Advances with pharmacotherapy for the treatment of interstitial lung disease

References

• Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med. 2020;383(10):958–968.
   PubMed Web of Science ®Google Scholar
• Richeldi L, Collard HR, Jones MG., et al. Idiopathic pulmonary fibrosis. Lancet. 2017;389(10082):1941–1952.
   PubMed Web of Science ®Google Scholar
• Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192(2):e3–19.
   PubMed Web of Science ®Google Scholar
• Nathan SD, Costabel U, Albera C, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis and more advanced lung function impairment. Respir Med. 2019;153:44–51.
   PubMed Web of Science ®Google Scholar
• Wuyts WA, Kolb M, Stowasser S, et al. First data on efficacy and safety of nintedanib in patients with idiopathic pulmonary fibrosis and forced vital capacity of ≤50 % of predicted value. Lung. 2016;194(5):739–743.
   PubMed Web of Science ®Google Scholar
• Cerri S, Monari M, Guerrieri A, et al. Real-life comparison of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis: a 24-month assessment. Respir Med. 2019;159:2–7.
   Web of Science ®Google Scholar
• Conte E, Gili E, Fagone E, et al. Effect of pirfenidone on proliferation, TGF-β-induced myofibroblast differentiation and fibrogenic activity of primary human lung fibroblasts. Eur J Pharm Sci. 2014;58:13–19.
   PubMed Web of Science ®Google Scholar
• Azuma A, Nukiwa T, Tsuboi E, et al. Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2005;171(9):1040–1047.
   PubMed Web of Science ®Google Scholar
• Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2010;35(4):821–829.
   PubMed Web of Science ®Google Scholar
• Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011. Published Online First: 2011. DOI:https://doi.org/10.1016/S0140-6736(11)60405-4.
   PubMed Web of Science ®Google Scholar
• King TE, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–2092.
   PubMed Web of Science ®Google Scholar
• Wollin L, Wex E, Pautsch A, et al. Mode of action of nintedanib in the treatment of idiopathic pulmonary fibrosis. Eur Respir J. 2015;45(5):1434–1445.
   PubMed Web of Science ®Google Scholar
• Richeldi L, Costabel U. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis luca. N Engl J Med. 2011;12:1079–1087. DOI:https://doi.org/10.1056/NEJMoa1103690.
   Google Scholar
• Richeldi L, Du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–2082.
   PubMed Web of Science ®Google Scholar
• Richeldi L, Cottin V, Du Bois RM, et al. Nintedanib in patients with idiopathic pulmonary fibrosis: combined evidence from the TOMORROW and INPULSIS® trials. Respir Med. 2016;113:74–79.
   PubMed Web of Science ®Google Scholar
• Crestani B, Huggins JT, Kaye M, et al. Long-term safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis: results from the open-label extension study, INPULSIS-ON. Lancet Respir Med. 2019. Published Online First: 2019. DOI:https://doi.org/10.1016/S2213-2600(18)30339-4.
   PubMedGoogle Scholar
• Vancheri C, Kreuter M, Richeldi L, et al. Nintedanib with add-on pirfenidone in idiopathic pulmonary fibrosis: results of the INJOURNEY trial. Am J Respir Crit Care Med. 2018;197(3):356–363.
   PubMed Web of Science ®Google Scholar
• Behr J, Kolb M, Song JW, et al. Nintedanib and sildenafil in patients with idiopathic pulmonary fibrosis and right heart dysfunction: a prespecified subgroup analysis of a double-blind randomized clinical trial (instage). Am J Respir Crit Care Med. 2019;200(12):1505–1512.
   PubMed Web of Science ®Google Scholar
• Kolb M, Raghu G, Wells AU, et al. Nintedanib plus sildenafil in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2018;379(18):1722–1731.
   PubMed Web of Science ®Google Scholar
• Richeldi L, Kolb M, Jouneau S, et al. Efficacy and safety of nintedanib in patients with advanced idiopathic pulmonary fibrosis. BMC Pulm Med. 2020;4:1–8. doi:https://doi.org/10.1186/s12890-019-1030-4.
