{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T18:32:44Z","timestamp":1792089164301,"version":"4.4.0"},"reference-count":35,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2025,6,13]],"date-time":"2025-06-13T00:00:00Z","timestamp":1749772800000},"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>The most serious complications of systemic sclerosis (SSc) and mixed connective tissue disease (MCTD) include lung fibrosis (LF) and pulmonary hypertension (PH). The aim of this study was to find any association between the serological profile and the incidence of these complications. The tested group included 121 persons (87 SSc, 34 MCTD); mean age 55.6 \u00b1 13.4 years. Patients were qualified for the LF presence group based on HRCT. Likelihood of PH was determined using echocardiography. The presence of antinuclear antibodies (ANA) was assessed using indirect immunofluorescence, ANA-profile, sclerosis-profile (using EUROIMMUN kits), and antiphospholipid antibodies (aPL) (using the ELISA method). Distribution of individual antibody types was at a level similar to the previously described groups in the Polish population and differed from the American and African population. A positive correlation was found between LF and the presence of anti-Scl-70 (p = 0.024) antibodies, negative correlation was found between LF and the presence of anti-histone (p = 0.03), anti-centromere A (p = 0.009), anti-centromere B (p = 0.014), and anti-nucleosomes (p = 0.03) antibodies. No correlation between the presence of aPL and the above complications was found. The prevalence of individual antibody types in SSc and MCTD may have ethnic and geographical grounds. Scl-70 antibodies correlate positively with LF. Anti-centromere, anti-histone, and anti-nucleosome antibodies reduce its risk. No correlation between aPL and the occurrence of LF and elevated PH risk was found.<\/jats:p>","DOI":"10.3390\/ijms26125684","type":"journal-article","created":{"date-parts":[[2025,6,13]],"date-time":"2025-06-13T06:19:28Z","timestamp":1749795568000},"page":"5684","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Antibody Profile of Systemic Sclerosis and Mixed Connective Tissue Disease and Its Relationship with Lung Fibrosis and Pulmonary Hypertension"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9770-1019","authenticated-orcid":false,"given":"Karolina","family":"Niklas","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Rehabilitation and Internal Diseases, Poznan University of Medical Science, 28 Czerwca 1956r. 135\/147, 61-545 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7326-6916","authenticated-orcid":false,"given":"Dorota","family":"Sikorska","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Rehabilitation and Internal Diseases, Poznan University of Medical Science, 28 Czerwca 1956r. 135\/147, 61-545 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5282-1570","authenticated-orcid":false,"given":"Tatiana","family":"Mularek-Kubzdela","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Poznan University of Medical Sciences, D\u0142uga 1\/2, 60-848 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanna","family":"Witoszy\u0144ska-Sobkowiak","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Rehabilitation and Internal Diseases, Poznan University of Medical Science, 28 Czerwca 1956r. 135\/147, 61-545 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iwona","family":"\u017bychowska","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Rehabilitation and Internal Diseases, Poznan University of Medical Science, 28 Czerwca 1956r. 135\/147, 61-545 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W\u0142odzimierz","family":"Samborski","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Rehabilitation and Internal Diseases, Poznan University of Medical Science, 28 Czerwca 1956r. 135\/147, 61-545 Poznan, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,6,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e293","DOI":"10.1016\/j.lpm.2014.06.007","article-title":"Update in systemic sclerosis-associated pulmonary arterial hypertension","volume":"43","author":"Gashouta","year":"2014","journal-title":"Presse Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1111\/j.1365-2133.2007.08392.x","article-title":"The clinical relevance of serum antinuclear antibodies in Japanese patients with systemic sclerosis","volume":"158","author":"Hamaguchi","year":"2008","journal-title":"Br. J. Dermatol."