{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T02:19:03Z","timestamp":1790216343966,"version":"4.1.0"},"reference-count":97,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2023,5,12]],"date-time":"2023-05-12T00:00:00Z","timestamp":1683849600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Lung involvement, especially interstitial lung disease, is a potentially severe extra-glandular manifestation of Primary Sjogren\u2019s Syndrome (pSS-ILD). ILD can manifest either as a late complication of pSS or anticipate sicca symptoms, likely reflecting two different patho-physiological entities. Presence of lung involvement in pSS subjects can remain subclinical for a long time; therefore, patients should be actively screened, and lung ultrasound is currently being investigated as a potential low cost, radiation-free, easily repeatable screening tool for detection of ILD. In contrast, rheumatologic evaluation, serology testing, and minor salivary gland biopsy are crucial for the recognition of pSS in apparently idiopathic ILD patients. Whether the HRCT pattern influences prognosis and treatment response in pSS-ILD is not clear; a UIP pattern associated with a worse prognosis in some studies, but not in others. Many aspects of pSS-ILD, including its actual prevalence, association with specific clinical\u2013serological characteristics, and prognosis, are still debated by the current literature, likely due to poor phenotypic stratification of patients in clinical studies. In the present review, we critically discuss these and other clinically relevant \u201chot topics\u201d in pSS-ILD. More specifically, after a focused discussion, we compiled a list of questions regarding pSS-ILD that, in our opinion, are not easily answered by the available literature. We subsequently tried to formulate adequate answers on the basis of an extensive literature search and our clinical experience. At the same, we highlighted different issues that require further investigation.<\/jats:p>","DOI":"10.3390\/jcm12103428","type":"journal-article","created":{"date-parts":[[2023,5,12]],"date-time":"2023-05-12T09:26:13Z","timestamp":1683883573000},"page":"3428","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Primary-Sj\u00f6gren\u2019s-Syndrome-Related Interstitial Lung Disease: A Clinical Review Discussing Current Controversies"],"prefix":"10.3390","volume":"12","author":[{"given":"Gaetano","family":"La Rocca","sequence":"first","affiliation":[{"name":"Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Ferro","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9933-1202","authenticated-orcid":false,"given":"Gianluca","family":"Sambataro","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Diseases, A.O.U. Policlinico \u201cG. Rodolico-San Marco\u201d, University of Catania, Via Santa Sofia 78, 95124 Catania, Italy"},{"name":"Artroreuma S.R.L., Rheumatology Outpatient Clinic Associated with the National Health System, Corso S. Vito 53, 95030 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Elefante","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Fonzetti","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni","family":"Fulvio","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inmaculada C.","family":"Navarro","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Mosca","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Baldini","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,5,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"931","DOI":"10.1056\/NEJMcp1702514","article-title":"Primary Sj\u00f6gren\u2019s Syndrome","volume":"378","author":"Mariette","year":"2018","journal-title":"N. Engl. J. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"e000022","DOI":"10.1136\/rmdopen-2014-000022","article-title":"EULAR Sj\u00f6gren\u2019s syndrome disease activity index [ESSDAI]: A user guide","volume":"1","author":"Seror","year":"2015","journal-title":"RMD Open."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1933","DOI":"10.1148\/rg.337125107","article-title":"CT Findings of Thoracic Manifestations of Primary Sj\u00f6gren Syndrome: Radiologic-Pathologic Correlation","volume":"33","author":"Egashira","year":"2013","journal-title":"Radiographics"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1183\/16000617.0011-2016","article-title":"Pulmonary manifestations of Sj\u00f6gren\u2019s syndrome","volume":"25","author":"Flament","year":"2016","journal-title":"Eur. Respir. Rev."