{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T02:31:27Z","timestamp":1760149887437,"version":"build-2065373602"},"reference-count":115,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2023,10,5]],"date-time":"2023-10-05T00:00:00Z","timestamp":1696464000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Sarcoidosis has been a well-recognised risk factor for pulmonary hypertension (PH) for a long time, but still, the knowledge about this concatenation is incomplete. Sarcoidosis-associated PH (SAPH) is an uncommon but serious complication associated with increased morbidity and mortality among sarcoidosis patients. The real epidemiology of SAPH remains unknown, and its pathomechanisms are not fully explained. Sarcoidosis is a heterogeneous and dynamic condition, and SAPH pathogenesis is believed to be multifactorial. The main roles in SAPH development play: parenchymal lung disease with the destruction of pulmonary vessels, the extrinsic compression of pulmonary vessels by conglomerate masses, lymphadenopathy or fibrosing mediastinitis, pulmonary vasculopathy, LV dysfunction, and portal hypertension. Recently, it has been recommended to individually tailor SAPH management according to the predominant pathomechanism, i.e., SAPH phenotype. Unfortunately, SAPH phenotyping is not a straightforward process. First, there are gaps in our understanding of undergoing processes. Second, the assessment of such a pivotal element as pulmonary vasculature on a microscopic level is non-feasible in SAPH patients antemortem. Finally, SAPH is a dynamic condition, multiple phenotypes usually coexist, and patients can switch between phenotypes during the course of sarcoidosis. In this article, we summarise the basic knowledge of SAPH, describe SAPH phenotypes, and highlight some practical problems related to SAPH phenotyping.<\/jats:p>","DOI":"10.3390\/diagnostics13193132","type":"journal-article","created":{"date-parts":[[2023,10,5]],"date-time":"2023-10-05T09:14:22Z","timestamp":1696497262000},"page":"3132","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Phenotypes of Sarcoidosis-Associated Pulmonary Hypertension\u2014A Challenging Mystery"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3808-7047","authenticated-orcid":false,"given":"Aneta","family":"Kacprzak","sequence":"first","affiliation":[{"name":"1st Department of Lung Diseases, National Tuberculosis and Lung Diseases Institute, Plocka 26, 01-138 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Witold","family":"Tomkowski","sequence":"additional","affiliation":[{"name":"1st Department of Lung Diseases, National Tuberculosis and Lung Diseases Institute, Plocka 26, 01-138 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Monika","family":"Szturmowicz","sequence":"additional","affiliation":[{"name":"1st Department of Lung Diseases, National Tuberculosis and Lung Diseases Institute, Plocka 26, 01-138 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,10,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2200879","DOI":"10.1183\/13993003.00879-2022","article-title":"2022 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"61","author":"Humbert","year":"2023","journal-title":"Eur. Respir. J."},{"key":"ref_2","unstructured":"World Health Organization (1961). Chronic cor pulmonale. Report of an expert committee. World Health Organ. Tech. Rep. Ser., 213, 35."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1483","DOI":"10.1378\/chest.128.3.1483","article-title":"Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension","volume":"128","author":"Sulica","year":"2005","journal-title":"Chest"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1136\/thx.2005.042838","article-title":"Pulmonary hypertension associated with sarcoidosis: Mechanisms, haemodynamics and prognosis","volume":"61","author":"Nunes","year":"2006","journal-title":"Thorax"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/j.rmed.2018.04.015","article-title":"Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry","volume":"139","author":"Baughman","year":"2018","journal-title":"Respir. Med."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"4061","DOI":"10.1038\/s41598-019-40030-w","article-title":"Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary Hypertension","volume":"9","author":"Parikh","year":"2019","journal-title":"Sci. Rep."