Abstract
Background: Interstitial Lung Diseases (ILDs) are characterized by shortness of breath caused by alveolar wall inflammation and/or fibrosis.
Objective: Our review aims to study the depth of various variants of ILD, diagnostic procedures, pathophysiology, molecular dysfunction and regulation, subject and objective assessment techniques, pharmacological intervention, exercise training and various modes of delivery for rehabilitation.
Method: Articles are reviewed from PubMed and Scopus and search engines.
Results: ILD is a rapidly progressing disease with a high mortality rate. Each variant has its own set of causal agents and expression patterns. Patients often find it challenging to self-manage due to persistent symptoms and a rapid rate of worsening. The present review elaborated on the pathophysiology, risk factors, molecular mechanisms, diagnostics, and therapeutic approaches for ILD will guide future requirements in the quest for innovative and tailored ILD therapies at the molecular and cellular levels.
Conclusion: The review highlights the rationale for conventional and novel therapeutic approaches for better management of ILD.
Graphical Abstract
[http://dx.doi.org/10.1183/09059180.00009113]
[http://dx.doi.org/10.1183/09031936.00131912] [PMID: 23222877]
[http://dx.doi.org/10.1183/16000617.0076-2018]
[http://dx.doi.org/10.1164/rccm.201308-1483ST] [PMID: 24032382]
[http://dx.doi.org/10.1016/j.pupt.2013.01.008] [PMID: 23380438]
[http://dx.doi.org/10.1111/crj.13466] [PMID: 35001525]
[http://dx.doi.org/10.1056/NEJMoa1103690] [PMID: 21992121]
[http://dx.doi.org/10.3390/ph14080819] [PMID: 34451916]
[http://dx.doi.org/10.1164/rccm.201706-1301OC] [PMID: 28889759]
[http://dx.doi.org/10.1378/chest.14-3218] [PMID: 26020856]
[http://dx.doi.org/10.1126/scitranslmed.3004700]
[http://dx.doi.org/10.1146/annurev-med-042711-142004] [PMID: 23020878]
[http://dx.doi.org/10.1083/jcb.201402006] [PMID: 25332161]
[http://dx.doi.org/10.1586/17476348.2016.1152186] [PMID: 26854382]
[http://dx.doi.org/10.1172/JCI87440] [PMID: 28145905]
[http://dx.doi.org/10.1172/JCI74942] [PMID: 25562319]
[http://dx.doi.org/10.1038/nm.4447]
[http://dx.doi.org/10.1016/j.immuni.2016.01.001] [PMID: 26921108]
[http://dx.doi.org/10.1165/rcmb.2014-0038OC] [PMID: 24918270]
[http://dx.doi.org/10.4049/jimmunol.1600265]
[http://dx.doi.org/10.1164/rccm.201607-1484OC] [PMID: 27684041]
[http://dx.doi.org/10.1016/S0140-6736(17)30866-8] [PMID: 28365056]
[http://dx.doi.org/10.1164/rccm.201506-1063ST] [PMID: 26177183]
[http://dx.doi.org/10.1136/thoraxjnl-2017-209967] [PMID: 28798214]
[http://dx.doi.org/10.3389/fmed.2017.00154] [PMID: 28993806]
[http://dx.doi.org/10.1164/rccm.201611-2201PP] [PMID: 28598197]
[http://dx.doi.org/10.1038/s41572-020-0191-z] [PMID: 32764620]
[http://dx.doi.org/10.4103/0366-6999.198917] [PMID: 28139521]
[http://dx.doi.org/10.1097/MCP.0000000000000144] [PMID: 25621562]
[http://dx.doi.org/10.1067/j.cpradiol.2014.10.002] [PMID: 25444537]
[http://dx.doi.org/10.1111/resp.12674] [PMID: 26564810]
[http://dx.doi.org/10.1016/j.jcpa.2013.03.006] [PMID: 23664319]
[http://dx.doi.org/10.1183/09059180.00008014]
[http://dx.doi.org/10.1002/art.38904] [PMID: 25302945]
[http://dx.doi.org/10.1371/journal.pone.0229997]