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"2230","DOI":"10.1093\/rheumatology\/kev200","article-title":"Characterization of systemic disease in primary Sj\u00f6gren\u2019s syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements","volume":"54","author":"Seror","year":"2015","journal-title":"Rheumatology"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1009","DOI":"10.1111\/1756-185X.13881","article-title":"Prevalence and risk factors of interstitial lung disease in patients with primary Sj\u00f6gren\u2019s syndrome: A systematic review and meta-analysis","volume":"23","author":"He","year":"2020","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1016\/j.autrev.2010.05.004","article-title":"Immunological profile in primary Sj\u00f6gren syndrome: Clinical significance, prognosis and long-term evolution to other auto-immune disease","volume":"9","author":"Fauchais","year":"2010","journal-title":"Autoimmun. Rev."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1097\/00005382-200110000-00009","article-title":"Lung Manifestation in Asymptomatic Patients with Primary Sj\u00f6gren Syndrome: Assessment with High Resolution CT and Pulmonary Function Tests","volume":"16","author":"Uffmann","year":"2001","journal-title":"J. Thorac. Imaging"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"655","DOI":"10.2214\/ajr.169.3.9275871","article-title":"Primary Sj\u00f6gren\u2019s syndrome and associated lung disease: CT findings in 50 patients","volume":"169","author":"Franquet","year":"1997","journal-title":"AJR Am. J. Roentgenol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1423","DOI":"10.1111\/1756-185X.13334","article-title":"Interstitial lung disease in non-sicca onset primary Sj\u00f6gren\u2019s syndrome: A large-scale case-control study","volume":"21","author":"Gao","year":"2018","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1016\/j.reuma.2015.09.003","article-title":"Sj\u00f6gren\u2019s syndrome-associated interstitial lung disease: A multicenter study","volume":"12","author":"Reina","year":"2016","journal-title":"Reumatol. Clin."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1586\/1744666X.2016.1109449","article-title":"Early diagnosis of primary Sj\u00f6gren\u2019s syndrome: EULAR-SS task force clinical recommendations","volume":"12","author":"Theander","year":"2016","journal-title":"Expert Rev. Clin. Immunol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"102447","DOI":"10.1016\/j.autrev.2019.102447","article-title":"Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sj\u00f6gren\u2019s syndrome: A systematic review from the Italian Society of Rheumatology","volume":"19","author":"Sambataro","year":"2020","journal-title":"Autoimmun. Rev."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"e003569","DOI":"10.1136\/bmjopen-2013-003569","article-title":"Primary Sjogren\u2019s syndrome 1976-2005 and associated interstitial lung disease: A population-based study of incidence and mortality","volume":"3","author":"Nannini","year":"2013","journal-title":"BMJ Open."},{"key":"ref_15","first-page":"291","article-title":"Interstitial lung disease in Sj\u00f6gren\u2019s syndrome: A clinical review","volume":"38","author":"Luppi","year":"2020","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1489","DOI":"10.1378\/chest.130.5.1489","article-title":"Interstitial Lung Disease in Primary Sj\u00f6gren Syndrome","volume":"130","author":"Parambil","year":"2006","journal-title":"Chest"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.3389\/fimmu.2019.01327","article-title":"Pulmonary Manifestations of Primary Sj\u00f6gren\u2019s Syndrome: Underlying Immunological Mechanisms, Clinical Presentation, and Management","volume":"10","author":"Gupta","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1111\/1756-185X.12583","article-title":"Clinical and laboratory profiles of primary Sjogren\u2019s syndrome in a Chinese population: A retrospective analysis of 315 patients","volume":"18","author":"Li","year":"2015","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1016\/j.chest.2020.10.011","article-title":"Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sj\u00f6gren\u2019s","volume":"159","author":"Lee","year":"2021","journal-title":"Chest"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1513\/AnnalsATS.201808-565CME","article-title":"Interstitial Pneumonia with Autoimmune Features","volume":"16","author":"Graney","year":"2019","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1183\/13993003.00150-2015","article-title":"An official European Respiratory Society\/American Thoracic Society research statement: Interstitial pneumonia with autoimmune features","volume":"46","author":"Fischer","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_22","first-page":"1140","article-title":"Progression and prognosis of interstitial pneumonia with autoimmune features: A longitudinal, prospective, multi-centre study","volume":"41","author":"Sambataro","year":"2023","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Sambataro, D., Sambataro, G., Pignataro, F., Zanframundo, G., Codullo, V., Fagone, E., Martorana, E., Ferro, F., Orlandi, M., and Del Papa, N. (2020). Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?. Diagnostics, 10.","DOI":"10.3390\/diagnostics10040208"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1007\/s12016-017-8639-y","article-title":"Cutaneous and Mucosal Manifestations of Sj\u00f6gren\u2019s Syndrome","volume":"53","author":"Generali","year":"2017","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.clim.2017.04.005","article-title":"Sjogren\u2019s syndrome: Clinical aspects","volume":"182","author":"Vivino","year":"2017","journal-title":"Clin. Immunol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1072","DOI":"10.1378\/chest.08-2839","article-title":"Minor Salivary Gland Biopsy To Detect Primary Sj\u00f6gren Syndrome in Patients With Interstitial Lung Disease","volume":"136","author":"Fischer","year":"2009","journal-title":"Chest"},{"key":"ref_27","first-page":"2344","article-title":"Glandular involvement in primary Sj\u00f6gren\u2019s syndrome patients with interstitial lung disease-onset and sicca-onset, a single centre cross-sectional study","volume":"40","author":"Ferro","year":"2022","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"2981","DOI":"10.1007\/s10067-018-4289-6","article-title":"A retrospective analysis of distinguishing features of chest HRCT and clinical manifestation in primary Sj\u00f6gren\u2019s syndrome-related interstitial lung disease in a Chinese population","volume":"37","author":"Dong","year":"2018","journal-title":"Clin. Rheumatol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"105895","DOI":"10.1016\/j.rmed.2020.105895","article-title":"Anti-Ro52 antibodies are a risk factor for interstitial lung disease in primary Sj\u00f6gren syndrome","volume":"163","author":"Buvry","year":"2020","journal-title":"Respir. Med."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.autrev.2016.09.017","article-title":"Interstitial lung disease in primary Sj\u00f6gren\u2019s syndrome","volume":"16","author":"Roca","year":"2017","journal-title":"Autoimmun. Rev."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"2108","DOI":"10.21037\/jtd.2018.03.120","article-title":"Risk factors for primary Sj\u00f6gren syndrome-associated interstitial lung disease","volume":"10","author":"Wang","year":"2018","journal-title":"J. Thorac. Dis."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"e11003","DOI":"10.1097\/MD.0000000000011003","article-title":"Prevalence, risk factors, and prognosis of interstitial lung disease in a large cohort of Chinese primary Sj\u00f6gren syndrome patients: A case-control study","volume":"97","author":"Gao","year":"2018","journal-title":"Medicine"},{"key":"ref_33","first-page":"2022","article-title":"Serology Driven Pulmonary Phenotype Characterization of Sj\u00f6gren Syndrome-associated Interstitial Lung Disease: A Monocentric Cohort Study","volume":"74","author":"Francesco","year":"2022","journal-title":"Arthritis Rheumatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1007\/s10067-020-05236-8","article-title":"Characteristics of primary Sj\u00f6gren\u2019s syndrome related lymphocytic interstitial pneumonia","volume":"40","author":"Dong","year":"2021","journal-title":"Clin. Rheumatol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1159\/000214841","article-title":"Pulmonary manifestations of Sj\u00f6gren\u2019s syndrome","volume":"78","author":"Shi","year":"2009","journal-title":"Respiration"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/j.rmed.2018.02.023","article-title":"Lymphocytic focus score is positively related to airway and interstitial lung diseases in primary Sj\u00f6gren\u2019s syndrome","volume":"137","author":"Kakugawa","year":"2018","journal-title":"Respir Med."