{"id":"https://openalex.org/W7224951003","doi":"https://doi.org/10.3390/ijms27219777","title":"Emerging Therapies in Pulmonary Fibrosis: A Common Pathway for the Action of Diverse Pharmacologic Agents","display_name":"Emerging Therapies in Pulmonary Fibrosis: A Common Pathway for the Action of Diverse Pharmacologic Agents","publication_year":2026,"publication_date":"2026-10-30","ids":{"openalex":"https://openalex.org/W7224951003","doi":"https://doi.org/10.3390/ijms27219777"},"language":"en","primary_location":{"id":"doi:10.3390/ijms27219777","doi":"https://doi.org/10.3390/ijms27219777","is_oa":true,"landing_page_url":"https://doi.org/10.3390/ijms27219777","pdf_url":null,"source":{"id":"https://openalex.org/S10623703","display_name":"International Journal of Molecular Sciences","issn_l":"1422-0067","issn":["1422-0067","1661-6596"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","medline","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310310987","host_organization_name":"Multidisciplinary Digital Publishing Institute","host_organization_lineage":["https://openalex.org/P4310310987"],"host_organization_lineage_names":["Multidisciplinary Digital Publishing Institute"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"International Journal of Molecular Sciences","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://doi.org/10.3390/ijms27219777","any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5009835317","display_name":"Graeme B. Bolger","orcid":"https://orcid.org/0000-0002-0797-7792","observed_orcids":["https://orcid.org/0000-0002-0797-7792"]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Graeme B. Bolger","raw_affiliation_strings":["BZI Pharma LLC, 100 Cummings Center, Suite 241-D, Beverly, MA, 01915, USA"],"raw_orcid":"https://orcid.org/0000-0002-0797-7792","affiliations":[{"raw_affiliation_string":"BZI Pharma LLC, 100 Cummings Center, Suite 241-D, Beverly, MA, 01915, USA","institution_ids":[]}]}],"institutions":[],"countries_distinct_count":0,"institutions_distinct_count":0,"corresponding_author_ids":["https://openalex.org/A5009835317"],"corresponding_institution_ids":[],"apc_list":{"value":2900,"currency":"CHF","value_usd":3499},"apc_paid":{"value":2900,"currency":"CHF","value_usd":3499},"fwci":0.0,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":{"value":0.85736943,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"27","issue":"21","first_page":"9777","last_page":"9777"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10870","display_name":"Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis","score":0.9976000189781189,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10870","display_name":"Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis","score":0.9976000189781189,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10728","display_name":"Pulmonary Hypertension Research and Treatments","score":0.0013000000035390258,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11330","display_name":"Systemic Sclerosis and Related Diseases","score":0.0003000000142492354,"subfield":{"id":"https://openalex.org/subfields/2745","display_name":"Rheumatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/pulmonary-fibrosis","display_name":"pulmonary fibrosis","score":0.9919999837875366},{"id":"https://openalex.org/keywords/pulmonary-inflammation","display_name":"lung inflammation","score":0.7699999809265137},{"id":"https://openalex.org/keywords/cytokine-signaling","display_name":"cytokine signaling","score":0.8140000104904175},{"id":"https://openalex.org/keywords/intracellular-signaling","display_name":"intracellular signaling","score":0.8989999890327454},{"id":"https://openalex.org/keywords/camp-signaling","display_name":"cAMP signaling","score":0.8180000185966492},{"id":"https://openalex.org/keywords/phosphodiesterase-4-inhibitors","display_name":"phosphodiesterase-4 inhibitors","score":0.7850000262260437},{"id":"https://openalex.org/keywords/treprostinil","display_name":"treprostinil","score":0.9909999966621399},{"id":"https://openalex.org/keywords/prostacyclin-analogues","display_name":"prostacyclin