Authors: Martina Šterclová Authors‘ workplace: Pneumologická klinika 2. LF UK a FN Motol a Homolka, Pneumologická klinika 1. LF UK a FTN, Praha Published in: Čes. slov. Farm., 2026; 75, 8-13 Category: Current Pharmacotherapy doi: https://doi.org/10.36290/csf.2026.003
Idiopathic interstitial pneumonias (IIPs) represent a heterogeneous group of rare lung diseases characterized by distinct radiological and histological phenotypes. The new 2025 classification provides a more detailed definition of individual entities, including the incorporation of idiopathic eosinophilic pneumonias and rare forms, without yet altering current therapeutic approaches. The most common form is idiopathic pulmonary fibrosis (IPF), where antifibrotic therapy with pirfenidone and nintedanib has been shown to improve prognosis. Recent clinical trials (FIBRONEER, TETON) suggest further therapeutic options. The treatment of other IIPs, particularly nonspecific interstitial pneumonia (NSIP), is based on systemic corticosteroids and immunosuppressive therapy, though evidence remains limited. Complications such as pulmonary hypertension and acute exacerbations significantly impact disease course. Non-pharmacological strategies, including oxygen therapy, pulmonary rehabilitation, and, in selected cases, lung transplantation, play an important role.
idiopathic pulmonary fibrosis – treatment – idiopathic interstitial pneumonias – lung transplantation.
1. Ryerson CJ, Adegunsoye A, Piciucchi S, et al. Update of the International Multidisciplinary Classification of the Interstitial Pneumonias: An ERS/ATS Statement. Eur Respir J. 2025 : 2500158.
2. Slabbynck K, Van Dorpe J, Peene I, et al. Endogenous Lipoid Pneumonia in Adult Autoinflammatory Disease. Arthritis Care Res (Hoboken) 2025 May 14. doi: 10.1002/acr.25570.
3. Gupta R, Morgan AD, George PM, Quint JK. Incidence, prevalence and mortality of idiopathic pulmonary fibrosis in England from 2008 to 2018: a cohort study. Thorax. 2024;79(7):624-631.
4. Kaul B, Lee JS, Zhang N, et al. Epidemiology of Idiopathic Pulmonary Fibrosis among U.S. Veterans, 2010-2019. Ann Am Thorac Soc. 2022;19(2):196-203.
5. Maher TM, Assassi S, Azuma A, et al; FIBRONEER-ILD Trial Investigators. Nerandomilast in Patients with Progressive Pulmonary Fibrosis. N Engl J Med 2025;392(22):2203-2214.
6. Richeldi L, Azuma A, Cottin V, et al; FIBRONEER-IPF Trial Investigators. Nerandomilast in Patients with Idiopathic Pulmonary Fibrosis. N Engl J Med. 2025;392(22):2193-2202.
7. Oldham JM, Ma SF, Martinez FJ, et al. TOLLIP, MUC5B, and the Response to N-Acetylcysteine among Individuals with Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2015;192(12):1475-82.
8. Mankikian J, Caille A, Reynaud-Gaubert M, et al; EVER-ILD investigators and the OrphaLung network. Rituximab and mycophenolate mofetil combination in patients with interstitial lung disease (EVER-ILD): a double-blind, randomised, placebo-controlled trial. Eur Respir J. 2023;61(6):2202071.
9. Lee SH, Park MS, Kim SY, et al. Factors affecting treatment outcome in patients with idiopathic nonspecific interstitial pneumonia: a nationwide cohort study. Respir Res. 2017;18(1):204.
10. Travis WD, Hunninghake G, King TE Jr, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med. 2008;177(12):1338-47.
11. Buschulte K, Kabitz HJ, Hagmeyer L, et al. Disease trajectories in interstitial lung diseases - data from the EXCITING-ILD registry. Respir Res. 2024;25(1):113.
12. Flaherty KR, Wells AU, Cottin V, et al; INBUILD Trial Investigators. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med 2019;381(18):1718-1727.
13. Radzikowska E, Fijolek J. Update on cryptogenic organizing pneumonia. Front Med (Lausanne). 2023;10 : 1146782.
14. Hellemons ME, Moor CC, von der Thüsen J, et al. Desquamative interstitial pneumonia: a systematic review of its features and outcomes. Eur Respir Rev 2020;29(156):190181.
15. Valladares C, Narvel A, Koutsenko B, et al. Examining the Role of Corticosteroids in the Management of Acute Interstitial Pneumonia: A Systematic Review. J Clin Med Res. 2025;17(4):223-230.
16. Murillo AD, Castrillon AI, Serrano CD, Fernandez-Trujillo L. Monoclonal antibodies in idiopathic chronic eosinophilic pneumonia: a scoping review. BMC Pulm Med 2024;24(1):74.