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1055\/s-0034-1371527","article-title":"Biomarkers in Connective Tissue Disease-Associated Interstitial Lung Disease","volume":"35","author":"Bonella","year":"2014","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1007\/s10067-015-3167-8","article-title":"Association of serum KL-6 levels with interstitial lung disease in patients with connective tissue disease: A cross-sectional study","volume":"35","author":"Oguz","year":"2016","journal-title":"Clin. Rheumatol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1016\/j.pharmthera.2019.05.014","article-title":"Review: Serum biomarkers in idiopathic pulmonary fibrosis and systemic sclerosis associated interstitial lung disease\u2014Frontiers and horizons","volume":"202","author":"Jee","year":"2019","journal-title":"Pharmacol. Ther."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1186\/s13075-021-02586-9","article-title":"The role of lung ultrasound B-lines and serum KL-6 in the screening and follow-up of rheumatoid arthritis patients for an identification of interstitial lung disease: Review of the literature, proposal for a preliminary algorithm, and clinical application to cases","volume":"23","author":"Wang","year":"2021","journal-title":"Arthritis Res. Ther."},{"key":"ref_41","first-page":"58","article-title":"Serum KL-6 levels reflect the severity of interstitial lung disease associated with connective tissue disease","volume":"21","author":"Lee","year":"2019","journal-title":"Thromb. Haemost."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"105811","DOI":"10.1016\/j.rmed.2019.105811","article-title":"Prognostic factors for primary Sj\u00f6gren\u2019s syndrome-associated interstitial lung diseases","volume":"159","author":"Kamiya","year":"2019","journal-title":"Respir Med."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"2040622320944802","DOI":"10.1177\/2040622320944802","article-title":"Increases in tumor markers are associated with primary Sj\u00f6gren\u2019s syndrome-associated interstitial lung disease","volume":"11","author":"Shi","year":"2020","journal-title":"Ther. Adv. Chronic Dis."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1561","DOI":"10.1007\/s10067-019-04906-6","article-title":"Characteristics of patients with primary Sj\u00f6gren\u2019s syndrome associated interstitial lung disease and relevant features of disease progression","volume":"39","author":"Zhang","year":"2020","journal-title":"Clin. Rheumatol."},{"key":"ref_45","doi-asserted-by":"crossref","unstructured":"Enomoto, Y., Takemura, T., Hagiwara, E., Iwasawa, T., Fukuda, Y., Yanagawa, N., Sakai, F., Baba, T., Nagaoka, S., and Ogura, T. (2013). Prognostic Factors in Interstitial Lung Disease Associated with Primary Sj\u00f6gren\u2019s Syndrome: A Retrospective Analysis of 33 Pathologically\u2013Proven Cases. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0073774"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"632","DOI":"10.1164\/rccm.200403-417OC","article-title":"Pulmonary manifestations of primary Sjogren\u2019s syndrome: A clinical, radiologic, and pathologic study","volume":"171","author":"Ito","year":"2005","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1164\/rccm.200607-912OC","article-title":"Prognosis of fibrotic interstitial pneumonia: Idiopathic versus collagen vascular disease-related subtypes","volume":"175","author":"Park","year":"2007","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1016\/j.semarthrit.2019.04.005","article-title":"Impact of the pattern of interstitial lung disease on mortality in rheumatoid arthritis: A systematic literature review and meta-analysis","volume":"49","author":"Singh","year":"2019","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1093\/rheumatology\/keq410","article-title":"Comparison between cryptogenic organizing pneumonia and connective tissue disease-related organizing pneumonia","volume":"50","author":"Yoo","year":"2011","journal-title":"Rheumatology"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1183\/09031936.00158507","article-title":"Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia","volume":"33","author":"Park","year":"2009","journal-title":"Eur. Respir. J."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"684699","DOI":"10.3389\/fimmu.2021.684699","article-title":"Interstitial Lung Disease in Connective Tissue Disease: A Common Lesion With Heterogeneous Mechanisms and Treatment Considerations","volume":"12","author":"Shao","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"1968","DOI":"10.1056\/NEJMoa1113354","article-title":"Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis","volume":"366","author":"Raghu","year":"2012","journal-title":"N. Engl. J. Med."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"2193","DOI":"10.1007\/s12325-019-01036-y","article-title":"Transbronchial Lung Cryobiopsy in Idiopathic Pulmonary Fibrosis: A State of the Art Review","volume":"36","author":"Lodhi","year":"2019","journal-title":"Adv. Ther."