   Google Scholar
• Cameli P, Refini RM, Bergantini L, et al. Long-term follow-up of patients with idiopathic pulmonary fibrosis treated with pirfenidone or nintedanib: a real-life comparison study. Front Mol Biosci. 2020;7:1–8.
   PubMed Web of Science ®Google Scholar
• Petnak T, Lertjitbanjong P, Charat Thongprayoon TM., et al.Impact of antifibrotic therapy on mortality and acute exacerbation in idiopathic pulmonary fibrosis: a systematic review and meta-analysis. Chest. 2021;160(5):1751–1763. doi:https://doi.org/10.1016/j.chest.2021.06.049.
   Web of Science ®Google Scholar
• Atkins CP, Loke YK, Wilson AM., et al.Outcomes in idiopathic pulmonary fibrosis: a meta-analysis from placebo controlled trials. Respir Med. 2014;108(2):376–387.
   PubMed Web of Science ®Google Scholar
• Shulgina L, Cahn AP, Chilvers ER, et al. Treating idiopathic pulmonary fibrosis with the addition of co-trimoxazole: a randomised controlled trial. Thorax. 2013;68(2):155–162.
   PubMed Web of Science ®Google Scholar
• Wilson AM, Clark AB, Cahn T, et al. Effect of co-trimoxazole (Trimethoprim-Sulfamethoxazole) vs placebo on death, lung transplant, or hospital admission in patients with moderate and severe idiopathic pulmonary fibrosis: the EME-TIPAC randomized clinical trial. J Am Med Assoc. 2020;324(22):2282–2291.
   Web of Science ®Google Scholar
• Flaherty FJMEYKR. Effect of antimicrobial therapy on respiratory hospitalization or death in adults with idiopathic pulmonary fibrosis the cleanUP-IPF randomized clinical trial. J Am Med Assoc. 2021;325(18):1841-1851. doi:https://doi.org/10.1001/jama.2021.4956.
   Google Scholar
• Chen CY, Chen CH, Wang CY, et al. The effect of additional antimicrobial therapy on the outcomes of patients with idiopathic pulmonary fibrosis: a systematic review and meta-analysis. Respir Res. 2021;22(1):1–9.
   PubMed Web of Science ®Google Scholar
• Krempaska K, Barnowski S, Gavini J, et al. Azithromycin has enhanced effects on lung fibroblasts from idiopathic pulmonary fibrosis (IPF) patients compared to controls. Respir Res. 2020;21(1):1–16.
   PubMed Web of Science ®Google Scholar
• Macaluso C, Furcada JM, Alzaher O, et al. The potential impact of azithromycin in idiopathic pulmonary fibrosis. Eur Respir J. 2019;53(2). DOI:https://doi.org/10.1183/13993003.00628-2018.
   Google Scholar
• Faverio P, Bini F, Vaghi A, et al. Long-term macrolides in diffuse interstitial lung diseases. Eur Respir Rev. 2017;26(146):1–7.
   Web of Science ®Google Scholar
• Kawamura K, Ichikado K, Yasuda Y, et al. Azithromycin for idiopathic acute exacerbation of idiopathic pulmonary fibrosis: a retrospective single-center study. BMC Pulm Med. 2017;17(1):94.
   PubMedGoogle Scholar
• Guler SA, Clarenbach C, Brutsche M, et al. Azithromycin for the treatment of chronic cough in idiopathic pulmonary fibrosis: a randomized controlled cross-over trial. Ann Am Thorac Soc. 2021;(12). DOI: https://doi.org/10.1513/AnnalsATS.202103-266OC.
   Google Scholar
• Nishi Y, Sano H, Kawashima T, et al. Role of galectin-3 in human pulmonary fibrosis. Allergol Int. 2007;56(1):57–65.
