Skip to main content
Top
Published in: BMC Pulmonary Medicine 1/2021

Open Access 01-12-2021 | Idiopathic Pulmonary Fibrosis | Research

Effectiveness of pirfenidone in idiopathic pulmonary fibrosis according to the autoantibody status: a retrospective cohort study

Authors: Myung Jin Song, Sang Hoon Lee, Ji Ye Jung, Young Ae Kang, Moo Suk Park, Young Sam Kim, Joon Chang, Song Yee Kim

Published in: BMC Pulmonary Medicine | Issue 1/2021

Login to get access

Abstract

Background

Pirfenidone is an anti-fibrotic agent shown to slow the progression of idiopathic pulmonary fibrosis (IPF). However, its effectiveness in association with serological autoimmune features in IPF remains unclear.

Methods

We retrospectively reviewed the medical records of patients with IPF treated at a tertiary care hospital in South Korea. The autoantibody status was defined as positive if we detected autoantibodies meeting the serological domain criteria for interstitial pneumonia with autoimmune features or anti-neutrophil cytoplasmic antibodies.

Results

We included 142 patients with IPF treated with pirfenidone for over six months (93 were autoantibody-positive and 49 were autoantibody-negative). The mean age was 69.5 ± 7.3 years, and 77.5% of the patients were male. The adjusted mean changes over one year were − 34.4 and − 112.2 mL (p = 0.168) in forced vital capacity (FVC), and − 0.53 and − 0.72 mL/mmHg/min (p = 0.356) in the lungs diffusion capacity for carbon monoxide (DLCO) in the autoantibody-negative and autoantibody-positive groups, respectively.

