Use of leflunomide in patients with chronic hypersensitivity pneumonitis

Abstract Background Prednisone has been shown to reverse lung function declines in hypersensitivity pneumonitis patients without established fibrosis. Second line immunosuppressants like azathioprine and mycophenolate mofetil have a steroid sparing effect and improve DLCO. There is no published lite...

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Main Authors: Sungryong Noh, Ruchi Yadav, Manshi Li, Xiaofeng Wang, Debasis Sahoo, Daniel A. Culver, Aman Pande
Format: Article
Language:English
Published: BMC 2020-07-01
Series:BMC Pulmonary Medicine
Subjects:
Online Access:http://link.springer.com/article/10.1186/s12890-020-01227-2
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author Sungryong Noh
Ruchi Yadav
Manshi Li
Xiaofeng Wang
Debasis Sahoo
Daniel A. Culver
Aman Pande
author_facet Sungryong Noh
Ruchi Yadav
Manshi Li
Xiaofeng Wang
Debasis Sahoo
Daniel A. Culver
Aman Pande
author_sort Sungryong Noh
collection DOAJ
description Abstract Background Prednisone has been shown to reverse lung function declines in hypersensitivity pneumonitis patients without established fibrosis. Second line immunosuppressants like azathioprine and mycophenolate mofetil have a steroid sparing effect and improve DLCO. There is no published literature on the use of leflunomide in such patients. Methods We reviewed our experience with leflunomide for treatment of chronic hypersensitivity pneumonitis in 40 patients. We stratified patients according to the presence or absence of significant (> 20%) fibrosis. We studied the effect of leflunomide on FVC and DLCO trajectory and reported the changes at 12 months. Results Treatment with leflunomide tended to improve the estimated FVC slope from 0.18 ± 1.90% (SEM) of predicted per year to 4.62 ± 1.65% of predicted (NS, p = 0.118). It significantly improved the FVC at 12 months of treatment by 4.4% of predicted (p = 0.02). DLCO continued to increase at 1.45 ± 1.44% (SEM) of predicted per year. Non-fibrotic cHP patients had the largest gain in pulmonary function. Their FVC increased by 8.3% (p = 0.001) and DLCO by 4.8% (p = 0.011). Patients with fibrotic cHP did not improve. Leflunomide treatment was associated with significant gastrointestinal and other adverse effects leading 40% of patients to discontinue therapy. It had a significant steroid sparing effect with half the patients weaned off prednisone entirely. Conclusions Leflunomide appears to be a fairly well tolerated steroid sparing immunosuppressant that improves pulmonary function in cHP patients. It is most effective in patients without significant fibrosis.
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spelling doaj.art-4c38506d6b7840f1917face9f476d57a2022-12-21T17:48:14ZengBMCBMC Pulmonary Medicine1471-24662020-07-0120111010.1186/s12890-020-01227-2Use of leflunomide in patients with chronic hypersensitivity pneumonitisSungryong Noh0Ruchi Yadav1Manshi Li2Xiaofeng Wang3Debasis Sahoo4Daniel A. Culver5Aman Pande6Respiratory Institute, Cleveland ClinicRespiratory Institute, Cleveland ClinicLerner Research Institute, Cleveland ClinicLerner Research Institute, Cleveland ClinicRespiratory Institute, Cleveland ClinicRespiratory Institute, Cleveland ClinicRespiratory Institute, Cleveland ClinicAbstract Background Prednisone has been shown to reverse lung function declines in hypersensitivity pneumonitis patients without established fibrosis. Second line immunosuppressants like azathioprine and mycophenolate mofetil have a steroid sparing effect and improve DLCO. There is no published literature on the use of leflunomide in such patients. Methods We reviewed our experience with leflunomide for treatment of chronic hypersensitivity pneumonitis in 40 patients. We stratified patients according to the presence or absence of significant (> 20%) fibrosis. We studied the effect of leflunomide on FVC and DLCO trajectory and reported the changes at 12 months. Results Treatment with leflunomide tended to improve the estimated FVC slope from 0.18 ± 1.90% (SEM) of predicted per year to 4.62 ± 1.65% of predicted (NS, p = 0.118). It significantly improved the FVC at 12 months of treatment by 4.4% of predicted (p = 0.02). DLCO continued to increase at 1.45 ± 1.44% (SEM) of predicted per year. Non-fibrotic cHP patients had the largest gain in pulmonary function. Their FVC increased by 8.3% (p = 0.001) and DLCO by 4.8% (p = 0.011). Patients with fibrotic cHP did not improve. Leflunomide treatment was associated with significant gastrointestinal and other adverse effects leading 40% of patients to discontinue therapy. It had a significant steroid sparing effect with half the patients weaned off prednisone entirely. Conclusions Leflunomide appears to be a fairly well tolerated steroid sparing immunosuppressant that improves pulmonary function in cHP patients. It is most effective in patients without significant fibrosis.http://link.springer.com/article/10.1186/s12890-020-01227-2Hypersensitivity pneumonitisImmunosuppressantLeflunomideFibrosisPulmonary function
spellingShingle Sungryong Noh
Ruchi Yadav
Manshi Li
Xiaofeng Wang
Debasis Sahoo
Daniel A. Culver
Aman Pande
Use of leflunomide in patients with chronic hypersensitivity pneumonitis
BMC Pulmonary Medicine
Hypersensitivity pneumonitis
Immunosuppressant
Leflunomide
Fibrosis
Pulmonary function
title Use of leflunomide in patients with chronic hypersensitivity pneumonitis
title_full Use of leflunomide in patients with chronic hypersensitivity pneumonitis
title_fullStr Use of leflunomide in patients with chronic hypersensitivity pneumonitis
title_full_unstemmed Use of leflunomide in patients with chronic hypersensitivity pneumonitis
title_short Use of leflunomide in patients with chronic hypersensitivity pneumonitis
title_sort use of leflunomide in patients with chronic hypersensitivity pneumonitis
topic Hypersensitivity pneumonitis
Immunosuppressant
Leflunomide
Fibrosis
Pulmonary function
url http://link.springer.com/article/10.1186/s12890-020-01227-2
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