   PubMedGoogle Scholar
• d’Alessandro M, De Vita E, Bergantini L, et al. Galactin-1, 3 and 9: potential biomarkers in idiopathic pulmonary fibrosis and other interstitial lung diseases. Respir Physiol Neurobiol. 2020;282:7–10.
   Web of Science ®Google Scholar
• Hirani N, MacKinnon AC, Nicol L, et al. Target inhibition of galectin-3 by inhaled TD139 in patients with idiopathic pulmonary fibrosis. Eur Respir J. 2021;57(5). DOI:https://doi.org/10.1183/13993003.02559-2020.
   Google Scholar
• Ahluwalia N, Shea BS, Tager AM., et al.New therapeutic targets in idiopathic pulmonary fibrosis. Aiming to rein in runaway wound-healing responses. Am J Respir Crit Care Med. 2014;190(8):867–878.
   PubMed Web of Science ®Google Scholar
• Chandriani S, DePianto DJ, N’Diaye EN, et al. Endogenously expressed IL-13Rα2 attenuates IL-13–mediated responses but does not activate signaling in human lung fibroblasts. J Immunol. 2014;193(1):111–119.
   PubMed Web of Science ®Google Scholar
• Park SW, Ahn MH, Jang HK, et al. Interleukin-13 and its receptors in idiopathic interstitial pneumonia: clinical implications for lung function. J Korean Med Sci. 2009;24(4):614–620.
   PubMed Web of Science ®Google Scholar
• Maher TM, Costabel U, Glassberg MK, et al. Phase 2 trial to assess lebrikizumab in patients with idiopathic pulmonary fibrosis. Eur Respir J. 2021;57(2). DOI:https://doi.org/10.1183/13993003.02442-2019.
   Google Scholar
• Oikonomou N, Mouratis MA, Tzouvelekis A, et al. Pulmonary autotaxin expression contributes to the pathogenesis of pulmonary fibrosis. Am J Respir Cell Mol Biol. 2012;47(5):566–574.
   PubMed Web of Science ®Google Scholar
• Montesi SB, Mathai SK, Brenner LN, et al. Docosatetraenoyl LPA is elevated in exhaled breath condensate in idiopathic pulmonary fibrosis. BMC Pulm Med. 2014;14(1):1–7.
   PubMedGoogle Scholar
• Maher TM, van der Aar EM, Van de Steen O, et al. Safety, tolerability, pharmacokinetics, and pharmacodynamics of GLPG1690, a novel autotaxin inhibitor, to treat idiopathic pulmonary fibrosis (FLORA): a phase 2a randomised placebo-controlled trial. Lancet Respir Med. 2018;6(8):627–635.
   PubMed Web of Science ®Google Scholar
• Maher TM, Kreuter M, Lederer DJ, et al. Rationale, design and objectives of two phase III, randomised, placebo-controlled studies of GLPG1690, a novel autotaxin inhibitor, in idiopathic pulmonary fibrosis (ISABELA 1 and 2). BMJ Open Respir Res. 2019;6:1–8.
   Web of Science ®Google Scholar
• Lipson KE, Wong C, Teng Y, et al. CTGF is a central mediator of tissue remodeling and fibrosis and its inhibition can reverse the process of fibrosis. Fibrogenesis Tissue Repair. 2012;5(S1):S24.
   PubMedGoogle Scholar
• Raghu G, Scholand MB, De Andrade J, et al. FG-3019 anti-connective tissue growth factor monoclonal antibody: results of an open-label clinical trial in idiopathic pulmonary fibrosis. Eur Respir J. 2016;47(5):1481–1491.
   PubMed Web of Science ®Google Scholar
• Richeldi L, Fernández Pérez ER, Costabel U, et al. Pamrevlumab, an anti-connective tissue growth factor therapy, for idiopathic pulmonary fibrosis (PRAISE): a phase 2, randomised, double-blind, placebo-controlled trial. Lancet Respir Med. 2020. Published Online First: 2020. DOI:https://doi.org/10.1016/S2213-2600(19)30262-0.
