Serum Anti-Heart and Anti-Intercalated Disk Autoantibodies: Novel Autoimmune Markers in Cardiac Sarcoidosis
Open accessAbstract
Background: Sarcoidosis is an immune-mediated disease. Cardiac involvement, a granulomatous form of myocarditis, is under-recognized and prognostically relevant. Anti-heart autoantibodies (AHAs) and anti-intercalated disk autoantibodies (AIDAs) are autoimmune markers in nonsarcoidosis myocarditis forms. Objective: The aim was to assess serum AHAs and AIDAs as autoimmune markers in cardiac sarcoidosis. Methods: This is a cross-sectional study on AHA and AIDA frequency in: 29 patients (aged 46 ± 12, 20 male) with biopsy-proven extracardiac sarcoidosis and biopsy-proven or clinically suspected and confirmed by 18-fluorodeoxyglucose positron emission tomography and/or cardiovascular magnetic resonance (CMR) cardiac involvement; 30 patients (aged 44 ± 11, 12 male) with biopsy-proven extracardiac sarcoidosis without cardiac involvement (no cardiac symptoms, normal 12-lead electrocardiogram, echocardiography and CMR), and control patients with noninflammatory cardiac disease (NICD) (n = 160), ischemic heart failure (IHF) (n = 141) and normal blood donors (NBDs) (n = 270). Sarcoidosis patients were recruited in two recruiting tertiary centers in the USA and Italy. AHAs and AIDAs were detected by indirect immunofluorescence on the human myocardium and skeletal muscle. Results: AHA and AIDA frequencies were higher in sarcoidosis with cardiac involvement (86%; 62%) than in sarcoidosis without cardiac involvement (0%; 0%), NICD (8%; 4%), IHF (7%; 2%) and NBD (9%; 0%) (p = 0.0001; p = 0.0001, respectively). Sensitivity and specificity for cardiac sarcoidosis were 86% and 92% for positive AHAs and 62% and 98% for positive AIDAs, respectively. AIDAs in cardiac sarcoidosis were associated with a higher number of involved organs (p = 0.04). Conclusions: Serum AHAs and AIDAs provide novel noninvasive diagnostic autoimmune markers for cardiac sarcoidosis.
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Anti-heart autoantibodies and anti-intercalated disk autoantibodies are present exclusively in patients with cardiac sarcoidosis and absent in those with sarcoidosis lacking cardiac involvement.
“findings by Caforio et al 218 indicate the presence of anti-heart autoantibodies and anti-intercalated disk autoantibodies exclusively in patients with cardiac sarcoidosis, absent in those with sarcoidosis lacking cardiac involvement.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [218]
Results §3.2 (page unavailable) found AHAs in 86% and AIDAs in 62% of the cardiac-sarcoidosis group, versus 0% for both in the group without cardiac involvement. The unqualified word “exclusively” overstates the finding: both antibodies also occurred in some non-sarcoidosis control groups, and neither was present in every cardiac-sarcoidosis patient. (LLM, conf=0.86)
Experimental — accuracy not yet measuredIn earlier studies, autoantibodies targeting heart antigens have been identified in both myocarditis patients and animal models, regardless of the underlying cause-viral infections or existing autoimmune conditions.
“In earlier studies, autoantibodies targeting heart antigens have been identified in both myocarditis patients and animal models, regardless of the underlying cause-viral infections or existing autoimmune conditions. 15-18,57,189,190,218”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [218]
The Introduction identifies anti-heart autoantibodies as markers in human myocarditis, and Discussion §4.3 describes cardiac myosin as an autoantibody target in several forms of myocarditis and in postinfectious immune-mediated heart diseases. The paper does not report findings in animal models or establish the claim specifically for viral infections, so those parts are unsupported by this source. (LLM, conf=0.86)
Experimental — accuracy not yet measuredAutoimmune responses against the heart may play a role in the onset and progress of cardiac sarcoidosis.
“This suggests a potential role of autoimmune responses against the heart in the onset and progress of cardiac sarcoidosis.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [218]
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- Authors
- Caforio, A. L. P., Baritussio, A., Marcolongo, R., Cheng, C.-Y., Pontara, E., Bison, E., Cattini, M. G., Gallo, N., Plebani, M., Iliceto, S., Semenzato, G., Maier, L., & Hamzeh, N.
- Affiliations
- University of Padua (IT)
- National Jewish Health (US)
- University of Iowa (US)
- Corresponding author
- Caforio, Alida L. P.
- ORCID
- Venue
- Journal of Clinical Medicine
- Publisher
- MDPI AG
- Published
- 2021-06-02
- Volume
- 10
- Issue
- 11
- Pages
- 2476
- ISSN
- 2077-0383 (ISSN-L)
- PMC
- PMC8199734
- OpenAlex
- W3166428737
- Cited by
- 22
- References
- 39
- Citations by year
- 2026: 6 · 2025: 1 · 2024: 7 · 2023: 2 · 2022: 4 · 2021: 2
- FWCI
- 2.36
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Journal of Clinical Medicine (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Padua Research Archive (University of Padova) (submittedVersion, cc-by)
- Funders
- Università degli Studi di Padova (BIRD2019)
- Topics
- Health Sciences › Medicine › Physiology › Sarcoidosis and Beryllium Toxicity Research
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Viral Infections and Immunology Research
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Vasculitis and related conditions
- Keywords
- cardiac sarcoidosis, autoimmune markers, myocarditis, granulomatous myocarditis, extracardiac sarcoidosis, sensitivity and specificity, non-invasive diagnosis, ischemic heart failure, organ involvement
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