Conclusions

Reductions in FVC and DLCO were similar in autoantibody-positive and autoantibody-negative patients with IPF treated with pirfenidone. Pirfenidone is effective in attenuating the progression of IPF, irrespective of the autoantibody status.
Literature
1.
go back to reference Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198(5):e44–68. Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018;198(5):e44–68.
2.
go back to reference Park JH, Kim DS, Park IN, Jang SJ, Kitaichi M, Nicholson AG, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med. 2007;175(7):705–11. Park JH, Kim DS, Park IN, Jang SJ, Kitaichi M, Nicholson AG, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med. 2007;175(7):705–11.
3.
go back to reference Vij R, Strek ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest. 2013;143(3):814–24. Vij R, Strek ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest. 2013;143(3):814–24.
4.
go back to reference Kono M, Nakamura Y, Yoshimura K, Enomoto Y, Oyama Y, Hozumi H, et al. Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular disease. Respir Med. 2016;117:40–7. Kono M, Nakamura Y, Yoshimura K, Enomoto Y, Oyama Y, Hozumi H, et al. Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular disease. Respir Med. 2016;117:40–7.
5.
go back to reference Hervier B, Benveniste O. Clinical heterogeneity and outcomes of antisynthetase syndrome. Curr Rheumatol Rep. 2013;15(8):349. Hervier B, Benveniste O. Clinical heterogeneity and outcomes of antisynthetase syndrome. Curr Rheumatol Rep. 2013;15(8):349.
6.
go back to reference Shmerling RH, Delbanco TL. The rheumatoid factor: an analysis of clinical utility. Am J Med. 1991;91:528–34. Shmerling RH, Delbanco TL. The rheumatoid factor: an analysis of clinical utility. Am J Med. 1991;91:528–34.
7.
go back to reference Oldham JM, Adegunsoye A, Valenzi E, Lee C, Witt L, Chen L, et al. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 2016;47(6):1767. Oldham JM, Adegunsoye A, Valenzi E, Lee C, Witt L, Chen L, et al. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 2016;47(6):1767.
8.
go back to reference Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46(4):976–87. Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46(4):976–87.
9.
go back to reference Vij R, Noth I, Strek ME. Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 2011;140:1292–9. Vij R, Noth I, Strek ME. Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 2011;140:1292–9.
10.
go back to reference Lee JS, Kim EJ, Lynch KL, Elicker B, Ryerson CJ, Katsumoto TR, et al. Prevalence and clinical significance of circulating autoantibodies in idiopathic pulmonary fibrosis. Respir Med. 2013;107(2):249–55. Lee JS, Kim EJ, Lynch KL, Elicker B, Ryerson CJ, Katsumoto TR, et al. Prevalence and clinical significance of circulating autoantibodies in idiopathic pulmonary fibrosis. Respir Med. 2013;107(2):249–55.
11.
go back to reference Moua T, Maldonado F, Decker PA, Daniels CE, Ryu JH. Frequency and implication of autoimmune serologies in idiopathic pulmonary fibrosis. Mayo Clin Proc. 2014;89(4):319–26. Moua T, Maldonado F, Decker PA, Daniels CE, Ryu JH. Frequency and implication of autoimmune serologies in idiopathic pulmonary fibrosis. Mayo Clin Proc. 2014;89(4):319–26.
12.
go back to reference Ghang B, Lee J, Kwon CO, Ahn SM, Oh JS, Hong S, et al. Clinical significance of autoantibody positivity in idiopathic pulmonary fibrosis. Respir Med. 2019;155:43–8. Ghang B, Lee J, Kwon CO, Ahn SM, Oh JS, Hong S, et al. Clinical significance of autoantibody positivity in idiopathic pulmonary fibrosis. Respir Med. 2019;155:43–8.
13.
go back to reference Goobie GC, Ford-Sahibzada CA, Fritzler MJ, Johannson KA, Fell CD. Autoantibody status is not associated with change in lung function or survival in patients with idiopathic pulmonary fibrosis. Respir Med. 2019;153:85–90. Goobie GC, Ford-Sahibzada CA, Fritzler MJ, Johannson KA, Fell CD. Autoantibody status is not associated with change in lung function or survival in patients with idiopathic pulmonary fibrosis. Respir Med. 2019;153:85–90.
14.
go back to reference Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824.
15.
go back to reference Reveille JD, Solomon DH. Evidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies. Arthritis Rheum. 2003;49(3):399–412. Reveille JD, Solomon DH. Evidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies. Arthritis Rheum. 2003;49(3):399–412.
16.
go back to reference Satoh M, Chan EK, Ho LA, Rose KM, Parks CG, Cohn RD, et al. Prevalence and sociodemographic correlates of antinuclear antibodies in the United States. Arthritis Rheum. 2012;64(7):2319–27. Satoh M, Chan EK, Ho LA, Rose KM, Parks CG, Cohn RD, et al. Prevalence and sociodemographic correlates of antinuclear antibodies in the United States. Arthritis Rheum. 2012;64(7):2319–27.
17.
go back to reference Grygiel-Górniak B, Rogacka N, Puszczewicz M. Antinuclear antibodies in healthy people and non-rheumatic diseases - diagnostic and clinical implications. Reumatologia. 2018;56(4):243–8. Grygiel-Górniak B, Rogacka N, Puszczewicz M. Antinuclear antibodies in healthy people and non-rheumatic diseases - diagnostic and clinical implications. Reumatologia. 2018;56(4):243–8.
18.
go back to reference Parra ER, Kairalla RA, Ribeiro de Carvalho CR, Eher E, Capelozzi VL. Inflammatory cell phenotyping of the pulmonary interstitium in idiopathic interstitial pneumonia. Respiration. 2007;74(2):159–69. Parra ER, Kairalla RA, Ribeiro de Carvalho CR, Eher E, Capelozzi VL. Inflammatory cell phenotyping of the pulmonary interstitium in idiopathic interstitial pneumonia. Respiration. 2007;74(2):159–69.
19.
go back to reference Balestro E, Calabrese F, Turato G, Lunardi F, Bazzan E, Marulli G, et al. Immune Inflammation and Disease Progression in Idiopathic Pulmonary Fibrosis. PloS one. 2016;11(5):e0154516-e. Balestro E, Calabrese F, Turato G, Lunardi F, Bazzan E, Marulli G, et al. Immune Inflammation and Disease Progression in Idiopathic Pulmonary Fibrosis. PloS one. 2016;11(5):e0154516-e.
20.
go back to reference Feghali-Bostwick CA, Tsai CG, Valentine VG, Kantrow S, Stoner MW, Pilewski JM, et al. Cellular and humoral autoreactivity in idiopathic pulmonary fibrosis. J Immunol. 2007;179(4):2592–9. Feghali-Bostwick CA, Tsai CG, Valentine VG, Kantrow S, Stoner MW, Pilewski JM, et al. Cellular and humoral autoreactivity in idiopathic pulmonary fibrosis. J Immunol. 2007;179(4):2592–9.
21.
go back to reference Feghali-Bostwick CA, Wilkes DS. Autoimmunity in idiopathic pulmonary fibrosis: are circulating autoantibodies pathogenic or epiphenomena? Am J Respir Crit Care Med. 2011;183(6):692–3. Feghali-Bostwick CA, Wilkes DS. Autoimmunity in idiopathic pulmonary fibrosis: are circulating autoantibodies pathogenic or epiphenomena? Am J Respir Crit Care Med. 2011;183(6):692–3.
22.
go back to reference Raghu G, Johnson WC, Lockhart D, Mageto Y. Treatment of idiopathic pulmonary fibrosis with a new antifibrotic agent, pirfenidone: results of a prospective, open-label Phase II study. Am J Respir Crit Care Med. 1999;159(4 Pt 1):1061–9. Raghu G, Johnson WC, Lockhart D, Mageto Y. Treatment of idiopathic pulmonary fibrosis with a new antifibrotic agent, pirfenidone: results of a prospective, open-label Phase II study. Am J Respir Crit Care Med. 1999;159(4 Pt 1):1061–9.
23.
go back to reference Solomon DH, Kavanaugh AJ, Schur PH. Evidence-based guidelines for the use of immunologic tests: antinuclear antibody testing. Arthritis Rheum. 2002;47(4):434–44. Solomon DH, Kavanaugh AJ, Schur PH. Evidence-based guidelines for the use of immunologic tests: antinuclear antibody testing. Arthritis Rheum. 2002;47(4):434–44.
24.
go back to reference Won-Il C. Pharmacological treatment of pulmonary fibrosis. J Korean Med Assoc (Korean). 2020;63(1):47–55. Won-Il C. Pharmacological treatment of pulmonary fibrosis. J Korean Med Assoc (Korean). 2020;63(1):47–55.
Metadata
Title
Effectiveness of pirfenidone in idiopathic pulmonary fibrosis according to the autoantibody status: a retrospective cohort study
Authors
Myung Jin Song
Sang Hoon Lee
Ji Ye Jung
Young Ae Kang
Moo Suk Park
Young Sam Kim
Joon Chang
Song Yee Kim
Publication date
01-12-2021
Publisher
BioMed Central
Published in
BMC Pulmonary Medicine / Issue 1/2021
Electronic ISSN: 1471-2466
DOI
https://doi.org/10.1186/s12890-021-01516-4

Other articles of this Issue 1/2021

BMC Pulmonary Medicine 1/2021 Go to the issue
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Watch this official video from ACC.24. Dr. Biykem Bozkurt discusses last year's major advances in heart failure and cardiomyopathies.