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1502","DOI":"10.1016\/j.hlc.2021.03.279","article-title":"Clinical Phenotypes of Sarcoidosis-Associated Pulmonary Hypertension","volume":"30","author":"Mathijssen","year":"2021","journal-title":"Heart Lung Circ."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1700465","DOI":"10.1183\/13993003.00465-2017","article-title":"Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension","volume":"50","author":"Boucly","year":"2017","journal-title":"Eur. Respir. J."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1900897","DOI":"10.1183\/13993003.00897-2019","article-title":"Prevalence of pulmonary hypertension in pulmonary sarcoidosis: The first large European prospective study","volume":"54","author":"Huitema","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"210165","DOI":"10.1183\/16000617.0165-2021","article-title":"WASOG statement on the diagnosis and management of sarcoidosis-associated pulmonary hypertension","volume":"31","author":"Savale","year":"2022","journal-title":"Eur. Respir. Rev."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2153","DOI":"10.1056\/NEJMra071714","article-title":"Sarcoidosis","volume":"357","author":"Iannuzzi","year":"2007","journal-title":"N. Engl. J. Med."},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Bernardinello, N., Petrarulo, S., Balestro, E., Cocconcelli, E., Veltkamp, M., and Spagnolo, P. (2021). Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis. Diagnostics, 11.","DOI":"10.3390\/diagnostics11091558"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.clim.2018.12.012","article-title":"The long-term prognostic significance of sarcoidosis-associated pulmonary hypertension\u2014A cohort study","volume":"199","author":"Tiosano","year":"2019","journal-title":"Clin. Immunol."},{"key":"ref_14","first-page":"e25520","article-title":"Association Between Sarcoidosis and Risk of Venous Thromboembolism: A Retrospective Chart Review","volume":"14","author":"Kassem","year":"2022","journal-title":"Cureus"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1016\/j.chest.2016.09.009","article-title":"Association of Sarcoidosis with Increased Risk of VTE: A Population-Based Study, 1976 to 2013","volume":"151","author":"Ungprasert","year":"2017","journal-title":"Chest"},{"key":"ref_16","first-page":"169","article-title":"Obstructive sleep apnea in sarcoidosis and impact of cpap treatment on fatigue","volume":"37","author":"Mari","year":"2020","journal-title":"Sarcoidosis Vasc. Diffus. Lung Dis."},{"key":"ref_17","first-page":"1052","article-title":"Geoepidemiological big data approach to sarcoidosis: Geographical and ethnic determinants","volume":"37","author":"Kostov","year":"2019","journal-title":"Clin. Exp. Rrheumatol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"809594","DOI":"10.3389\/fcvm.2021.809594","article-title":"Prevalence of Sarcoidosis-Associated Pulmonary Hypertension: A Systematic Review and Meta-Analysis","volume":"8","author":"Zhang","year":"2022","journal-title":"Front. Cardiovasc. Med."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1901747","DOI":"10.1183\/13993003.01747-2019","article-title":"Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: Results from an international registry","volume":"55","author":"Shlobin","year":"2020","journal-title":"Eur. Respir. J."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1016\/S2213-2600(20)30317-9","article-title":"Pulmonary vascular resistance and clinical outcomes in patients with pulmonary hypertension: A retrospective cohort study","volume":"8","author":"Maron","year":"2020","journal-title":"Lancet Respir. Med."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1159\/000194667","article-title":"Pulmonary haemodynamics at rest and during exercise in patients with sarcoidosis","volume":"46","author":"Zych","year":"1984","journal-title":"Respiration"},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Groen, K., Huitema, M.P., van der Vis, J.J., Post, M.C., Grutters, J.C., Baughman, R.P., and van Moorsel, C.H.M. (2022). Pulmonary Hypertension Associated Genetic Variants in Sarcoidosis Associated Pulmonary Hypertension. Diagnostics, 12.","DOI":"10.3390\/diagnostics12102564"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.chest.2017.07.008","article-title":"Predictors of mortality in pulmonary sarcoidosis","volume":"153","author":"Kirkil","year":"2018","journal-title":"Chest"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1078","DOI":"10.1378\/chest.09-2002","article-title":"Survival in sarcoidosis-associated pulmonary hypertension: The importance of hemodynamic evaluation","volume":"138","author":"Baughman","year":"2010","journal-title":"Chest"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"106762","DOI":"10.1016\/j.rmed.2022.106762","article-title":"Four-year survival rate in pulmonary sarcoidosis with extensive pulmonary hypertension screening","volume":"195","author":"Huitema","year":"2022","journal-title":"Respir. Med."