title: Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease.
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%.
  
  
  CONCLUSIONS
  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.
authors: Autoimmune and Interstitial Lung Disease Program, National Jewish Health, Denver, Colorado 80206, USA. fischera@njhealth.org
authors: Aryeh
authors: A
authors: Fischer
authors: Kevin K
authors: KK
authors: Brown
authors: Roland M
authors: RM
authors: Du Bois
authors: Stephen K
authors: SK
authors: Frankel
authors: Gregory P
authors: GP
authors: Cosgrove
authors: Evans R
authors: ER
authors: Fernandez-Perez
authors: Tristan J
authors: TJ
authors: Huie
authors: Mahalakshmi
authors: M
authors: Krishnamoorthy
authors: Richard T
authors: RT
authors: Meehan
authors: Amy L
authors: AL
authors: Olson
authors: Joshua J
authors: JJ
authors: Solomon
authors: Jeffrey J
authors: JJ
authors: Swigris
mesh_terms: D003240:Connective Tissue Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008137:Longitudinal Studies; D008168:Lung / Q000187:drug effects* / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000627:therapeutic use; D010352:Patient Dropouts; D012129:Respiratory Function Tests
chemical_list: D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid
keywords: CONNECTIVE TISSUE DISEASE; INTERSTITIAL LUNG DISEASE; MYCOPHENOLATE MOFETIL