   Web of Science ®Google Scholar
• Pilling D, Buckley CD, Salmon M, et al. Inhibition of fibrocyte differentiation by serum amyloid P. J Immunol. 2003;171(10):5537–5546.
   PubMed Web of Science ®Google Scholar
• Dillingh MR, van Den Blink B, Moerland M, et al. Recombinant human serum amyloid P in healthy volunteers and patients with pulmonary fibrosis. Pulm Pharmacol Ther. 2013;26(6):672–676.
   PubMed Web of Science ®Google Scholar
• Raghu G, van Den Blink B, Hamblin MJ, et al. Long-term treatment with recombinant human pentraxin 2 protein in patients with idiopathic pulmonary fibrosis: an open-label extension study. Lancet Respir Med. 2019;7(8):657–664.
   PubMed Web of Science ®Google Scholar
• Raghu G, Van Den Blink B, Hamblin MJ, et al. Effect of recombinant human pentraxin 2 vs placebo on change in forced vital capacity in patients with idiopathic pulmonary fibrosis a randomized clinical trial. J Am Med Assoc. 2018;319(22):2299–2307.
   PubMed Web of Science ®Google Scholar
• Song JW, Lee H-K, Lee CK, et al. Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia. Sarcoidosis, Vasc Diffus Lung Dis off J WASOG. 2013;30:103–112.
   PubMed Web of Science ®Google Scholar
• Kadura S, Raghu G. Rheumatoid arthritis-interstitial lung disease: manifestations and current concepts in pathogenesis and management. Eur Respir Rev. 2021;30(160). DOI:https://doi.org/10.1183/16000617.0011-2021.
   Google Scholar
• Yamano Y, Taniguchi H, Kondoh Y, et al. Multidimensional improvement in connective tissue disease-associated interstitial lung disease: two courses of pulse dose methylprednisolone followed by low-dose prednisone and tacrolimus. Respirology. 2018;23:1041–1048.
   PubMed Web of Science ®Google Scholar
• Morisset J, Johnson C, Rich E, et al. Management of myositis-related interstitial lung disease. Chest. 2016;150(5):1118–1128.
   PubMed Web of Science ®Google Scholar
• Lega J-C, Fabien N, Reynaud Q, et al. The clinical phenotype associated with myositis-specific and associated autoantibodies: a meta-analysis revisiting the so-called antisynthetase syndrome. Autoimmun Rev. 2014;13:883–891.
   PubMed Web of Science ®Google Scholar
• Long K, Danoff SK. Interstitial lung disease in polymyositis and dermatomyositis. Clin Chest Med. 2019;40(3):561–572.
   PubMed Web of Science ®Google Scholar
• Bruni C, Tashkin DP, Steen V, et al. Intravenous versus oral cyclophosphamide for lung and/or skin fibrosis in systemic sclerosis: an indirect comparison from EUSTAR and randomised controlled trials. Clin Exp Rheumatol. 2020;38:S161–8.
   PubMed Web of Science ®Google Scholar
• Harper L, Morgan MD, Walsh M, et al. Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up. Ann Rheum Dis. 2012;71(6):955–960.
   PubMed Web of Science ®Google Scholar
• Barnes H, Holland AE, Westall GP, et al. Cyclophosphamide for connective tissue disease-associated interstitial lung disease. Cochrane Database Syst Rev. 2018;2018. DOI:https://doi.org/10.1002/14651858.CD010908.pub2.
   Google Scholar
• Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354(25):2655–2666.
   PubMed Web of Science ®Google Scholar
• Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708–719.
   PubMed Web of Science ®Google Scholar
• Smolen JS, Landewé RBM, Bijlsma JWJ, et al. EULAR recommendations for the management of rheumatoid arthritis with synthetic and biological disease-modifying antirheumatic drugs: 2019 update. Ann Rheum Dis. 2020;79(6):S685–99.