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"105997","DOI":"10.1016\/j.rmed.2020.105997","article-title":"Predictors of mortality in fibrosing pulmonary sarcoidosis","volume":"169","author":"Jeny","year":"2020","journal-title":"Respir. Med."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1913","DOI":"10.1016\/S0735-1097(23)02357-4","article-title":"Mortality and Morbidity of Pulmonary Hypertension amongst Patients with Sarcoidosis\u2014A Nationwide Study","volume":"81","author":"Malik","year":"2023","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1186\/s12931-022-02094-7","article-title":"Characterization of the PF-ILD phenotype in patients with advanced pulmonary sarcoidosis","volume":"23","author":"Schimmelpennink","year":"2022","journal-title":"Respir. Res."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.1378\/chest.14-2546","article-title":"Prostacyclin and oral vasodilator therapy in sarcoidosis-associated pulmonary hypertension: A retrospective case series","volume":"148","author":"Bonham","year":"2015","journal-title":"Chest"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1016\/j.amjcard.2012.09.031","article-title":"Clinical characteristics, haemodynamics and treatment of pulmonary hypertension in sarcoidosis in a single centre, and meta-analysis of the published data","volume":"111","author":"Dobarro","year":"2013","journal-title":"Am. J. Cardiol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1136\/thoraxjnl-2019-214348","article-title":"BTS Clinical Statement on pulmonary sarcoidosis","volume":"76","author":"Thillai","year":"2021","journal-title":"Thorax"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1097\/MCP.0000000000000893","article-title":"Novel insights in fibrotic pulmonary sarcoidosis","volume":"28","author":"Comes","year":"2022","journal-title":"Curr. Opin. Pulm. Med."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1034\/j.1399-3003.1999.14d02.x","article-title":"ATS\/ERS\/WASOG statement on sarcoidosis. Sarcoidosis Statement Committee. American Thoracic Society. European Respiratory Society. World Association for Sarcoidosis and Other Granulomatous Disorders","volume":"14","author":"Costabel","year":"1999","journal-title":"Eur. Respir. J."},{"key":"ref_34","first-page":"A3289","article-title":"Prevalence and Epidemiology of Pulmonary Hypertension Amongst Patients with Sarcoidosis","volume":"207","author":"Shah","year":"2023","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1183\/09031936.05.00083404","article-title":"Pulmonary hypertension in advanced sarcoidosis: Epidemiology and clinical characteristics","volume":"25","author":"Shorr","year":"2005","journal-title":"Eur. Respir. J."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"D109","DOI":"10.1016\/j.jacc.2013.10.036","article-title":"Pulmonary hypertension in chronic lung diseases","volume":"62","author":"Seeger","year":"2013","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1801916","DOI":"10.1183\/13993003.01914-2018","article-title":"Pulmonary hypertension in chronic lung disease and hypoxia","volume":"53","author":"Nathan","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"e013310","DOI":"10.1161\/JAHA.119.013310","article-title":"Hypoxic Pulmonary Vasoconstriction and the Diffusing Capacity in Pulmonary Hypertension Secondary to Idiopathic Pulmonary Fibrosis","volume":"8","author":"Sakao","year":"2019","journal-title":"J. Am. Heart Assoc."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"1178","DOI":"10.1096\/fj.04-3261fje","article-title":"Initial apoptosis is followed by increased proliferation of apoptosis-resistant endothelial cells","volume":"19","author":"Sakao","year":"2005","journal-title":"FASEB J."