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1159\/000444089","article-title":"Safety and Diagnostic Yield of Transbronchial Lung Cryobiopsy in Diffuse Parenchymal Lung Diseases: A Comparative Study versus Video-Assisted Thoracoscopic Lung Biopsy and a Systematic Review of the Literature","volume":"91","author":"Ravaglia","year":"2016","journal-title":"Respiration"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"1759720X211059605","DOI":"10.1177\/1759720X211059605","article-title":"Bronchoalveolar lavage and lung biopsy in connective tissue diseases, to do or not to do?","volume":"13","author":"Tomassetti","year":"2021","journal-title":"Ther. Adv. Musculoskelet. Dis."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/2110-5820-4-1","article-title":"Lung ultrasound in the critically ill","volume":"4","author":"Lichtenstein","year":"2014","journal-title":"Ann. Intensive Care"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1016\/j.echo.2005.05.019","article-title":"Ultrasound Lung Comets: A Clinically Useful Sign of Extravascular Lung Water","volume":"19","author":"Picano","year":"2006","journal-title":"J. Am. Soc. Echocardiogr."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"379","DOI":"10.11152\/mu-1573","article-title":"A common misunderstanding in lung ultrasound: The comet tail artefact","volume":"20","author":"Jenssen","year":"2018","journal-title":"Med. Ultrason."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1007\/s11547-019-01053-5","article-title":"Ultrasound as a potential tool for the assessment of interstitial lung disease in rheumatic patients. Where are we now?","volume":"124","author":"Gutierrez","year":"2019","journal-title":"La Radiol. Med."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"173","DOI":"10.7863\/jum.2003.22.2.173","article-title":"Transthoracic sonography of diffuse parenchymal lung disease: The role of comet tail artifacts","volume":"22","author":"Reissig","year":"2003","journal-title":"J. Ultrasound. Med."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1382","DOI":"10.1093\/rheumatology\/kep263","article-title":"Ultrasound lung comets in systemic sclerosis: A chest sonography hallmark of pulmonary interstitial fibrosis","volume":"48","author":"Gargani","year":"2009","journal-title":"Rheumatology"},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1007\/s11739-015-1329-y","article-title":"Lung ultrasound in systemic sclerosis: Correlation with high-resolution computed tomography, pulmonary function tests and clinical variables of disease","volume":"11","author":"Gigante","year":"2016","journal-title":"Intern. Emerg. Med."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1136\/annrheumdis-2011-201072","article-title":"Lung ultrasound for the screening of interstitial lung disease in very early systemic sclerosis","volume":"72","author":"Barskova","year":"2013","journal-title":"Ann. Rheum. Dis."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"R134","DOI":"10.1186\/ar3446","article-title":"Utility of a simplified ultrasound assessment to assess interstitial pulmonary fibrosis in connective tissue disorders\u2014Preliminary results","volume":"13","author":"Gutierrez","year":"2011","journal-title":"Arthritis Res. Ther."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1641","DOI":"10.3899\/jrheum.120104","article-title":"Ultrasound in the Assessment of Pulmonary Fibrosis in Connective Tissue Disorders: Correlation with High-Resolution Computed Tomography","volume":"39","author":"Tardella","year":"2012","journal-title":"J. Rheumatol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1007\/s11739-016-1582-8","article-title":"Assessment of interstitial lung disease in Sj\u00f6gren\u2019s syndrome by lung ultrasound: A pilot study of correlation with high-resolution chest tomography","volume":"12","author":"Vasco","year":"2017","journal-title":"Intern. Emerg. Med."},{"key":"ref_67","first-page":"1718","article-title":"AB0510 Lung Ultrasound of Pleural Irregularities in Sub-clinical Primary Sj\u00f6gren\u2019s Syndrome-Lung Involvement: A Single Centre Experience","volume":"78","author":"Ferro","year":"2019","journal-title":"Ann. Rheum. Dis."},{"key":"ref_68","first-page":"165","article-title":"The use of ultrasound for assessing interstitial lung involvement in connective tissue diseases","volume":"36","author":"Ferro","year":"2018","journal-title":"Ann. Rheum. Dis."