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It is estimated that up to 50% of patients with idiopathic inflammatory myopathy will be complicated by interstitial lung disease (ILD), and having ILD may impart a poor prognosis. A recent review of the Johns Hopkins myositis-related ILD (M-ILD) cohort has shown that azathioprine (AZA) and mycophenolate mofetil (MMF) use is associated improved lung function and less prednisone use.
A retrospective study of M-ILD patients seen at Johns Hopkins and treated with AZA or MMF and no other steroid sparing agents were included. They identified a total of 66 AZA treated and 44 MMF treated M-ILD patients.
Treatment with either AZA or MMF lead to improved FVC% and the prednisone lowering over 2-5 years. There was no difference in post-treatment FVC% or DLCO% between groups.
There was a difference in lower prednisone doses at 36 months in the AZA group and in the AZA group only did the DLCO% improve.
Both AZA and MMF were effective steroid sparing agents capable of improving lung function in M-ILD patients.