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1186\/s12931-020-01570-2","article-title":"Pulmonary hypertension secondary to pulmonary fibrosis: Clinical data, histopathology and molecular insights","volume":"21","author":"Ruffenach","year":"2020","journal-title":"Respir. Res."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"2045894020956592","DOI":"10.1177\/2045894020956592","article-title":"Revisiting the mechanism of hypoxic pulmonary vasoconstriction using isolated perfused\/ventilated mouse lung","volume":"10","author":"Jain","year":"2020","journal-title":"Pulm. Circ."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1801887","DOI":"10.1183\/13993003.01887-2018","article-title":"Pathology and pathobiology of pulmonary hypertension: State of the art and research perspectives","volume":"53","author":"Humbert","year":"2018","journal-title":"Eur. Respir. J."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"00571","DOI":"10.1183\/23120541.00571-2021","article-title":"Vascular remodelling in idiopathic pulmonary fibrosis patients and its detrimental effect on lung physiology: Potential role of endothelial-to-mesenchymal transition","volume":"8","author":"Gaikwad","year":"2022","journal-title":"ERJ Open Res."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"e000532","DOI":"10.1136\/bmjresp-2019-000532","article-title":"Pulmonary vasculopathy in explanted lungs from patients with interstitial lung disease undergoing lung transplantation","volume":"7","author":"Dotan","year":"2020","journal-title":"BMJ Open Respir. Res."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1016\/j.resinv.2016.05.005","article-title":"An autopsy case of refractory pulmonary hypertension with sarcoidosis","volume":"54","author":"Ota","year":"2016","journal-title":"Respir. Investig."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1016\/j.jcmg.2021.05.023","article-title":"Pulmonary Artery 18F-Fluorodeoxyglucose Uptake by PET\/CMR as a Marker of Pulmonary Hypertension in Sarcoidosis","volume":"15","author":"Maier","year":"2022","journal-title":"JACC Cardiovasc. Imaging"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.jcmg.2021.08.014","article-title":"Pulmonary Arterial 18F-FDG Uptake in Sarcoidosis: A Novel Biosignal for Subclinical Pulmonary Hypertension","volume":"15","author":"Ovadia","year":"2022","journal-title":"JACC Cardiovasc. Imaging"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1378\/chest.122.1.233","article-title":"Outcomes for patients with sarcoidosis awaiting lung transplantation","volume":"122","author":"Shorr","year":"2002","journal-title":"Chest"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"00124","DOI":"10.1183\/23120541.00124-2017","article-title":"A stepwise composite echocardiographic score predicts severe pulmonary hypertension in patients with interstitial lung disease","volume":"4","author":"Bax","year":"2018","journal-title":"ERJ Open Res."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"e01065","DOI":"10.1002\/rcr2.1065","article-title":"Pulmonary sarcoidosis with a cavitary lesion in the lung caused by a TNF-\u03b1 inhibitor: A case report","volume":"10","author":"Hamada","year":"2022","journal-title":"Respirol. Case Rep."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"2004079","DOI":"10.1183\/13993003.04079-2020","article-title":"ERS clinical practice guidelines on treatment of sarcoidosis","volume":"58","author":"Baughman","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"190146","DOI":"10.1183\/16000617.0146-2019","article-title":"Delphi consensus recommendations for a treatment algorithm in pulmonary sarcoidosis","volume":"29","author":"Rahaghi","year":"2020","journal-title":"Eur. Rrespir. Rev."