{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T18:45:39Z","timestamp":1792521939472,"version":"4.5.0"},"reference-count":230,"publisher":"MDPI AG","issue":"24","license":[{"start":{"date-parts":[[2025,12,17]],"date-time":"2025-12-17T00:00:00Z","timestamp":1765929600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Connective tissue disease-associated interstitial lung disease (CTD-ILD) represents a significant cause of morbidity and mortality. It is characterized by the progressive convergence of chronic inflammation, immune dysregulation, and fibrotic remodeling in the lung parenchyma. While often conceptualized through a model of idiopathic pulmonary fibrosis (IPF), CTD-ILD is fundamentally an immune-driven pathology with distinct inflammatory mechanisms in which adaptive immunity plays a profound role. This narrative review explores the \u201cinflammation\u2013immunity\u2013fibrosis continuum\u201d in CTD-ILD, elaborating the intricate cellular and molecular pathways that distinguish it from IPF. We highlight the central role of persistent T-cell responses and B-cell dysregulation, which often occur within organized tertiary lymphoid structures in the lung. This review examines how these immune processes are propagated by multiple cytokine pathways, including the TGF-\u03b2\/SMAD, JAK\/STAT, and phosphodiesterase-4 signaling pathways, which serve as crucial links between inflammation and fibrosis. This distinct immune mechanism in CTD-ILD explains why immunomodulatory agents are a cornerstone of CTD-ILD treatment, in contrast to their limited efficacy in IPF, and emphasizes the current paradigm of combining immunosuppression with antifibrotic drugs to target the dual drivers of the disease.<\/jats:p>","DOI":"10.3390\/ijms262412126","type":"journal-article","created":{"date-parts":[[2025,12,17]],"date-time":"2025-12-17T09:43:46Z","timestamp":1765964626000},"page":"12126","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Immunopathogenic Mechanisms in Connective Tissue Disease-Associated Interstitial Lung Disease: Incessant Loop of Immunity to Fibrosis"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0932-2826","authenticated-orcid":false,"given":"Jae Ha","family":"Lee","sequence":"first","affiliation":[{"name":"Division of Pulmonology and Critical Care Medicine, Inje University Haeundae Paik Hospital, Inje University College of Medicine, Busan 48108, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji Hoon","family":"Jang","sequence":"additional","affiliation":[{"name":"Division of Pulmonology and Critical Care Medicine, Inje University Haeundae Paik Hospital, Inje University College of Medicine, Busan 48108, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3640-9185","authenticated-orcid":false,"given":"Sunggun","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Inje University Haeundae Paik Hospital, Inje University College of Medicine, Busan 48108, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2733-9501","authenticated-orcid":false,"given":"Minyoung","family":"Her","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Inje University Haeundae Paik Hospital, Inje University College of Medicine, Busan 48108, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,12,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Cerro Chiang, G., and Parimon, T. 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