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1007\/s10067-019-04744-6","article-title":"Survival analysis of patients with Sj\u00f6gren\u2019s syndrome in Turkey: A tertiary hospital-based study","volume":"39","author":"Yazisiz","year":"2020","journal-title":"Clin. Rheumatol."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"e26777","DOI":"10.1097\/MD.0000000000026777","article-title":"Characteristics and mortality in primary Sj\u00f6gren syndrome\u2013related interstitial lung disease","volume":"100","author":"Gao","year":"2021","journal-title":"Medicine"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1007\/s10067-019-04753-5","article-title":"Associated factors with interstitial lung disease and health-related quality of life in Chinese patients with primary Sj\u00f6gren\u2019s syndrome","volume":"39","author":"Zhao","year":"2020","journal-title":"Clin. Rheumatol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"1153","DOI":"10.1007\/s10067-021-05984-1","article-title":"Risk factors for progression of interstitial lung disease in Sj\u00f6gren\u2019s syndrome: A single-centered, retrospective study","volume":"41","author":"He","year":"2022","journal-title":"Clin. Rheumatol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1136\/annrheumdis-2019-216114","article-title":"EULAR recommendations for the management of Sj\u00f6gren\u2019s syndrome with topical and systemic therapies","volume":"79","author":"Bombardieri","year":"2020","journal-title":"Ann. Rheum. Dis."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"125","DOI":"10.2967\/jnumed.121.262944","article-title":"Perspective on Fibroblast Activation Protein-Specific PET\/CT in Fibrotic Interstitial Lung Diseases: Imaging Fibrosis-A New Paradigm for Molecular Imaging?","volume":"63","author":"Schmidkonz","year":"2022","journal-title":"J. Nucl. Med."},{"key":"ref_75","first-page":"91","article-title":"Pirfenidone for primary Sj\u00f6gren\u2019s syndrome-related fibrotic interstitial pneumonia","volume":"34","author":"Enomoto","year":"2017","journal-title":"Sarcoidosis Vasc. Diffus. Lung Dis."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"1718","DOI":"10.1056\/NEJMoa1908681","article-title":"Nintedanib in Progressive Fibrosing Interstitial Lung Diseases","volume":"381","author":"Flaherty","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"L487","DOI":"10.1152\/ajplung.00301.2018","article-title":"Profibrotic effect of IL-17A and elevated IL-17RA in idiopathic pulmonary fibrosis and rheumatoid arthritis-associated lung disease support a direct role for IL-17A\/IL-17RA in human fibrotic interstitial lung disease","volume":"316","author":"Zhang","year":"2019","journal-title":"Am. J. Physiol. Cell. Mol. Physiol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"1965935","DOI":"10.1155\/2018\/1965935","article-title":"The TGF-\u03b21 Signaling Pathway as an Attractive Target in the Fibrosis Pathogenesis of Sj\u00f6gren\u2019s Syndrome","volume":"2018","author":"Sisto","year":"2018","journal-title":"Mediat. Inflamm."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1111\/cei.13337","article-title":"Interleukin-17 and -22 synergy linking inflammation and EMT-dependent fibrosis in Sj\u00f6gren\u2019s syndrome","volume":"198","author":"Sisto","year":"2019","journal-title":"Clin. Exp. Immunol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"3003","DOI":"10.4049\/jimmunol.1004081","article-title":"Blocking IL-17A promotes the resolution of pulmonary inflammation and fibrosis via TGF-beta1-dependent and -independent mechanisms","volume":"187","author":"Mi","year":"2011","journal-title":"J. Immunol."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"1167","DOI":"10.1016\/j.autrev.2014.08.022","article-title":"Unmasking the pathogenic role of IL-17 axis in primary Sj\u00f6gren\u2019s syndrome: A new era for therapeutic targeting?","volume":"13","author":"Alunno","year":"2014","journal-title":"Autoimmun. Rev."},{"key":"ref_82","doi-asserted-by":"crossref","unstructured":"Rafael-Vidal, C., P\u00e9rez, N., Altab\u00e1s, I., Garcia, S., and Pego-Reigosa, J.M. (2020). Blocking IL-17: A Promising Strategy in the Treatment of Systemic Rheumatic Diseases. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21197100"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1016\/j.resinv.2014.02.003","article-title":"Features of usual interstitial pneumonia in patients with primary Sj\u00f6gren\u2019s syndrome compared with idiopathic pulmonary fibrosis","volume":"52","author":"Enomoto","year":"2014","journal-title":"Respir. Investig."