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1016\/j.chest.2021.07.2162","article-title":"Riociguat for Sarcoidosis-Associated Pulmonary Hypertension: Results of a 1-Year Double-Blind, Placebo-Controlled Trial","volume":"161","author":"Baughman","year":"2022","journal-title":"Chest"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"e7595","DOI":"10.1097\/MD.0000000000007595","article-title":"Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis: Cohort study of a 40-year clinical experience at a tertiary referral center in Barcelona, Spain","volume":"96","author":"Villalba","year":"2017","journal-title":"Medicine"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1378\/chest.15-0615","article-title":"Histopathology of Explanted Lungs from Patients with a Diagnosis of Pulmonary Sarcoidosis","volume":"149","author":"Zhang","year":"2016","journal-title":"Chest"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1097\/PAS.0b013e3182785a2d","article-title":"End-stage sarcoid lung disease is distinct from usual interstitial pneumonia","volume":"37","author":"Xu","year":"2013","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_57","first-page":"44","article-title":"Volumetric FDG PET analysis of global lung inflammation: New tool for precision medicine in pulmonary sarcoidosis?","volume":"35","author":"Adams","year":"2018","journal-title":"Sarcoidosis Vasc. Diffus. Lung Dis."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1186\/s13550-019-0505-x","article-title":"Quantification of pulmonary disease activity in sarcoidosis measured with 18F-FDG PET\/CT: SUVmax versus total lung glycolysis","volume":"9","author":"Schimmelpennink","year":"2019","journal-title":"EJNMMI Res."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/j.rmed.2012.11.011","article-title":"Severity of pulmonary involvement and (18)F-FDG PET activity in sarcoidosis","volume":"107","author":"Mostard","year":"2013","journal-title":"Respir. Med."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"750","DOI":"10.1183\/09031936.00025212","article-title":"Imaging of sarcoidosis of the airways and lung parenchyma and correlation with lung function","volume":"40","author":"Nunes","year":"2012","journal-title":"Eur. Respir. J."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1055\/s-0040-1712534","article-title":"Radiographic and Histopathologic Features in Sarcoidosis: A Pictorial Display","volume":"41","author":"Shaikh","year":"2020","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"595077","DOI":"10.3389\/fmed.2021.595077","article-title":"Unsupervised Clustering Reveals Sarcoidosis Phenotypes Marked by a Reduction in Lymphocytes Relate to Increased Inflammatory Activity on 18FDG-PET\/CT","volume":"8","author":"Vagts","year":"2021","journal-title":"Front. Med."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1515\/jomb-2016-0017","article-title":"Verifying Sarcoidosis Activity: Chitotriosidase versus ACE in Sarcoidosis\u2014A Case-control Study","volume":"35","year":"2016","journal-title":"J. Med. Biochem."},{"key":"ref_64","doi-asserted-by":"crossref","unstructured":"Le\u0161tan Ramov\u0161, Z., Sodin-\u0160emrl, S., Lakota, K., \u010cu\u010dnik, S., Manevski, D., Zba\u010dnik, R., Zupan\u010di\u010d, M., Verbi\u010d, M., and Ter\u010delj, M. (2023). Correlation of the High-Resolution Computed Tomography Patterns of Intrathoracic Sarcoidosis with Serum Levels of SAA, CA 15.3, SP-D, and Other Biomarkers of Interstitial Lung Disease. Int. J. Mol. Sci., 24.","DOI":"10.3390\/ijms241310794"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1443","DOI":"10.3389\/fimmu.2020.01443","article-title":"Biomarkers in the Diagnosis and Prognosis of Sarcoidosis: Current Use and Future Prospects","volume":"11","author":"Kraaijvanger","year":"2020","journal-title":"Front. Immunol."},{"key":"ref_66","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_67","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/S2213-2600(20)30036-9","article-title":"Nintedanib in patients with progressive fibrosing interstitial lung diseases-subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: A randomised, double-blind, placebo-controlled, parallel-group trial","volume":"8","author":"Wells","year":"2020","journal-title":"Lancet Respir. Med."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"1040","DOI":"10.1513\/AnnalsATS.202103-343OC","article-title":"Nintedanib in Progressive Pulmonary Fibrosis: A Systematic Review and Meta-Analysis","volume":"19","author":"Ghazipura","year":"2022","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1016\/S2213-2600(20)30554-3","article-title":"Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): A double-blind, randomised, placebo-controlled, phase 2b trial","volume":"9","author":"Behr","year":"2021","journal-title":"Lancet Respir. Med."},{"key":"ref_70","first-page":"e2022011","article-title":"Value of pulmonary function testing identifying progressive pulmonary disease in fibrotic sarcoidosis: Results of a prospective feasibility study","volume":"39","author":"Baughman","year":"2022","journal-title":"Sarcoidosis Vasc. Diffus. Lung Dis."