[http://dx.doi.org/10.1183/13993003.00357-2015] [PMID: 26585429]
[http://dx.doi.org/10.3390/cells9030691] [PMID: 32168865]
[http://dx.doi.org/10.1378/chest.13-2626] [PMID: 24576924]
[http://dx.doi.org/10.1007/s10067-019-04922-6] [PMID: 31916109]
[http://dx.doi.org/10.1097/BOR.0000000000000111] [PMID: 25191993]
[http://dx.doi.org/10.1016/j.pharmthera.2020.107798] [PMID: 33359599]
[http://dx.doi.org/10.1080/13543784.2020.1782885] [PMID: 32538186]
[http://dx.doi.org/10.3389/fphar.2021.797292] [PMID: 35126134]
[http://dx.doi.org/10.1111/all.15017] [PMID: 34293188]
[http://dx.doi.org/10.1097/CM9.0000000000001461] [PMID: 33879753]
[http://dx.doi.org/10.3390/app112110313]
[http://dx.doi.org/10.3181/00379727-214-44065] [PMID: 9012357]
[http://dx.doi.org/10.1016/S0891-5849(03)00060-1] [PMID: 12706492]
[http://dx.doi.org/10.1164/ajrccm.157.5.9707141] [PMID: 9603153]
[http://dx.doi.org/10.1016/j.amsu.2021.102245] [PMID: 33854771]
[http://dx.doi.org/10.3390/cells12081154] [PMID: 37190064]
[http://dx.doi.org/10.1007/978-3-031-12950-6_34]
[http://dx.doi.org/10.1183/16000617.0011-2021] [PMID: 34168062]
[http://dx.doi.org/10.1016/S2665-9913(23)00008-5]
[http://dx.doi.org/10.1007/s11926-014-0411-1] [PMID: 24523015]
[http://dx.doi.org/10.1513/AnnalsATS.201606-426FR] [PMID: 27560196]
[http://dx.doi.org/10.1016/S2213-2600(19)30480-1] [PMID: 32113575]
[http://dx.doi.org/10.3390/ijms24032405] [PMID: 36768729]
[http://dx.doi.org/10.1186/2213-0802-2-4] [PMID: 25505696]
[http://dx.doi.org/10.1183/16000617.0062-2017]
[http://dx.doi.org/10.1371/journal.pone.0026059] [PMID: 22163265]
[http://dx.doi.org/10.1023/A:1015949118495] [PMID: 12162428]
[http://dx.doi.org/10.1165/rcmb.4903] [PMID: 12540492]
[http://dx.doi.org/10.1016/j.cbi.2010.03.047] [PMID: 20380827]
[http://dx.doi.org/10.1164/rccm.201002-0185OC] [PMID: 20639441]
[http://dx.doi.org/10.1513/pats.200601-003TK] [PMID: 16738202]
[http://dx.doi.org/10.1186/1755-1536-5-S1-S23] [PMID: 23259497]
[http://dx.doi.org/10.1016/j.ccm.2011.11.002] [PMID: 22365247]
[http://dx.doi.org/10.1016/j.bbadis.2013.03.001] [PMID: 23499992]
[http://dx.doi.org/10.1164/rccm.201306-1141WS] [PMID: 24160862]
[http://dx.doi.org/10.1038/s41598-019-41933-4] [PMID: 30940853]
[http://dx.doi.org/10.1165/rcmb.2005-0352OC] [PMID: 16357363]
[http://dx.doi.org/10.1164/rccm.200602-203OC] [PMID: 16946128]
[http://dx.doi.org/10.1096/fj.201601077R] [PMID: 28258190]
[http://dx.doi.org/10.1371/journal.pone.0121246] [PMID: 25785991]
[http://dx.doi.org/10.1016/j.cmet.2015.11.011] [PMID: 26686024]
[PMID: 9843857]
[http://dx.doi.org/10.1016/j.exger.2006.02.006] [PMID: 16600555]
[http://dx.doi.org/10.1172/JCI115501] [PMID: 1752942]
[http://dx.doi.org/10.1183/09031936.93.04040400] [PMID: 1855569]
[http://dx.doi.org/10.1378/chest.104.1.47] [PMID: 8325116]
[http://dx.doi.org/10.1164/ajrccm/147.6_Pt_1.1507] [PMID: 8099261]
[http://dx.doi.org/10.1164/ajrccm.152.5.7582298] [PMID: 7582298]
[http://dx.doi.org/10.1056/NEJM198707233170404] [PMID: 3600711]
[http://dx.doi.org/10.1183/09031936.00125407] [PMID: 18216050]
[http://dx.doi.org/10.4049/jimmunol.179.4.2592] [PMID: 17675522]
[http://dx.doi.org/10.2214/AJR.11.7329] [PMID: 22623570]
[http://dx.doi.org/10.1164/rccm.201309-1634ST] [PMID: 24127811]
[PMID: 34559419]
[http://dx.doi.org/10.1136/thoraxjnl-2016-208638] [PMID: 28213592]
