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Immunol."],"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To identify the existence of a correlation among the various organs affected, focusing primarily on immuno-dermatological aspects, and to create a risk prediction model of organ-specific complications.<\/jats:p><\/jats:sec><jats:sec><jats:title>Material and Methods<\/jats:title><jats:p>Fifty-two patients with stable scleroderma, followed between 2015 and 2019, were investigated through an extensive multidisciplinary evaluation in the last year.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients with lung involvement presented a worse degree of skin fibrosis than patients without it (p &amp;lt;0.001). No relationship was observed for the heart, kidney, and esophagus. Patients with pulmonary involvement had a lower pressure of the low esophagus sphincter and a higher Warrick score than patients without it (p &amp;lt;0.05). Age was significantly higher in patients with kidney involvement. Diffuse scleroderma patients had a worse pulmonary impairment than limited scleroderma patients (p &amp;lt;0.05). The manometric \u201csclerodermic\u201d pattern was observed to be the most frequent (55.6%, p &amp;lt;0.05) in dcSSc patients while the sclerodermic and normal pattern were equally represented (41.2 and 32.4% respectively, p &amp;lt;0.05) in lcSSc patients. When compared to the negative serological groups, anti-Scl-70 positive patients presented a worse lung involvement while anti-centromere patients presented a better lung outcome (p &amp;lt;0.05). PM-Scl 100\/75 positive patients presented mostly a pulmonary fibrotic pattern (p &amp;lt;0.05) and, also, heart complications were more likely associated with anti PM-Scl 100\/75 positivity (p &amp;lt;0.05). The risk prediction model for organ-specific complications had an accuracy of 84.4% (95%CI 78, 89) in complication-site prediction, AUC of 0.871, 86% of sensitivity, and 83% of specificity, Cohen\u2019s Kappa (k) of 0.68.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Out of all the organs studied, the skin is the one that correlates with the lung. Patients with a diffuse form of disease presented more frequently the anti Scl-70 antibody and had a worse lung and esophageal involvement (scleroderma pattern) than the negative group. Conversely, patients with limited disease presented all positive for the anti-centromere antibody with a better lung involvement than the negative group, without any difference among the esophageal manometric pattern. Anti PM-Scl 100\/75 antibody patients were associated with pulmonary fibrosis and presented cardiac involvement. The model created has demonstrated excellent values of sensitivity, specificity, and accuracy, but further studies are needed for validation.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fimmu.2021.588753","type":"journal-article","created":{"date-parts":[[2021,6,2]],"date-time":"2021-06-02T12:35:38Z","timestamp":1622637338000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":8,"title":["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"],"prefix":"10.3389","volume":"12","author":[{"given":"Emanuele","family":"Cozzani","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Muracchioli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuseppe","family":"Murdaca","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mirko","family":"Beccalli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simone","family":"Caprioli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrizia","family":"Zentilin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pietro","family":"Ameri","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marco","family":"Grosso","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rodolfo","family":"Russo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luca","family":"Carmisciano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aurora","family":"Parodi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2021,6,2]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.5301\/jsrd.5000231","article-title":"Standardization of the Modified Rodnan Skin Score for Use in Clinical Trials of Systemic Sclerosis","volume":"2","author":"Khanna","year":"2017","journal-title":"J Scleroderma Relat Disord"},{"key":"B2","article-title":"Scleroderma (Systemic Sclerosis): Classification, Subsets and Pathogenesis","volume":"15","author":"LeRoy","year":"1988","journal-title":"J Rheumatol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1111\/j.1572-0241.2008.02119","article-title":"The Role of Nonacid Reflux in NERD: Lessons Learned From Impedance-Ph Monitoring in 150 Patients Off Therapy","volume":"103","author":"Savarino","year":"2008","journal-title":"Am J Gastroenterol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1016\/j.dld.2016.06.021","article-title":"Practice Guidelines on the Use of Esophageal Manometry -\u00a0a GISMAD-SIGE-AIGO Medical Position Statement","volume":"48","author":"Savarino","year":"2016","journal-title":"Digest Liver Dis"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00034805","article-title":"Standardisation of Spirometry","volume":"26","author":"Miller","year":"2005","journal-title":"Eur Respir J"},{"key":"B6","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1183\/09041950.005s1693","article-title":"Lung Volumes and Forced Ventilatory Flows. 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