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"2102571","DOI":"10.1183\/13993003.02571-2021","article-title":"Prevalence and characteristics of progressive fibrosing interstitial lung disease in a prospective registry","volume":"60","author":"Hambly","year":"2022","journal-title":"Eur. Respir. J."},{"key":"ref_72","doi-asserted-by":"crossref","unstructured":"Nashatyreva, M.S., Trofimenko, I.N., Chernyak, B.A., and Avdeev, S.N. (2023). Pulmonary Fibrosis and Progressive Pulmonary Fibrosis in a Prospective Registry of Interstitial Lung Diseases in Eastern Siberia. Life, 13.","DOI":"10.3390\/life13010212"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"e002667","DOI":"10.1136\/rmdopen-2022-002667","article-title":"Real-world impact of antifibrotics on prognosis in patients with progressive fibrosing interstitial lung disease","volume":"9","author":"Niitsu","year":"2023","journal-title":"RMD Open"},{"key":"ref_74","doi-asserted-by":"crossref","unstructured":"Bergantini, L., Nardelli, G., d\u2019Alessandro, M., Montuori, G., Piccioli, C., Rosi, E., Gangi, S., Cavallaro, D., Cameli, P., and Bargagli, E. (2022). Combined Sarcoidosis and Idiopathic Pulmonary Fibrosis (CSIPF): A New Phenotype or a Fortuitous Overlap? Scoping Review and Case Series. J. Clin. Med., 11.","DOI":"10.3390\/jcm11072065"},{"key":"ref_75","doi-asserted-by":"crossref","unstructured":"Kamp, J.C., Neubert, L., Stark, H., Hinrichs, J.B., Boekhoff, C., Seidel, A.D., Ius, F., Haverich, A., Gottlieb, J., and Welte, T. (2022). Comparative Analysis of Gene Expression in Fibroblastic Foci in Patients with Idiopathic Pulmonary Fibrosis and Pulmonary Sarcoidosis. Cells, 11.","DOI":"10.3390\/cells11040664"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1016\/j.chest.2020.02.016","article-title":"A Randomized, Double-Blind, Placebo-Controlled Study of Pulsed, Inhaled Nitric Oxide in Subjects at Risk of Pulmonary Hypertension Associated with Pulmonary Fibrosis","volume":"158","author":"Nathan","year":"2020","journal-title":"Chest"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1056\/NEJMoa2008470","article-title":"Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease","volume":"384","author":"Waxman","year":"2021","journal-title":"N. Engl. J. Med."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"1699","DOI":"10.1378\/chest.12-1594","article-title":"Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction","volume":"143","author":"Han","year":"2013","journal-title":"Chest"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"258","DOI":"10.5603\/ARM.a2017.0043","article-title":"Sarcoidosis-associated pulmonary hypertension treated with sildenafil","volume":"85","author":"Kacprzak","year":"2017","journal-title":"Adv. Respir. Med."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1016\/j.healun.2007.11.576","article-title":"Pulmonary hypertension in end-stage pulmonary sarcoidosis: Therapeutic effect of sildenafil?","volume":"27","author":"Milman","year":"2008","journal-title":"J. Heart Lung Transplant."},{"key":"ref_81","first-page":"82","article-title":"Treatment of sarcoidosis-associated pulmonary hypertension: A single centre retrospective experience using targeted therapies","volume":"31","author":"Keir","year":"2014","journal-title":"Sarcoidosis Vasc. Diffus. Lung Dis."},{"key":"ref_82","first-page":"74","article-title":"Safety of macitentan in sarcoidosis-associated pulmonary hypertension: A case-series","volume":"37","author":"Mathijssen","year":"2020","journal-title":"Sarcoidosis Vasc. Diffus. Lung Dis."},{"key":"ref_83","first-page":"234","article-title":"Treatment of newly diagnosed sarcoid-associated pulmonary hypertension with ambrisentan and tadalafil combination therapy","volume":"37","author":"Abston","year":"2020","journal-title":"Sarcoidosis Vasc. Diffus. Lung Dis."},{"key":"ref_84","first-page":"184","article-title":"Long-term outcomes of epoprostenol therapy in sarcoid associated pulmonary hypertension","volume":"37","author":"Abston","year":"2020","journal-title":"Sarcoidosis Vasc. Diffus. Lung Dis."