title: Mycophenolate mofetil is an effective and safe option for the management of systemic sclerosis-associated interstitial lung disease: results from the Australian Scleroderma Cohort Study.
abstract: OBJECTIVE
  To report the efficacy and tolerability of mycophenolate mofetil (MMF) and azathioprine (AZA) in the management of systemic sclerosis-associated interstitial lung disease (SSc-ILD).
  
  
  METHODS
  Patients in the Australian Scleroderma Cohort Study treated with at least 3 months of MMF or AZA for SSc-ILD confirmed on high resolution computed tomography (HRCT) chest were identified and their pulmonary function tests (PFTs) retrieved. Individuals with available results for T-1 (12 months prior to treatment commencement), T0 (date of treatment commencement) and at least one subsequent time point were included in the drug efficacy analysis. The Wilcoxon signed-rank test was used to compare absolute FVC at T1, T0, 12 months (T1), 24 months (T2) and 36 months (T3). Analysis of drug tolerability included all identified patients treated with MMF or AZA.
  
  
  RESULTS
  18/22 patients treated with MMF and 29/49 treated with AZA had adequate PFTs for inclusion in the drug efficacy analysis. Median absolute FVC at T1 for MMF treatment was 2.50L, declining to 2.12L at T0 (p=0.02). Following MMF therapy, FVC results were stable at T1 (2.13L, p=0.86), T2 (2.17L, p=0.65) and T3 (2.25L, p=0.78). In the AZA group, a statistically significant decline did not occur prior to treatment, however FVC results remained stable at T1, T2 and T3.Adverse events leading to early discontinuation (<12 months treatment) were less common in the MMF group (4/22 vs. 13/49). Gastrointestinal complications were the main cause of discontinuation in both groups.
  
  
  CONCLUSIONS
  In patients with SSc-ILD with declining pulmonary function, MMF therapy was associated with stability for up to 36 months. Early adverse events leading to discontinuation occurred less frequently in patients treated with MMF than in AZA treated patients.
authors.affiliation: Department of Rheumatology, Monash Health, Clayton VIC, Australia.
authors.forename: Claire
authors.initials: C
authors.lastname: Owen
authors.affiliation: Department of Rheumatology, Monash Health, Clayton VIC, Australia.
authors.forename: Gene-Siew
authors.initials: GS
authors.lastname: Ngian
authors.affiliation: Department of Rheumatology, Monash Health, Clayton VIC, Australia.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Elford
authors.affiliation: Derriford Hospital, Plymouth, UK.
authors.forename: Owen
authors.initials: O
authors.lastname: Moore
authors.affiliation: St. Vincent's Hospital, Melbourne VIC, Australia.
authors.forename: Wendy
authors.initials: W
authors.lastname: Stevens
authors.affiliation: St. Vincent's Hospital, Melbourne VIC, Australia; and University of Melbourne, Parkville VIC, Australia.
authors.forename: Mandana
authors.initials: M
authors.lastname: Nikpour
authors.affiliation: St. Vincent's Hospital, Melbourne VIC, Australia.
authors.forename: Candice
authors.initials: C
authors.lastname: Rabusa
authors.affiliation: Royal Adelaide Hospital, North Terrace SA, Australia; and Discipline of Medicine, University of Adelaide, SA, Australia.
authors.forename: Susanna
authors.initials: S
authors.lastname: Proudman
authors.affiliation: Royal Perth Hospital, Perth WA, Australia.
authors.forename: Janet
authors.initials: J
authors.lastname: Roddy
authors.affiliation: Menzies Institute for Medical Research, Hobart TAS, Australia.
authors.forename: Jane
authors.initials: J
authors.lastname: Zochling
authors.affiliation: The Queen Elizabeth Hospital, Woodville South SA, Australia; and Discipline of Medicine, University of Adelaide, SA, Australia.
authors.forename: Catherine
authors.initials: C
authors.lastname: Hill
authors.affiliation: The St. George Hospital, Kogarah NSW, Australia.
authors.forename: Allan
authors.initials: A
authors.lastname: Sturgess
authors.affiliation: Canberra Hospital, Garran ACT, Australia.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Tymms
authors.affiliation: Royal Prince Alfred Hospital, Camperdown NSW, Australia.
authors.forename: Peter
authors.initials: P
authors.lastname: Youssef
authors.affiliation: Department of Rheumatology, Monash Health, Clayton VIC, Australia; and Department of Medicine, Monash University, Clayton, VIC, Australia. josahhar@bigpond.com.
authors.forename: Joanne
authors.initials: J
authors.lastname: Sahhar
mesh_terms: D000328:Adult; D000368:Aged; D001315:Australia; D001379:Azathioprine / Q000627:therapeutic use; D016208:Databases, Factual; D004359:Drug Therapy, Combination; 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 / Q000627:therapeutic use*; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid; D001379:Azathioprine