TY - JOUR
T1 - Long-Term Treatment With Azathioprine and Mycophenolate Mofetil for Myositis-Related Interstitial Lung Disease
AU - Huapaya, Julio A.
AU - Silhan, Leann
AU - Pinal-Fernandez, Iago
AU - Casal-Dominguez, Maria
AU - Johnson, Cheilonda
AU - Albayda, Jemima
AU - Paik, Julie J.
AU - Sanyal, Abanti
AU - Mammen, Andrew L.
AU - Christopher-Stine, Lisa
AU - Danoff, Sonye K.
N1 - Publisher Copyright:
© 2019 American College of Chest Physicians
PY - 2019/11
Y1 - 2019/11
N2 - Background: The efficacy of azathioprine (AZA) and mycophenolate mofetil (MMF) for interstitial lung disease (ILD) has been described, but mainly in connective tissue disease-associated ILD. The objective of this study was to evaluate the effect of AZA and MMF on lung function and prednisone dose in myositis-related ILD (M-ILD). Methods: In this retrospective study, patients with M-ILD seen at Johns Hopkins and treated with AZA or MMF and no other steroid-sparing agents were included. Linear mixed-effects models adjusted for sex, age, antisynthetase antibody, and smoking status were used to compare the change in FVC % predicted, diffusing capacity of the lungs for carbon monoxide (DLCO) % predicted, and prednisone dose. Results: Sixty-six patients with M-ILD were treated with AZA and 44 with MMF. At treatment initiation, mean FVC % predicted and DLCO % predicted were significantly lower in the AZA group than in the MMF group. In both groups, FVC % predicted improved and the prednisone dose was reduced over 2 to 5 years; however, for DLCO % predicted, only the AZA group improved. The adjusted model showed no significant difference in posttreatment FVC % predicted or DLCO % predicted between groups (mean difference of 1.9 and –8.2, respectively), but a 6.6-mg lower dose of prednisone at 36 months in the AZA group. Adverse events were more frequent with AZA than MMF (33.3% vs 13.6%; P = .04). Conclusions: In M-ILD, AZA treatment was associated with improved FVC % predicted and DLCO % predicted, and lower prednisone dose. Patients treated with MMF had improved FVC % predicted and lower prednisone dose. After 36 months, patients treated with AZA received a lower prednisone dose than those treated with MMF.
AB - Background: The efficacy of azathioprine (AZA) and mycophenolate mofetil (MMF) for interstitial lung disease (ILD) has been described, but mainly in connective tissue disease-associated ILD. The objective of this study was to evaluate the effect of AZA and MMF on lung function and prednisone dose in myositis-related ILD (M-ILD). Methods: In this retrospective study, patients with M-ILD seen at Johns Hopkins and treated with AZA or MMF and no other steroid-sparing agents were included. Linear mixed-effects models adjusted for sex, age, antisynthetase antibody, and smoking status were used to compare the change in FVC % predicted, diffusing capacity of the lungs for carbon monoxide (DLCO) % predicted, and prednisone dose. Results: Sixty-six patients with M-ILD were treated with AZA and 44 with MMF. At treatment initiation, mean FVC % predicted and DLCO % predicted were significantly lower in the AZA group than in the MMF group. In both groups, FVC % predicted improved and the prednisone dose was reduced over 2 to 5 years; however, for DLCO % predicted, only the AZA group improved. The adjusted model showed no significant difference in posttreatment FVC % predicted or DLCO % predicted between groups (mean difference of 1.9 and –8.2, respectively), but a 6.6-mg lower dose of prednisone at 36 months in the AZA group. Adverse events were more frequent with AZA than MMF (33.3% vs 13.6%; P = .04). Conclusions: In M-ILD, AZA treatment was associated with improved FVC % predicted and DLCO % predicted, and lower prednisone dose. Patients treated with MMF had improved FVC % predicted and lower prednisone dose. After 36 months, patients treated with AZA received a lower prednisone dose than those treated with MMF.
KW - antisynthetase syndrome
KW - azathioprine
KW - interstitial lung disease
KW - mycophenolate mofetil
KW - myositis
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U2 - 10.1016/j.chest.2019.05.023
DO - 10.1016/j.chest.2019.05.023
M3 - Article
C2 - 31238042
AN - SCOPUS:85072733937
SN - 0012-3692
VL - 156
SP - 896
EP - 906
JO - CHEST
JF - CHEST
IS - 5
ER -