.1% ± 1.8%, p = 0.0008; and 7.3% ± 2.6%, p = 0.004, respectively); and in estimated percentage predicted diffusing capacity (DLCO%) from MMF initiation to 52 and 104 weeks (6.3% ± 2.8%, p = 0.02; 7.1% ± 2.8%, p = 0.01). In the subgroup without usual interstitial pneumonia (UIP)-pattern injury, MMF significantly improved FVC% and DLCO%, and in the subgroup with UIP-pattern injury, MMF was associated with stability in FVC% and DLCO%.

Conclusion: In a large diverse cohort of CTD-ILD, MMF was well tolerated and had a low rate of discontinuation. Treatment with MMF was associated with either stable or improved pulmonary physiology over a median 2.5 years of followup. MMF appears to be a promising therapy for the spectrum of CTD-ILD.

Keywords: CONNECTIVE TISSUE DISEASE; INTERSTITIAL LUNG DISEASE; MYCOPHENOLATE MOFETIL.

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Figures

Figure 1
Figure 1
Cohort formation. MMF: mycophenolate mofetil; ILD: interstitial lung disease; NJH: National Jewish Health, Boulder, CO; CTD: connective tissue disease.
Figure 2
Figure 2
Mean prednisone dose over time in subjects with rheumatoid arthritis (RA), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM), or lung-dominant connective tissue disease (CTD). MMF: myco-phenolate mofetil.
Figure 3
Figure 3
A. Mixed-effects model estimates for percentage of predicted forced vital capacity (FVC%) over time for the entire cohort. B. Mixed-effects model estimates for percentage of predicted diffusing capacity (DLCO%) over time for the entire cohort. Solid line is the mean; broken lines show 95% confidence bands. MMF: mycophenolate mofetil.
Figure 4
Figure 4
Mixed-effects model estimates for percentage of predicted forced vital capacity (FVC%) in subjects with rheumatoid arthritis (RA), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM), or lung-dominant connective tissue disease (CTD). MMF: mycophenolate mofetil.
Figure 5
Figure 5
Mixed-effects model estimates for percentage of predicted forced vital capacity (FVC%) in subjects with connective tissue disease with usual interstitial pneumonia (CTD-UIP) or non-UIP. Solid line: CTD-UIP, n = 32, surgical lung biopsy = 15. Broken line: CTD-non-UIP, n = 93, surgical lung biopsy = 36. MMF: myco- phenolate mofetil.
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