   PubMed Web of Science ®Google Scholar
• Li L, Liu R, Zhang Y, et al. A retrospective study on the predictive implications of clinical characteristics and therapeutic management in patients with rheumatoid arthritis-associated interstitial lung disease. Clin Rheumatol. 2020;39(5);1457–1470. DOI: https://doi.org/10.1007/s10067-019-04846-1.
   Web of Science ®Google Scholar
• Allison AC. Mechanisms of action of mycophenolate mofetil. Lupus. 2005;14(3_suppl):2–8.
   PubMed Web of Science ®Google Scholar
• Fischer A, Brown KK, Du Bois RM, et al. Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease. J Rheumatol. 2013;40(5):640–646.
   PubMed Web of Science ®Google Scholar
• Vaiarello V, Schiavetto S, Foti F, et al. Mycophenolate mofetil improves exercise tolerance in systemic sclerosis patients with interstitial lung disease: a pilot study. Rheumatol Ther. 2020;7(4):1037–1044.
   PubMed Web of Science ®Google Scholar
• Xinyu M, Tang R, Luo M, et al. Efficacy of mycophenolate mofetil versus cyclophosphamide in systemic sclerosis-related interstitial lung disease: a systematic review and meta-analysis. Clin Rheumatol. 2021;40(8):3185-3193. doi:https://doi.org/10.1007/s10067-021-05794-5.
   Google Scholar
• Omair MA, Alahmadi A, Johnson SR. Safety and effectiveness of mycophenolate in systemic sclerosis. A systematic review. PLoS One. 2015;10(5):e0124205.
   PubMed Web of Science ®Google Scholar
• Owen C, Ngian G-S, Elford K, et al. 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. Clin Exp Rheumatol. 2016;34 Suppl 1:170–176.
   Web of Science ®Google Scholar
• Panopoulos ST, Bournia V-K, Trakada G, et al. Mycophenolate versus cyclophosphamide for progressive interstitial lung disease associated with systemic sclerosis: a 2-year case control study. Lung. 2013;191(5):483–489.
   PubMed Web of Science ®Google Scholar
• Adler S, Huscher D, Siegert E, et al. Systemic sclerosis associated interstitial lung disease - individualized immunosuppressive therapy and course of lung function: results of the EUSTAR group. Arthritis Res Ther. 2018;20(1):1–12.
   PubMedGoogle Scholar
• Bérezné A, Ranque B, Valeyre D, et al. Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open-label study. J Rheumatol. 2008;35(6):1064–1072.
   PubMed Web of Science ®Google Scholar
• Oldham JM, Lee C, Valenzi E, et al. Azathioprine response in patients with fibrotic connective tissue disease-associated interstitial lung disease. Respir Med. 2016;121:117–122.
   PubMed Web of Science ®Google Scholar
• Kremer JM. Methotrexate pulmonary toxicity: deep inspiration. Arthritis Rheumatol. 2020;72(12):1959–1962.
   PubMed Web of Science ®Google Scholar
• Conway R, Low C, Coughlan RJ, et al. Methotrexate and lung disease in rheumatoid arthritis: a meta-analysis of randomized controlled trials. Arthritis Rheumatol (Hoboken, NJ). 2014;66(4):803–812.
   PubMed Web of Science ®Google Scholar
• Kur-Zalewska J, Kisiel B, Kania-Pudło M, et al. A dose-dependent beneficial effect of methotrexate on the risk of interstitial lung disease in rheumatoid arthritis patients. PLoS One. 2021;16(4):1–11.
   Web of Science ®Google Scholar
• Juge PA, Lee JS, Lau J, et al. Methotrexate and rheumatoid arthritis associated interstitial lung disease. Eur Respir J. 2021;57(2). DOI:https://doi.org/10.1183/13993003.00337-2020.
   Google Scholar
• Ruaro B, Confalonieri M, Matucci-Cerinic M, et al. The treatment of lung involvement in systemic sclerosis. Pharmaceuticals. 2021;14(2):1–12.