analogues","score":0.9539999961853027},{"id":"https://openalex.org/keywords/anti-inflammatory-therapy","display_name":"anti-inflammatory therapy","score":0.48500001430511475},{"id":"https://openalex.org/keywords/extracellular-matrix-deposition","display_name":"extracellular matrix deposition","score":0.7620000243186951}],"concepts":[{"id":"https://openalex.org/C2776914184","wikidata":"https://www.wikidata.org/wiki/Q101991","display_name":"Inflammation","level":2,"score":0.7117000222206116},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.6869000196456909},{"id":"https://openalex.org/C2777714996","wikidata":"https://www.wikidata.org/wiki/Q7886","display_name":"Lung","level":2,"score":0.6118999719619751},{"id":"https://openalex.org/C2780559512","wikidata":"https://www.wikidata.org/wiki/Q605709","display_name":"Fibrosis","level":2,"score":0.54830002784729},{"id":"https://openalex.org/C2780942790","wikidata":"https://www.wikidata.org/wiki/Q372016","display_name":"Pathogenesis","level":2,"score":0.5123000144958496},{"id":"https://openalex.org/C2778341716","wikidata":"https://www.wikidata.org/wiki/Q2290446","display_name":"Idiopathic pulmonary fibrosis","level":3,"score":0.5008999705314636},{"id":"https://openalex.org/C62478195","wikidata":"https://www.wikidata.org/wiki/Q828130","display_name":"Signal transduction","level":2,"score":0.4794999957084656},{"id":"https://openalex.org/C2781244666","wikidata":"https://www.wikidata.org/wiki/Q32446","display_name":"Pulmonary fibrosis","level":3,"score":0.47209998965263367},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.44279998540878296},{"id":"https://openalex.org/C2778690821","wikidata":"https://www.wikidata.org/wiki/Q212354","display_name":"Cytokine","level":2,"score":0.43959999084472656},{"id":"https://openalex.org/C189165786","wikidata":"https://www.wikidata.org/wiki/Q193825","display_name":"Extracellular matrix","level":2,"score":0.3463999927043915},{"id":"https://openalex.org/C60644358","wikidata":"https://www.wikidata.org/wiki/Q128570","display_name":"Bioinformatics","level":1,"score":0.3296999931335449},{"id":"https://openalex.org/C98274493","wikidata":"https://www.wikidata.org/wiki/Q128406","display_name":"Pharmacology","level":1,"score":0.32829999923706055},{"id":"https://openalex.org/C2780930700","wikidata":"https://www.wikidata.org/wiki/Q1128595","display_name":"Pulmonary hypertension","level":2,"score":0.3021000027656555},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.29499998688697815},{"id":"https://openalex.org/C2781128415","wikidata":"https://www.wikidata.org/wiki/Q412050","display_name":"Prostacyclin","level":2,"score":0.2809999883174896},{"id":"https://openalex.org/C2777543607","wikidata":"https://www.wikidata.org/wiki/Q1153419","display_name":"Interstitial lung disease","level":3,"score":0.2800999879837036},{"id":"https://openalex.org/C79879829","wikidata":"https://www.wikidata.org/wiki/Q5571762","display_name":"Intracellular","level":2,"score":0.2720000147819519},{"id":"https://openalex.org/C164027704","wikidata":"https://www.wikidata.org/wiki/Q3491837","display_name":"Proinflammatory cytokine","level":3,"score":0.2621999979019165},{"id":"https://openalex.org/C535046627","wikidata":"https://www.wikidata.org/wiki/Q30612","display_name":"Clinical trial","level":2,"score":0.25049999356269836}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.3390/ijms27219777","doi":"https://doi.org/10.3390/ijms27219777","is_oa":true,"landing_page_url":"https://doi.org/10.3390/ijms27219777","pdf_url":null,"source":{"id":"https://openalex.org/S10623703","display_name":"International Journal of Molecular Sciences","issn_l":"1422-0067","issn":["1422-0067","1661-6596"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","medline","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310310987","host_organization_name":"Multidisciplinary Digital Publishing Institute","host_organization_lineage":["https://openalex.org/P4310310987"],"host_organization_lineage_names":["Multidisciplinary