},{"key":"ref_3","first-page":"104","article-title":"Disease subsets, antinuclear antibody profile, and clinical features in 127 French and 247 US adult patients with systemic sclerosis","volume":"34","author":"Meyer","year":"2007","journal-title":"J. Rheumatol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"576","DOI":"10.3109\/08916934.2011.592886","article-title":"Distinctive autoantibody profile in Mexican Mestizo systemic sclerosis patients","volume":"44","year":"2011","journal-title":"Autoimmunity"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1002\/art.39432","article-title":"Characteristics and survival of anti-U1 RNP antibody-positive patients with connective tissue disease-associated pulmonary arterial hypertension","volume":"68","author":"Sobanski","year":"2016","journal-title":"Arthritis Rheumatol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1016\/j.semarthrit.2016.10.004","article-title":"Clinical relevance of antiphospholipid antibodies in systemic sclerosis: A systematic review and meta-analysis","volume":"46","author":"Merashli","year":"2017","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1038\/nrrheum.2009.238","article-title":"Autoantibodies as predictive tools in systemic sclerosis","volume":"6","author":"Nihtyanova","year":"2010","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1355","DOI":"10.1136\/annrheumdis-2011-200742","article-title":"EUSTAR Co-authors. Update on the profile of the EUSTAR cohort: An analysis of the EULAR Scleroderma Trials and Research group database","volume":"71","author":"Meier","year":"2012","journal-title":"Ann. Rheum. Dis."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1921","DOI":"10.1007\/s10067-015-2981-3","article-title":"A cross-sectional study of autoantibody profiles in the Waikato systemic sclerosis cohort, New Zealand","volume":"34","author":"Chang","year":"2015","journal-title":"Clin. Rheumatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"987","DOI":"10.5604\/17322693.1117543","article-title":"Serological profile of patients with systemic sclerosis","volume":"68","author":"Wielosz","year":"2014","journal-title":"Postepy Hig. Med. Dosw."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"\u017bebryk, P., Przymusza\u0142a, P., Nowak, J.K., Piorunek, T., Mularek-Kubzdela, T., and Puszczewicz, M. (2023). Autoantibodies and Clinical Correlations in Polish Systemic Sclerosis Patients: A Cross-Sectional Study. J. Clin. Med., 12.","DOI":"10.3390\/jcm12020657"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1843","DOI":"10.1002\/acr.22872","article-title":"Epidemiology of mixed connective tissue disease, 1985-2014: A population-based study","volume":"68","author":"Ungprasert","year":"2016","journal-title":"Arthritis Care Res."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1007\/s00296-009-1107-0","article-title":"Clinical significance of antiphospholipid antibodies in Indian scleroderma patients","volume":"30","author":"Gupta","year":"2009","journal-title":"Rheumatol. Int."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"89","DOI":"10.4103\/2229-5178.153009","article-title":"Association of anti-phospholipid antibodies with connective tissue diseases","volume":"6","author":"Rai","year":"2015","journal-title":"Indian. Dermatol. Online J."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"32","DOI":"10.4274\/tjh.2012.0059","article-title":"Antiphospholipid antibodies and systemic scleroderma","volume":"30","author":"Ly","year":"2013","journal-title":"Turk. J. Haematol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1002\/art.1780370111","article-title":"Clinical and prognostic associations based on serum antinuclear antibodies in Japanese patients with systemic sclerosis","volume":"37","author":"Kuwana","year":"1994","journal-title":"Arthritis Rheum."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1007\/s10354-007-0451-5","article-title":"Autoantibodies in systemic sclerosis (scleroderma): Clues for clinical evaluation, prognosis and pathogenesis","volume":"158","author":"Grassegger","year":"2008","journal-title":"Wien. Med. Wochenschr."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1111\/j.1756-185X.2011.01688.x","article-title":"South Australian Scleroderma Register: Autoantibodies as predictive biomarkers of phenotype and outcome","volume":"15","author":"Graf","year":"2012","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1111\/j.1346-8138.2009.00762.x","article-title":"Autoantibody profiles in systemic sclerosis: Predictive value for clinical evaluation and prognosis","volume":"37","author":"Hamaguchi","year":"2010","journal-title":"J. Dermatol."},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Cozzani, E., Muracchioli, A., Murdaca, G., Beccalli, M., Caprioli, S., Zentilin, P., Ameri, P., Grosso, M., Russo, R., and Carmisciano, L. (2021). Correlation Between Skin and Affected Organs in 52 Sclerodermic Patients Followed in a Diseases Management Team: Development of a Risk Prediction Model of Organ-Specific Complications. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.588753"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1016\/j.revmed.2003.11.012","article-title":"Nuclear auto-antibodies: A useful tool for the diagnosis, the classification and the prognosis of systemic sclerosis","volume":"25","author":"Hachulla","year":"2004","journal-title":"Rev. Med. Interne"},{"key":"ref_22","first-page":"98","article-title":"RESCLE investigators, Autoimmune Diseases Study Group (GEAS). Influence of antibody profile in clinical features and prognosis in a cohort of Spanish patients with systemic sclerosis","volume":"35","year":"2017","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"2407526","DOI":"10.1080\/07853890.2024.2407526","article-title":"Autoantibodies in systemic sclerosis overlap syndrome and their correlation with organ damage and survival","volume":"56","author":"Wang","year":"2024","journal-title":"Ann. Med."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1007\/s13317-011-0015-y","article-title":"Prevalence of anti-histone antibodies, their clinical significance and correlation with other autoantibodies in a cohort of Italian scleroderma patients","volume":"2","author":"Morozzi","year":"2011","journal-title":"Auto. Immun. Highlights"},{"key":"ref_25","first-page":"70","article-title":"Silica-associated systemic sclerosis occurring after an occupational exposure to arc welding","volume":"25","author":"Alaya","year":"2016","journal-title":"Pan Afr. Med. J."},{"key":"ref_26","first-page":"S133","article-title":"The association of antiphospholipid antibodies with cardiopulmonary manifestations of systemic sclerosis","volume":"32","author":"Morrisroe","year":"2014","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"184","DOI":"10.5603\/CJ.a2018.0096","article-title":"Pulmonary arterial hypertension in the elderly: Clinical perspectives","volume":"27","author":"Rothbard","year":"2020","journal-title":"Cardiol. J."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1016\/j.semarthrit.2015.06.011","article-title":"Survival in systemic sclerosis-pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry","volume":"45","author":"Hinchcliff","year":"2015","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"e2072","DOI":"10.1002\/hsr2.2072","article-title":"Neurological, cardiac, musculoskeletal, and renal manifestations of scleroderma along with insights into its genetics, pathophysiology, diagnostic, and therapeutic updates","volume":"7","author":"Prajjwal","year":"2024","journal-title":"Health Sci. Rep."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1177\/0961203308101719","article-title":"The frequency of anti-beta2-glycoprotein I antibodies is low and these antibodies are associated with pulmonary hypertension in mixed connective tissue disease","volume":"18","author":"Hasegawa","year":"2009","journal-title":"Lupus"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"e11437","DOI":"10.1097\/MD.0000000000011437","article-title":"Prevalence of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease","volume":"97","author":"Niklas","year":"2018","journal-title":"Medicine"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"2737","DOI":"10.1002\/art.38098","article-title":"2013 classification criteria for systemic sclerosis: An American College of Rheumatology\/European League against Rheumatism collaborative initiative","volume":"65","author":"Khanna","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_33","unstructured":"Kasukawa, R., and Sharp, G.C. (1987). Classification and diagnostic criteria for mixed connective tissue disease. Mixed Connective Tissue Disease and Anti-Nuclear Antibodies, Elsevier Science."},{"key":"ref_34","unstructured":"Kasukawa, R., and Sharp, G.C. (1987). Preliminary diagnostic criteria for classification of mixed connective tissue disease. Mixed Connective Tissue Disease and Anti-Nuclear Antibodies, Elsevier Science."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1127","DOI":"10.5603\/KP.2015.0242","article-title":"2015 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"73","author":"Humbert","year":"2015","journal-title":"Kardiol. Pol."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/26\/12\/5684\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T17:51:23Z","timestamp":1760032283000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/26\/12\/5684"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6,13]]},"references-count":35,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2025,6]]}},"alternative-id":["ijms26125684"],"URL":"https:\/\/doi.org\/10.3390\/ijms26125684","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,6,13]]}}}