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"1481","DOI":"10.1378\/chest.130.5.1481","article-title":"Sarcoidosis-associated pulmonary hypertension: Outcome with long-term epoprostenol treatment","volume":"130","author":"Fisher","year":"2006","journal-title":"Chest"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"1455","DOI":"10.1378\/chest.08-1881","article-title":"Treatment of sarcoidosis-associated pulmonary hypertension. A two-center experience","volume":"135","author":"Barnett","year":"2009","journal-title":"Chest"},{"key":"ref_87","first-page":"110","article-title":"Inhaled iloprost for sarcoidosis associated pulmonary hypertension","volume":"26","author":"Baughman","year":"2009","journal-title":"Sarcoidosis Vasc. Diffus. Lung Dis."},{"key":"ref_88","first-page":"139","article-title":"Ambrisentan for sarcoidosis associated pulmonary hypertension","volume":"28","author":"Judson","year":"2011","journal-title":"Sarcoidosis Vasc. Diffus. Lung Dis."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1086\/688775","article-title":"Tadalafil therapy for sarcoidosis-associated pulmonary hypertension","volume":"6","author":"Ford","year":"2016","journal-title":"Pulm. Circ."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1378\/chest.13-1766","article-title":"Bosentan for sarcoidosis-associated pulmonary hypertension: A double-blind placebo controlled randomized trial","volume":"145","author":"Baughman","year":"2014","journal-title":"Chest"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"3881","DOI":"10.1007\/s12325-022-02229-8","article-title":"The Antifibrotic Effects of Inhaled Treprostinil: An Emerging Option for ILD","volume":"39","author":"Kolb","year":"2022","journal-title":"Adv. Ther."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"1266","DOI":"10.1016\/S2213-2600(21)00165-X","article-title":"Inhaled treprostinil and forced vital capacity in patients with interstitial lung disease and associated pulmonary hypertension: A post-hoc analysis of the INCREASE study","volume":"9","author":"Nathan","year":"2021","journal-title":"Lancet Respir. Med."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"e12245","DOI":"10.1002\/pul2.12245","article-title":"Pulmonary vasodilator therapy in sarcoidosis-associated pulmonary hypertension may decrease lung function decline and mortality","volume":"5","author":"Gayen","year":"2023","journal-title":"Pulm. Circ."},{"key":"ref_94","first-page":"w14677","article-title":"Bosentan for patients with steroid-resistant pulmonary sarcoidosis: A randomised controlled trial","volume":"148","author":"Hostettler","year":"2018","journal-title":"Swiss. Med. Wkly."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1016\/S0022-5223(19)42725-6","article-title":"Granulomatous and fibrous mediastinitis. A review and analysis of 180 cases","volume":"57","author":"Schowengerdt","year":"1969","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"e1800","DOI":"10.1097\/MD.0000000000001800","article-title":"Pulmonary Hypertension Complicating Fibrosing Mediastinitis","volume":"94","author":"Seferian","year":"2015","journal-title":"Medicine"},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"100616","DOI":"10.1016\/j.cpcardiol.2020.100616","article-title":"Characteristics and Outcomes of Pulmonary Angioplasty With or Without Stenting for Sarcoidosis-Associated Pulmonary Hypertension: Systematic Review and Individual Participant Data Meta-Analysis","volume":"46","author":"Condado","year":"2021","journal-title":"Curr. Probl. Cardiol."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1111\/crj.12435","article-title":"Interventional therapy in sarcoidosis-associated pulmonary arterial stenosis and pulmonary hypertension","volume":"11","author":"Liu","year":"2015","journal-title":"Clin. Respir. J."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"2775","DOI":"10.2169\/internalmedicine.51.7693","article-title":"Sarcoidosis complicated with major pulmonary artery obstruction and stenosis","volume":"51","author":"Hasegawa","year":"2012","journal-title":"Intern. Med."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"1216","DOI":"10.1016\/0046-8177(92)90288-E","article-title":"Pulmonary vascular involvement in sarcoidosis: A report of 40 autopsy cases","volume":"23","author":"Takemura","year":"1992","journal-title":"Hum. Pathol."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1007\/BF01600167","article-title":"Pulmonary vascular involvement in sarcoidosis: Granulomatous angiitis and microangiopathy in transbronchial lung biopsies","volume":"418","author":"Takemura","year":"1991","journal-title":"Virchows Arch. A Pathol. Anat. Histopathol."