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"e142","DOI":"10.1016\/S2665-9913(19)30135-3","article-title":"Assessment of the anti-CD40 antibody iscalimab in patients with primary Sj\u00f6gren\u2019s syndrome: A multicentre, randomised, double-blind, placebo-controlled, proof-of-concept study","volume":"2","author":"Fisher","year":"2020","journal-title":"Lancet Rheumatol."},{"key":"ref_85","first-page":"2211","article-title":"Sj\u00f6gren\u2019s syndrome: One year in review 2022","volume":"40","author":"Chatzis","year":"2022","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_86","doi-asserted-by":"crossref","unstructured":"Montero, P., Milara, J., Roger, I., and Cortijo, J. (2021). Role of JAK\/STAT in Interstitial Lung Diseases; Molecular and Cellular Mechanisms. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22126211"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"773352","DOI":"10.3389\/fimmu.2021.773352","article-title":"Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies","volume":"12","author":"Nombel","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"1407","DOI":"10.1016\/S2213-2600(21)00331-3","article-title":"Efficacy and safety of baricitinib for the treatment of hospitalised adults with COVID-19 [COV-BARRIER]: A randomised, double-blind, parallel-group, placebo-controlled phase 3 trial","volume":"9","author":"Marconi","year":"2021","journal-title":"Lancet Respir. Med."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1136\/annrheumdis-2022-eular.3231","article-title":"Pos1242 Baricitinib and Pulse Steroids Combined Treatment in Severe COVID-19 Pneumonia: Preliminary Data from a Rheumatologic Experience in Intensive Care Unit","volume":"81","author":"Ferro","year":"2022","journal-title":"Ann. Rheum. Dis."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"1158768","DOI":"10.3389\/fmed.2023.1158768","article-title":"Targeting intracellular pathways in idiopathic inflammatory myopathies: A narrative review","volume":"10","author":"Ferro","year":"2023","journal-title":"Front. Med."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"4797","DOI":"10.1093\/rheumatology\/keac167","article-title":"Safety and Efficacy of Filgotinib, Lanraplenib, and Tirabrutinib in Sj\u00f6gren\u2019s Syndrome: Randomised, Phase 2, Double-Blind, Placebo-Controlled Study","volume":"61","author":"Price","year":"2022","journal-title":"Rheumatology"},{"key":"ref_92","doi-asserted-by":"crossref","unstructured":"Zhang, N., Liu, S., Zhang, Z., Liu, Y., Mi, L., and Xu, K. (2023). Lung transplantation: A viable option for connective tissue disease. Arthritis Care Res.","DOI":"10.1002\/acr.25133"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1007\/s11926-017-0650-z","article-title":"Lung Transplantation in Patients With Systemic Sclerosis","volume":"19","author":"Shah","year":"2017","journal-title":"Curr. Rheumatol. Rep."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1016\/j.healun.2021.01.1391","article-title":"Risk of primary graft dysfunction following lung transplantation in selected adults with connective tissue disease-associated interstitial lung disease","volume":"40","author":"Natalini","year":"2021","journal-title":"J. Heart Lung Transplant."},{"key":"ref_95","first-page":"1666","article-title":"Outcomes after lung transplantation among Chinese patients with connective tissue disease-associated interstitial lung disease and pulmonary hypertension: A retrospective cohort study","volume":"40","author":"Ju","year":"2022","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1016\/j.healun.2016.12.013","article-title":"Survival and outcomes after lung transplantation for non-scleroderma connective tissue\u2013related interstitial lung disease","volume":"36","author":"Courtwright","year":"2017","journal-title":"J. Heart Lung Transplant."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"888","DOI":"10.21037\/atm-20-3516","article-title":"Double lung transplantation for Sj\u00f6gren\u2019s syndrome-related interstitial lung disease: A case report and review of literature","volume":"8","author":"Cao","year":"2020","journal-title":"Ann. Transl. Med."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/12\/10\/3428\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T19:33:40Z","timestamp":1760124820000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/12\/10\/3428"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,12]]},"references-count":97,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2023,5]]}},"alternative-id":["jcm12103428"],"URL":"https:\/\/doi.org\/10.3390\/jcm12103428","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,5,12]]}}}