   Web of Science ®Google Scholar
• Burt RK, Shah SJ, Dill K, et al. Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet. 2011;378(9790):498–506.
   PubMed Web of Science ®Google Scholar
• Khanna D, Denton CP, Lin CJF, et al. Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate). Ann Rheum Dis. 2018;77(2):212–220.
   PubMed Web of Science ®Google Scholar
• Khanna D, Lin CJF, Furst DE, et al. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med. 2020;8(10):963–974.
   PubMed Web of Science ®Google Scholar
• Keir GJ, Maher TM, Ming D, et al. Rituximab in severe, treatment-refractory interstitial lung disease. Respirology. 2014;19(3):353–359.
   PubMed Web of Science ®Google Scholar
• Duarte AC, Cordeiro A, Fernandes BM, et al. Rituximab in connective tissue disease-associated interstitial lung disease. Clin Rheumatol. 2019;38(7):2001–2009. DOI: https://doi.org/10.1007/s10067-019-04557-7.
   Web of Science ®Google Scholar
• Bejan-Angoulvant T, Naccache JM, Caille A, et al. Evaluation of efficacy and safety of rituximab in combination with mycophenolate mofetil in patients with nonspecific interstitial pneumonia non-responding to a first-line immunosuppressive treatment (EVER-ILD): a double-blind placebo-controlled randomize. Respir Med Res. 2020;78:100770.
   PubMedGoogle Scholar
• Kelly CA, Nisar M, Arthanari S, et al. Rheumatoid arthritis related interstitial lung disease - improving outcomes over 25 years: a large multicentre UK study. Revmatol. 2021;60:1882–1890.
   Google Scholar
• Vicente-Rabaneda EF, Atienza-Mateo B, Blanco R, et al. Efficacy and safety of abatacept in interstitial lung disease of rheumatoid arthritis: a systematic literature review. Autoimmun Rev. 2021;(6). DOI: https://doi.org/10.1016/j.autrev.2021.102830.
   Google Scholar
• Kremer Joel M. Effects of abatacept in patients with methotrexate-resistant active rheumatoid arthritis: a randomized trial. Ann Intern Med. 2006;144(12):865-76. doi:https://doi.org/10.7326/0003-4819-144-12-200606200-00003.
   Google Scholar
• Tardella M, Di M, Marina C, et al. Abatacept in rheumatoid arthritis ‑ associated interstitial lung disease : short ‑ term outcomes and predictors of progression. Clin Rheumatol. 2021;40(12):4861–4867. Published Online First: 2021. DOI: https://doi.org/10.1007/s10067-021-05854-w.
   Web of Science ®Google Scholar
• Harigai M. Growing evidence of the safety of JAK inhibitors in patients with rheumatoid arthritis. Rheumatol (United Kingdom). 2019;58:i34–42.
   PubMed Web of Science ®Google Scholar
• Rubbert-Roth A, Enejosa J, Pangan AL, et al. Trial of upadacitinib or abatacept in rheumatoid arthritis. N Engl J Med. 2020;383(16):1511–1521.
   PubMed Web of Science ®Google Scholar
• Vacchi C, Manfredi A, Cassone G, et al. Tofacitinib for the treatment of severe interstitial lung disease related to rheumatoid arthritis. Case Rep Med. 2021;2021:6–10.
   Web of Science ®Google Scholar
• Khanna D, Albera C, Fischer A, et al. An open-label, phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS trial. J Rheumatol. 2016;43(9):1672–1679.
   PubMed Web of Science ®Google Scholar
• Maher TM, Mayes MD, Kreuter M, et al. Effect of nintedanib on lung function in patients with systemic sclerosis−associated interstitial lung disease: further analyses of a randomized, double-blind, placebo-controlled trial. Arthritis Rheumatol. 2021;73(4):671–676.