},{"key":"ref_102","first-page":"170","article-title":"Granulomatous pulmonary angiitis in sarcoidosis","volume":"101","author":"Rosen","year":"1977","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"480","DOI":"10.1111\/pin.13104","article-title":"Pulmonary infarction caused by sarcoidosis vascular involvement: A case report","volume":"71","author":"Tasaki","year":"2021","journal-title":"Pathol. Int."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"00549","DOI":"10.1183\/23120541.00549-2022","article-title":"Intimal granulomatous angiitis in sarcoid pulmonary vasculitis: Worth remembering","volume":"9","author":"Bernaudin","year":"2023","journal-title":"ERJ Open Res."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1183\/09031936.00044609","article-title":"Sarcoidosis-related pulmonary veno-occlusive disease presenting with recurrent haemoptysis","volume":"34","author":"Jones","year":"2009","journal-title":"Eur. Respir. J."},{"key":"ref_106","first-page":"809","article-title":"Sarcoidosis simulating pulmonary veno-occlusive disease","volume":"134","author":"Hoffstein","year":"1986","journal-title":"Am. Rev. Respir. Dis."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"234","DOI":"10.3390\/hearts2020019","article-title":"Cardiac Sarcoidosis: Pathophysiology, Diagnosis, and Management","volume":"2","author":"Kayali","year":"2021","journal-title":"Hearts"},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"1969","DOI":"10.1161\/CIRCULATIONAHA.109.851352","article-title":"Detection of myocardial damage in patients with sarcoidosis","volume":"120","author":"Patel","year":"2009","journal-title":"Circulation"},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1016\/j.acvd.2016.12.014","article-title":"Cardiac sarcoidosis: Diagnosis, therapeutic management and prognostic factors","volume":"110","author":"Sene","year":"2017","journal-title":"Arch. Cardiovasc. Dis."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"232","DOI":"10.3389\/fmed.2019.00232","article-title":"Hepatic Sarcoidosis: Natural History and Management Implications","volume":"6","author":"Sedki","year":"2019","journal-title":"Front. Med."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"732","DOI":"10.1111\/j.1440-1746.2003.03335.x","article-title":"Clinical manifestations of sarcoid liver disease","volume":"19","author":"Blich","year":"2004","journal-title":"J. Gastroenterol. Hepatol."},{"key":"ref_112","doi-asserted-by":"crossref","unstructured":"Calender, A., Weichhart, T., Valeyre, D., and Pacheco, Y. (2020). Current Insights in Genetics of Sarcoidosis: Functional and Clinical Impacts. J. Clin. Med., 9.","DOI":"10.3390\/jcm9082633"},{"key":"ref_113","doi-asserted-by":"crossref","unstructured":"Cocconcelli, E., Bernardinello, N., Castelli, G., Petrarulo, S., Bellani, S., Saetta, M., Spagnolo, P., and Balestro, E. (2023). Molecular Mechanism in the Development of Pulmonary Fibrosis in Patients with Sarcoidosis. Int. J. Mol. Sci., 24.","DOI":"10.20944\/preprints202305.1855.v1"},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1007\/s00408-020-00346-1","article-title":"Can a New Scoring System Improve Prediction of Pulmonary Hypertension in Newly Recognised Interstitial Lung Diseases?","volume":"198","author":"Sobiecka","year":"2020","journal-title":"Lung"},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"e26","DOI":"10.1164\/rccm.202002-0251ST","article-title":"Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline","volume":"201","author":"Crouser","year":"2020","journal-title":"Am. J. Respir. Crit. Care Med."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/13\/19\/3132\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T21:01:34Z","timestamp":1760130094000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/13\/19\/3132"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,5]]},"references-count":115,"journal-issue":{"issue":"19","published-online":{"date-parts":[[2023,10]]}},"alternative-id":["diagnostics13193132"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics13193132","relation":{},"ISSN":["2075-4418"],"issn-type":[{"type":"electronic","value":"2075-4418"}],"subject":[],"published":{"date-parts":[[2023,10,5]]}}}