17. Sergew A, Fernández Pérez ER. Current Approach to Diagnosis and Management of Pulmonary Eosinophilic Syndromes: Eosinophilic Pneumonias, Eosinophilic Granulomatosis with Polyangiitis, and Hypereosinophilic Syndrome. Semin Respir Crit Care Med. 2016;37(3):441-56.
18. Lawrence A, Myall KJ, Mukherjee B, Marino P. Converging Pathways: A Review of Pulmonary Hypertension in Interstitial Lung Disease. Life (Basel). 2024;14(9):1203.
19. Ang HL, Schulte M, Chan RK, et al. Pulmonary Hypertension in Interstitial Lung Disease: A Systematic Review and Meta-Analysis. Chest. 2024;166(4):778-792.
20. Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease. N Engl J Med 2021;384(4):325-334.
21. Waxman A, Restrepo-Jaramillo R, Thenappan T, et al. Long-term inhaled treprostinil for pulmonary hypertension due to interstitial lung disease: INCREASE open-label extension study. Eur Respir J. 2023;61(6):2202414.
22. Nathan SD, Behr J, Cottin V, et al. Study design and rationale for the TETON phase 3, randomised, controlled clinical trials of inhaled treprostinil in the treatment of idiopathic pulmonary fibrosis. BMJ Open Respir Res. 2022;9(1):e001310.
23. Yogeswaran A, Hassoun PM, Saleh K, et al.; PVRI-GoDeep-Consortium. Hemodynamics and Phosphodiesterase-5 Inhibitor Treatment Associated with Survival in ILD-PH: A PVRI GoDeep Meta-Registry Analysis. Am J Respir Crit Care Med. 2025. doi: 10.1164/rccm.202412-2371OC.
24. Collard HR, Ryerson CJ, Corte TJ, et al. Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report. Am J Respir Crit Care Med. 2016;194(3):265-75.
25. Kim MJ, Yang J, Song JW. Acute exacerbation of progressive pulmonary fibrosis: incidence and outcomes. Respir Res. 2024;25(1):415.
26. Srivali N, De Giacomi F, Moua T, Ryu JH. Corticosteroid therapy for treating acute exacerbation of interstitial lung diseases: a systematic review. Thorax. 2025;80(3):140-149.
27. Yamakawa H, Sato S, Ohta H, et al. Baricitinib for Acute Exacerbation in Idiopathic Interstitial Pneumonias: A Preliminary Study Using Propensity Score Matching. Respiration. 2025 : 1-12.
28. Creamer AW, Barratt SL. Does ambulatory oxygen improve quality of life in patients with fibrotic lung disease? Results from the AmbOx trial. Breathe (Sheff). 2019;15(2):140-143.
29. Lin LY, Wu YC, Wu JS, et al. Oxygen therapy for exercise capacity in fibrotic interstitial lung disease: A systematic review and meta-analysis of randomised controlled trials. Respir Med. 2024;227 : 107657.
30. Khor YH, Palm A, Wong AW, et al. Effects of long-term oxygen therapy on acute exacerbation and hospital burden: the national DISCOVERY study. Thorax. 2025;80(6):378-384.
31. Palm A, Ekström M. Hypoxemia severity and survival in ILD and COPD on long-term oxygen therapy - The population-based DISCOVERY study. Respir Med. 2021;189 : 106659.
32. Özmen İ, Yıldırım E, Karakış M, et al. Are the gains from pulmonary rehabilitation the same in idiopathic pulmonary fibrosis and other interstitial lung diseases? Sarcoidosis Vasc Diffuse Lung Dis. 2024;41(3):e2024038.
33. Zhao Q, Mo N, Li R, et al. Effects of exercise training on exercise capacity of patients with interstitial lung disease: a systematic review and meta-analysis. BMC Pulm Med. 2025;25(1):356.
34. Selman M, Buendia-Roldan I, Pardo A. Decoding the complexity: mechanistic insights into comorbidities in idiopathic pulmonary fibrosis. Eur Respir J. 2025;65(5):2402418.
35. Leard LE, Holm AM, Valapour M, et al. Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2021;40(11):1349-1379.
36. Yang L, Xiang Z, Dai M, et al. Prognosis of Lung Transplantation in Patients with Acute Exacerbations of Interstitial Lung Disease: A Meta-Analysis Based on Cohort Studies. Ann Thorac Cardiovasc Surg. 2024;30(1):24-00086.
37. Warrior K, Sayad K, O'Hara CP, Dilling DF. Impact of Acute Exacerbation of Idiopathic Pulmonary Fibrosis on Lung Transplant Outcomes. Transplantation. 2024;108(6):1460-1465.
38. Dotan Y, Vaidy A, Shapiro WB, et al. Effect of Acute Exacerbation of Idiopathic Pulmonary Fibrosis on Lung Transplantation Outcome. Chest. 2018;154(4):818-826.
Don‘t have an account? Create new account
Enter the email address that you registered with. We will send you instructions on how to set a new password.