Digital Publishing Institute"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"International Journal of Molecular Sciences","raw_type":"journal-article"}],"best_oa_location":{"id":"doi:10.3390/ijms27219777","doi":"https://doi.org/10.3390/ijms27219777","is_oa":true,"landing_page_url":"https://doi.org/10.3390/ijms27219777","pdf_url":null,"source":{"id":"https://openalex.org/S10623703","display_name":"International Journal of Molecular Sciences","issn_l":"1422-0067","issn":["1422-0067","1661-6596"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","medline","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310310987","host_organization_name":"Multidisciplinary Digital Publishing Institute","host_organization_lineage":["https://openalex.org/P4310310987"],"host_organization_lineage_names":["Multidisciplinary Digital Publishing Institute"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"International Journal of Molecular Sciences","raw_type":"journal-article"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":0.9476000070571899,"display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W7169503694","https://openalex.org/W2007244358","https://openalex.org/W7213980299","https://openalex.org/W4414188427","https://openalex.org/W7130421068","https://openalex.org/W2024260450","https://openalex.org/W3033331543","https://openalex.org/W2079498853","https://openalex.org/W7129015747","https://openalex.org/W7097933470"],"abstract_inverted_index":{"Fibrotic":[0,34],"lung":[1,35,72,86,99,197],"diseases":[2],"are":[3,110,192],"a":[4,95,173],"heterogeneous":[5],"set":[6],"of":[7,13,25,68,71,79,85,98,196],"disorders":[8],"characterized":[9],"by":[10,17,40,116],"progressive":[11],"infiltration":[12],"the":[14,69,76,83,90,134,149,154,176],"pulmonary":[15,27,147],"interstitium":[16],"inflammatory":[18],"cells":[19],"and":[20,29,58,74,119,153,199],"fibroblasts,":[21],"leading":[22,51],"to":[23,52,124],"destruction":[24],"normal":[26],"tissue":[28],"increased":[30],"extracellular":[31],"matrix":[32],"deposition.":[33],"disease":[36],"can":[37],"be":[38,125],"initiated":[39],"numerous":[41],"triggers\u2014genetic,":[42],"immunologic,":[43],"pharmacologic,":[44],"environmental,":[45],"infectious\u2014that":[46],"share":[47],"many":[48],"downstream":[49],"effectors,":[50],"injury":[53],"patterns":[54],"with":[55],"substantial":[56],"pathologic":[57],"clinical":[59,182,207],"overlap.":[60],"Recent":[61,136],"preclinical":[62],"studies":[63],"have":[64,102,121,140,185,204,212],"expanded":[65],"our":[66],"knowledge":[67],"mechanisms":[70],"inflammation":[73,93,132,200],"implicated":[75],"key":[77,96,194],"role":[78],"specific":[80,105],"cytokines":[81],"in":[82,114,133,138,146,202,215],"pathogenesis":[84],"inflammation.":[87],"They":[88,101],"reinforce":[89],"concept":[91],"that":[92,109,120,130,189,209],"is":[94,159],"precursor":[97],"fibrosis.":[100],"also":[103],"identified":[104],"intracellular":[106],"signaling":[107,118,178],"pathways":[108],"modulated,":[111],"at":[112],"least":[113],"part,":[115],"cytokine":[117],"been":[122],"shown":[123],"emerging":[126],"targets":[127,191],"for":[128],"drugs":[129,144,170],"target":[131],"lung.":[135],"advances":[137],"therapeutics":[139],"yielded":[141],"two":[142,169],"new":[143,181],"active":[145],"fibrosis,":[148],"PDE4-selective":[150],"inhibitor":[151],"nerandomilast":[152],"prostacyclin":[155],"analog":[156],"treprostinil.":[157],"There":[158],"now":[160],"compelling":[161],"evidence":[162,208],"that,":[163],"despite":[164],"their":[165],"structural":[166],"differences,":[167],"these":[168,190,210],"act":[171],"through":[172],"common":[174],"mechanism,":[175],"cAMP":[177],"pathway.":[179],"The":[180],"trial":[183],"results":[184],"provided":[186,205],"critical":[187],"confirmation":[188],"indeed":[193],"regulators":[195],"fibrosis":[198],"and,":[201],"turn,":[203],"well-validated":[206],"agents":[211],"therapeutic":[213],"value":[214],"patients.":[216]},"counts_by_year":[],"updated_date":"2026-11-01T11:40:01.658920","created_date":"2026-10-31T00:00:00"}
