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
ObjectiveTo 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.Material and MethodsFifty-two patients with stable scleroderma, followed between 2015 and...
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Frontiers Media S.A.
2021-06-01
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Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2021.588753/full |
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record_format |
Article |
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DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Emanuele Cozzani Andrea Muracchioli Giuseppe Murdaca Mirko Beccalli Simone Caprioli Patrizia Zentilin Pietro Ameri Marco Grosso Rodolfo Russo Luca Carmisciano Aurora Parodi |
spellingShingle |
Emanuele Cozzani Andrea Muracchioli Giuseppe Murdaca Mirko Beccalli Simone Caprioli Patrizia Zentilin Pietro Ameri Marco Grosso Rodolfo Russo Luca Carmisciano Aurora Parodi 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 Frontiers in Immunology systemic sclerosis scleroderma mRSS (Rodnan Score) predictive model organ complications |
author_facet |
Emanuele Cozzani Andrea Muracchioli Giuseppe Murdaca Mirko Beccalli Simone Caprioli Patrizia Zentilin Pietro Ameri Marco Grosso Rodolfo Russo Luca Carmisciano Aurora Parodi |
author_sort |
Emanuele Cozzani |
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 |
title_short |
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 |
title_full |
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 |
title_fullStr |
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 |
title_full_unstemmed |
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 |
title_sort |
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 |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Immunology |
issn |
1664-3224 |
publishDate |
2021-06-01 |
description |
ObjectiveTo 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.Material and MethodsFifty-two patients with stable scleroderma, followed between 2015 and 2019, were investigated through an extensive multidisciplinary evaluation in the last year.ResultsPatients with lung involvement presented a worse degree of skin fibrosis than patients without it (p <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 <0.05). Age was significantly higher in patients with kidney involvement. Diffuse scleroderma patients had a worse pulmonary impairment than limited scleroderma patients (p <0.05). The manometric “sclerodermic” pattern was observed to be the most frequent (55.6%, p <0.05) in dcSSc patients while the sclerodermic and normal pattern were equally represented (41.2 and 32.4% respectively, p <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 <0.05). PM-Scl 100/75 positive patients presented mostly a pulmonary fibrotic pattern (p <0.05) and, also, heart complications were more likely associated with anti PM-Scl 100/75 positivity (p <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’s Kappa (k) of 0.68.ConclusionsOut 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. |
topic |
systemic sclerosis scleroderma mRSS (Rodnan Score) predictive model organ complications |
url |
https://www.frontiersin.org/articles/10.3389/fimmu.2021.588753/full |
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doaj-fdb7a35207b24598ab4f1cc3c45016472021-06-02T08:22:26ZengFrontiers Media S.A.Frontiers in Immunology1664-32242021-06-011210.3389/fimmu.2021.588753588753Correlation Between Skin and Affected Organs in 52 Sclerodermic Patients Followed in a Diseases Management Team: Development of a Risk Prediction Model of Organ-Specific ComplicationsEmanuele Cozzani0Andrea Muracchioli1Giuseppe Murdaca2Mirko Beccalli3Simone Caprioli4Patrizia Zentilin5Pietro Ameri6Marco Grosso7Rodolfo Russo8Luca Carmisciano9Aurora Parodi10Dermatologic Unit, University of Genoa, DiSSal, Ospedale-Policlinico San Martino, IRCCS, Genova, ItalyDermatologic Unit, University of Genoa, DiSSal, Ospedale-Policlinico San Martino, IRCCS, Genova, ItalyDepartments of Internal Medicine, Scleroderma Unit, Clinical Immunology Unit, University of Genova, Ospedale-Policlinico San Martino, IRCCS, Genova, ItalyDepartments of Internal Medicine, Scleroderma Unit, Clinical Immunology Unit, University of Genova, Ospedale-Policlinico San Martino, IRCCS, Genova, ItalyDepartment of Healt Sciences DiSSal, University of Genova, Ospedale-Policlinico San Martino, IRCCS, Genova, ItalyDivision of Gastroenterology, Department of Internal Medicine, University of Genova, Ospedale-Policlinico San Martino, IRCCS, Genova, ItalyCardiovascular Disease Unit, IRCCS, Ospedale-Policlinico San Martino; Department of Internal Medicine, University of Genova, Genova, ItalyInterventional Pneumology Unit, Ospedale-Policlinico San Martino, IRCCS, Genova, ItalyNephrology, Dialysis, and Transplantation, Ospedale-Policlinico San Martino, IRCCS, Genova, ItalyDepartment of Healt Sciences DiSSal, University of Genova, Ospedale-Policlinico San Martino, IRCCS, Genova, ItalyDermatologic Unit, University of Genoa, DiSSal, Ospedale-Policlinico San Martino, IRCCS, Genova, ItalyObjectiveTo 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.Material and MethodsFifty-two patients with stable scleroderma, followed between 2015 and 2019, were investigated through an extensive multidisciplinary evaluation in the last year.ResultsPatients with lung involvement presented a worse degree of skin fibrosis than patients without it (p <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 <0.05). Age was significantly higher in patients with kidney involvement. Diffuse scleroderma patients had a worse pulmonary impairment than limited scleroderma patients (p <0.05). The manometric “sclerodermic” pattern was observed to be the most frequent (55.6%, p <0.05) in dcSSc patients while the sclerodermic and normal pattern were equally represented (41.2 and 32.4% respectively, p <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 <0.05). PM-Scl 100/75 positive patients presented mostly a pulmonary fibrotic pattern (p <0.05) and, also, heart complications were more likely associated with anti PM-Scl 100/75 positivity (p <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’s Kappa (k) of 0.68.ConclusionsOut 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.https://www.frontiersin.org/articles/10.3389/fimmu.2021.588753/fullsystemic sclerosissclerodermamRSS (Rodnan Score)predictive modelorgan complications |