Serum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates

Background: Heart involvement (HInv) in systemic sclerosis (SSc) may relate to myocarditis and is associated with poor prognosis. Serum anti-heart (AHA) and anti-intercalated disk autoantibodies (AIDA) are organ and disease-specific markers of isolated autoimmune myocarditis. We assessed frequencies...

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Autores principales:	Alida Linda Patrizia Caforio, Giacomo De Luca, Anna Baritussio, Mara Seguso, Nicoletta Gallo, Elisa Bison, Maria Grazia Cattini, Elena Pontara, Luna Gargani, Alessia Pepe, Corrado Campochiaro, Mario Plebani, Sabino Iliceto, Giovanni Peretto, Antonio Esposito, Lorenzo Tofani, Alberto Moggi-Pignone, Lorenzo Dagna, Renzo Marcolongo, Marco Matucci-Cerinic, Cosimo Bruni
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	myocarditis autoimmunity autoantibodies systemic sclerosis prognosis Medicine (General) R5-920
Acceso en línea:	https://doaj.org/article/5ee2a4acdc69483b82bbf3129489b530
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spelling	oai:doaj.org-article:5ee2a4acdc69483b82bbf3129489b5302021-11-25T17:22:12ZSerum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates10.3390/diagnostics111121652075-4418https://doaj.org/article/5ee2a4acdc69483b82bbf3129489b5302021-11-01T00:00:00Zhttps://www.mdpi.com/2075-4418/11/11/2165https://doaj.org/toc/2075-4418Background: Heart involvement (HInv) in systemic sclerosis (SSc) may relate to myocarditis and is associated with poor prognosis. Serum anti-heart (AHA) and anti-intercalated disk autoantibodies (AIDA) are organ and disease-specific markers of isolated autoimmune myocarditis. We assessed frequencies, clinical correlates, and prognostic impacts of AHA and AIDA in SSc. Methods: The study included consecutive SSc patients (<i>n</i> = 116, aged 53 ± 13 years, 83.6% females, median disease duration 7 years) with clinically suspected heart involvement (symptoms, abnormal ECG, abnormal troponin I or natriuretic peptides, and abnormal echocardiography). All SSc patients underwent CMR. Serum AHA and AIDA were measured by indirect immunofluorescence in SSc and in control groups of non-inflammatory cardiac disease (NICD) (<i>n</i> = 160), ischemic heart failure (IHF) (<i>n</i> = 141), and normal blood donors (NBD) (<i>n</i> = 270). AHA and AIDA status in SSc was correlated with baseline clinical, diagnostic features, and outcome. Results: The frequency of AHA was higher in SSc (57/116, 49%, <i>p</i> < 0.00001) than in NICD (2/160, 1%), IHF (2/141, 1%), or NBD (7/270, 2.5%). The frequency of AIDA was higher (65/116, 56%, <i>p</i> < 0.00001) in SSc than in NICD (6/160, 3.75%), IHF (3/141, 2%), or NBD (1/270, 0.37%). AHAs were associated with interstitial lung disease (<i>p</i> = 0.04), history of chest pain (<i>p</i> = 0.026), abnormal troponin (<i>p</i> = 0.006), AIDA (<i>p</i> = 0.000), and current immunosuppression (<i>p</i> = 0.01). AHAs were associated with death (<i>p</i> = 0.02) and overall cardiac events during follow-up (<i>p</i> = 0.017). Conclusions: The high frequencies of AHA and AIDA suggest a high burden of underdiagnosed autoimmune HInv in SSc. In keeping with the negative prognostic impact of HInv in SSc, AHAs were associated with dismal prognosis.Alida Linda Patrizia CaforioGiacomo De LucaAnna BaritussioMara SegusoNicoletta GalloElisa BisonMaria Grazia CattiniElena PontaraLuna GarganiAlessia PepeCorrado CampochiaroMario PlebaniSabino IlicetoGiovanni PerettoAntonio EspositoLorenzo TofaniAlberto Moggi-PignoneLorenzo DagnaRenzo MarcolongoMarco Matucci-CerinicCosimo BruniMDPI AGarticlemyocarditisautoimmunityautoantibodiessystemic sclerosisprognosisMedicine (General)R5-920ENDiagnostics, Vol 11, Iss 2165, p 2165 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	myocarditis autoimmunity autoantibodies systemic sclerosis prognosis Medicine (General) R5-920