   PubMed Web of Science ®Google Scholar
• Highland KB, Distler O, Kuwana M, et al. Efficacy and safety of nintedanib in patients with systemic sclerosis-associated interstitial lung disease treated with mycophenolate: a subgroup analysis of the SENSCIS trial. Lancet Respir Med. 2021;9(1):96–106.
   PubMed Web of Science ®Google Scholar
• Morisset J, Johannson KA, Vittinghoff E, et al. Use of mycophenolate mofetil or azathioprine for the management of chronic hypersensitivity pneumonitis. Chest. 2017;151(3):619–625.
   PubMed Web of Science ®Google Scholar
• Adegunsoye A, Oldham JM, Fernández Pérez ER, et al. Outcomes of immunosuppressive therapy in chronic hypersensitivity pneumonitis. ERJ Open Res. 2017;3:00016–2017.
   PubMedGoogle Scholar
• Shibata S, Furusawa H, Inase N., et al. Pirfenidone in chronic hypersensitivity pneumonitis: a real-life experience. Sarcoidosis, Vasc Diffus Lung Dis off J WASOG. 2018;35:139–142.
   PubMed Web of Science ®Google Scholar
• Mateos-Toledo H, Mejía-Ávila M, Rodríguez-Barreto Ó, et al. An open-label study with pirfenidone on chronic hypersensitivity pneumonitis. Arch Bronconeumol. 2020;56(3):163–169.
   PubMed Web of Science ®Google Scholar
• Tzilas V, Tzouvelekis A, Bouros E, et al. Clinical experience with antifibrotics in fibrotic hypersensitivity pneumonitis: a 3-year real-life observational study. ERJ Open Res. 2020;6(4):00152–2020.
   PubMedGoogle Scholar
• Wells AU, Brown KK, Flaherty KR, et al. What’s in a name? that which we call IPF, by any other name would act the same. Eur Respir J. 2018;51(5):1–12.
   Web of Science ®Google Scholar
• Jürgen Behr M, Antje Prasse M, Michael Kreuter M, et al. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med. 2021;9(5):476-486. doi:https://doi.org/10.1016/S2213-2600(20)30554-3
   Google Scholar
• Maher TM, Corte TJ, Fischer A, et al. Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med. 2020;8(2):147–157.
   PubMed Web of Science ®Google Scholar
• Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019:1–10. DOI:https://doi.org/10.1056/nejmoa1908681.
   Google Scholar
• Wells AU, Flaherty KR, Brown KK, et al. Nintedanib in patients with progressive fibrosing interstitial lung diseases—subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. Lancet Respir Med. 2020;8(5):453–460.
   PubMed Web of Science ®Google Scholar
• Baughman RP, Valeyre D, Korsten P, et al. ERS clinical practice guidelines on treatment of sarcoidosis. Eur Respir J. 2021:2004079. DOI:https://doi.org/10.1183/13993003.04079-2020.
   Google Scholar
• Paramothayan NS, Lasserson TJ, Jones P., et al.Corticosteroids for pulmonary sarcoidosis. Cochrane Database Syst Rev. 2005;2010. DOI:https://doi.org/10.1002/14651858.CD001114.pub2.
   Google Scholar
• Russell E, Luk F, Manocha S, et al. Long term follow-up of infliximab efficacy in pulmonary and extra-pulmonary sarcoidosis refractory to conventional therapy. Semin Arthritis Rheum. 2013;43(1):119–124.
   PubMed Web of Science ®Google Scholar
• Sweiss NJ, Noth I, Mirsaeidi M, et al. Efficacy results of a 52-week trial of adalimumab in the treatment of refractory sarcoidosis. Sarcoidosis, Vasc Diffus Lung Dis off J WASOG. 2014;31:46–54.
   PubMed Web of Science ®Google Scholar
• Behr J, Prasse A, Kreuter M, et al. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med. 2021;9(5):476–486.
   PubMed Web of Science ®Google Scholar
• Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis–associated interstitial lung disease. N Engl J Med. 2019;380(26):2518–2528.
   PubMed Web of Science ®Google Scholar