[http://dx.doi.org/10.1186/s12931-018-0884-y] [PMID: 30236104]
[http://dx.doi.org/10.1016/j.rmed.2013.11.016] [PMID: 24332409]
[PMID: 25006558]
[http://dx.doi.org/10.1177/1479972313493098] [PMID: 23897928]
[http://dx.doi.org/10.1016/j.ccm.2019.02.011] [PMID: 31078218]
[http://dx.doi.org/10.1186/s12890-019-0936-1] [PMID: 31638951]
[http://dx.doi.org/10.1111/resp.12360] [PMID: 25112470]
[http://dx.doi.org/10.1111/resp.12945] [PMID: 27862639]
[http://dx.doi.org/10.1016/j.apmr.2016.05.017] [PMID: 27320420]
[http://dx.doi.org/10.1378/chest.96.4.767] [PMID: 2791670]
[http://dx.doi.org/10.1016/j.rmed.2004.10.018] [PMID: 15878493]
[http://dx.doi.org/10.1136/thx.54.7.581] [PMID: 10377201]
[http://dx.doi.org/10.1097/01823246-200718020-00005]
[http://dx.doi.org/10.1016/j.rmed.2021.106436] [PMID: 33992496]
[http://dx.doi.org/10.1016/S0099-1767(00)90093-X] [PMID: 10839848]
[http://dx.doi.org/10.1513/AnnalsATS.201703-244FR] [PMID: 28933948]
[http://dx.doi.org/10.1016/S0272-5231(21)01076-5] [PMID: 8088096]
[http://dx.doi.org/10.1159/000192773] [PMID: 19246958]
[http://dx.doi.org/10.1249/01.MSS.0000117161.66394.07] [PMID: 15076800]
[PMID: 14258950]
[http://dx.doi.org/10.1093/ije/24.2.381] [PMID: 7635600]
[http://dx.doi.org/10.1081/COPD-200050513] [PMID: 17136966]
[http://dx.doi.org/10.1002/acr.20541] [PMID: 22588759]
[http://dx.doi.org/10.1186/1477-7525-1-29] [PMID: 12914662]
[http://dx.doi.org/10.1081/COPD-200050651] [PMID: 17136967]
[http://dx.doi.org/10.1186/1477-7525-8-77] [PMID: 20673370]
[http://dx.doi.org/10.1136/thoraxjnl-2012-201581] [PMID: 22555278]
[http://dx.doi.org/10.1111/resp.12807] [PMID: 27173103]
[http://dx.doi.org/10.1177/1479972315626014] [PMID: 26768011]
[http://dx.doi.org/10.1016/j.physio.2015.10.002] [PMID: 26596172]
[http://dx.doi.org/10.1186/s12931-019-1019-9] [PMID: 30866948]
[http://dx.doi.org/10.1183/09031936.00081512] [PMID: 23100507]
[http://dx.doi.org/10.1016/j.rmr.2011.06.006] [PMID: 21943547]
[http://dx.doi.org/10.1183/23120541.00045-2019] [PMID: 30972352]
[http://dx.doi.org/10.1097/HCR.0000000000000418] [PMID: 31241519]
[http://dx.doi.org/10.31189/2165-7629-9.2.67]
[http://dx.doi.org/10.1378/chest.06-2418] [PMID: 17494825]
[http://dx.doi.org/10.5772/intechopen.84283]
[http://dx.doi.org/10.1177/1753465816676048] [PMID: 28150539]
[http://dx.doi.org/10.1097/HCR.0000000000000083] [PMID: 25313451]
[http://dx.doi.org/10.1080/09593985.2019.1678207]
[http://dx.doi.org/10.1016/j.rmed.2011.11.014] [PMID: 22182340]
[http://dx.doi.org/10.1016/j.resp.2019.103367] [PMID: 31911201]
[http://dx.doi.org/10.1016/j.resp.2009.01.010] [PMID: 19450767]
[http://dx.doi.org/10.1016/j.arbr.2016.11.003] [PMID: 27818024]
[http://dx.doi.org/10.1159/000370126] [PMID: 25633076]
[http://dx.doi.org/10.1016/j.pec.2019.01.010] [PMID: 30683609]
[http://dx.doi.org/10.1513/AnnalsATS.201512-836OC] [PMID: 27064659]
[http://dx.doi.org/10.1111/resp.13913]
[http://dx.doi.org/10.1056/NEJMra1012478] [PMID: 22435372]
[http://dx.doi.org/10.1152/japplphysiol.01277.2013]
[PMID: 26770084]
[http://dx.doi.org/10.1298/ptr.R0012] [PMID: 33981523]
[http://dx.doi.org/10.1183/13993003.congress-2018.PA4148]
[http://dx.doi.org/10.1378/chest.109.6.1566] [PMID: 8769513]
[http://dx.doi.org/10.2165/00007256-200131010-00002] [PMID: 11219499]