spellingShingle	myocarditis autoimmunity autoantibodies systemic sclerosis prognosis Medicine (General) R5-920 Alida Linda Patrizia Caforio Giacomo De Luca Anna Baritussio Mara Seguso Nicoletta Gallo Elisa Bison Maria Grazia Cattini Elena Pontara Luna Gargani Alessia Pepe Corrado Campochiaro Mario Plebani Sabino Iliceto Giovanni Peretto Antonio Esposito Lorenzo Tofani Alberto Moggi-Pignone Lorenzo Dagna Renzo Marcolongo Marco Matucci-Cerinic Cosimo Bruni Serum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates
description	Background: Heart involvement (HInv) in systemic sclerosis (SSc) may relate to myocarditis and is associated with poor prognosis. Serum anti-heart (AHA) and anti-intercalated disk autoantibodies (AIDA) are organ and disease-specific markers of isolated autoimmune myocarditis. We assessed frequencies, clinical correlates, and prognostic impacts of AHA and AIDA in SSc. Methods: The study included consecutive SSc patients (<i>n</i> = 116, aged 53 ± 13 years, 83.6% females, median disease duration 7 years) with clinically suspected heart involvement (symptoms, abnormal ECG, abnormal troponin I or natriuretic peptides, and abnormal echocardiography). All SSc patients underwent CMR. Serum AHA and AIDA were measured by indirect immunofluorescence in SSc and in control groups of non-inflammatory cardiac disease (NICD) (<i>n</i> = 160), ischemic heart failure (IHF) (<i>n</i> = 141), and normal blood donors (NBD) (<i>n</i> = 270). AHA and AIDA status in SSc was correlated with baseline clinical, diagnostic features, and outcome. Results: The frequency of AHA was higher in SSc (57/116, 49%, <i>p</i> < 0.00001) than in NICD (2/160, 1%), IHF (2/141, 1%), or NBD (7/270, 2.5%). The frequency of AIDA was higher (65/116, 56%, <i>p</i> < 0.00001) in SSc than in NICD (6/160, 3.75%), IHF (3/141, 2%), or NBD (1/270, 0.37%). AHAs were associated with interstitial lung disease (<i>p</i> = 0.04), history of chest pain (<i>p</i> = 0.026), abnormal troponin (<i>p</i> = 0.006), AIDA (<i>p</i> = 0.000), and current immunosuppression (<i>p</i> = 0.01). AHAs were associated with death (<i>p</i> = 0.02) and overall cardiac events during follow-up (<i>p</i> = 0.017). Conclusions: The high frequencies of AHA and AIDA suggest a high burden of underdiagnosed autoimmune HInv in SSc. In keeping with the negative prognostic impact of HInv in SSc, AHAs were associated with dismal prognosis.
format	article
author	Alida Linda Patrizia Caforio Giacomo De Luca Anna Baritussio Mara Seguso Nicoletta Gallo Elisa Bison Maria Grazia Cattini Elena Pontara Luna Gargani Alessia Pepe Corrado Campochiaro Mario Plebani Sabino Iliceto Giovanni Peretto Antonio Esposito Lorenzo Tofani Alberto Moggi-Pignone Lorenzo Dagna Renzo Marcolongo Marco Matucci-Cerinic Cosimo Bruni
author_facet	Alida Linda Patrizia Caforio Giacomo De Luca Anna Baritussio Mara Seguso Nicoletta Gallo Elisa Bison Maria Grazia Cattini Elena Pontara Luna Gargani Alessia Pepe Corrado Campochiaro Mario Plebani Sabino Iliceto Giovanni Peretto Antonio Esposito Lorenzo Tofani Alberto Moggi-Pignone Lorenzo Dagna Renzo Marcolongo Marco Matucci-Cerinic Cosimo Bruni
author_sort	Alida Linda Patrizia Caforio
title	Serum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates
title_short	Serum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates
title_full	Serum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates
title_fullStr	Serum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates
title_full_unstemmed	Serum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates
title_sort	serum organ-specific anti-heart and anti-intercalated disk autoantibodies as new autoimmune markers of cardiac involvement in systemic sclerosis: frequency, clinical and prognostic correlates
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/5ee2a4acdc69483b82bbf3129489b530
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Serum Organ-Specific Anti-Heart and Anti-Intercalated Disk Autoantibodies as New Autoimmune Markers of Cardiac Involvement in Systemic Sclerosis: Frequency, Clinical and Prognostic Correlates

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