Anti-heart and anti-intercalated disk autoantibodies: evidence for autoimmunity in idiopathic recurrent acute pericarditis

Caforio, Alida L.P.; Brucato, Antonio; Doria, Andrea; Brambilla, Giovanni; Angelini, Annalisa; Ghirardello, Anna; Bottaro, Stefania; Tona, Francesco; Betterle, Corrado; Daliento, Luciano; Thiene, Gaetano; Iliceto, Sabino

doi:10.1136/hrt.2009.187138

Cited by 145 publications

(121 citation statements)

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“…Another finding of this retrospective study was the association of positive AIDA status with a high number of relapses, hospitalizations, and more refractory symptoms, in keeping with high immune activation during the "hot" phases of pericardial inflammation. 19 This further reinforces the autoimmune hypothesis in IRAP. Whether or not AIDA may provide useful serum predictors of IRAP relapses and/or guide immunosuppressive therapy warrants future assessment by prospective studies.…”

Section: Is Irap As An O-s Aid?supporting

confidence: 72%

“…19 In particular Caforio et al 19 reported a high frequency of AHA of the cross-reactive 1 type and of AIDA by indirect immunofluorescence (IF) in patients with IRAP compared to non-inflammatory heart disease, ischemic heart failure, and normal control subjects. This finding is in keeping with an autoimmune component of the disease in at least 55% of patients.…”

Section: Is Irap As An O-s Aid?mentioning

confidence: 99%

“…19 It is likely that the antibody markers are more prevalent in the index episode. Another finding of this retrospective study was the association of positive AIDA status with a high number of relapses, hospitalizations, and more refractory symptoms, in keeping with high immune activation during the "hot" phases of pericardial inflammation.…”

Section: Is Irap As An O-s Aid?mentioning

confidence: 99%

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Acute idiopathic pericarditis: current immunological theories

Caforio¹,

Marcolongo²,

Brucato³

et al. 2012

RRCC

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Idiopathic recurrent acute pericarditis (IRAP) is a rare disease of suspected immune-mediated pathogenesis. It represents a diagnosis of exclusion. It is necessary to rule out infectious and noninfectious causes of pericardial inflammation, including systemic autoimmune and immune-related disorders, eg, Sjögren's disease, systemic lupus erythematosus. Since pericarditis may precede diagnosis of these disorders, IRAP diagnosis is often made after a long follow-up. According to the two main pathogenetic theories IRAP may represent an organ-specific autoimmune disease or an autoinflammatory disease (AInfD). The main evidence for autoimmunity in IRAP is provided by the detection of serum antiheart and antiintercalateddisk autoantibodies, and the response to anti-inflammatory or immunosuppressive therapy. The findings of familial forms and of proinflammatory cytokines in the pericardial fluid in IRAP would be in keeping with both organ-specific autoimmune disease and AInfD. In fact, AInfD are genetic disorders characterized by primary dysfunction of the innate immune system, due to mutations of genes involved in the regulation of the inflammatory response, in the absence of antigen specific T cells or autoantibodies. In AInfD there are active disease phases with raised non-cardiac specific inflammatory markers, such as C-reactive protein, as well as symptom-free intervals with possible C-reactive protein normalization. A minority of IRAP patients (6%) carry a mutation in the TNFRSF1A gene, encoding the receptor for tumor necrosis factor-alfa. This suggests that some IRAP patients may have an atypical or subclinical form of AInfD. Thus, IRAP may represent a syndrome with distinct pathogenetic mechanisms in different patients' subsets.

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Section: Is Irap As An O-s Aid?supporting

confidence: 72%

Section: Is Irap As An O-s Aid?mentioning

confidence: 99%

Section: Is Irap As An O-s Aid?mentioning

confidence: 99%

See 1 more Smart Citation

Acute idiopathic pericarditis: current immunological theories

Caforio¹,

Marcolongo²,

Brucato³

et al. 2012

RRCC

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“…Furthermore, anti-nuclear antibodies, anti-heart and antiintercalated-disk antibodies were detected in the idiopathic PE. Therefore, these evidences were thought to support the inflammatory pathogenesis in idiopathic PE (16,17) .…”

Section: Discussionmentioning

confidence: 99%

The Relation of Inflammatory Markers to Idiopathic Pericardial Effusion

Zencir¹

2017

Kosuyolu Heart Journal

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Introduction: With the growing evidence about the role of inflammation on cardiovascular disorders, studies have been focused on inflammatory markers for risk evaluation and disease activity monitoring. The aim of this study was to investigate whether inflammation has a role in idiopathic pericardial effusion (PE) and to reveal its correlation with the disease activity. Patients and Methods:The study population consisted of 107 patients with PE and 35 age-matched and sex-matched healthy volunteers. Each patient underwent a comprehensive clinical evaluation to identify the probable underlying systemic diseases or other potential causes for PE. Results:High-sensitivity C-reactive protein levels (hs-CRP), neutrophil/lymphocyte ratio (NLR) and platelet/lymphocyte ratio (PLR) (p< 0.001 for each) were significantly higher in the PE group than in healthy volunteers. The patients with PE were divided into three subgroups according to the amount of PE. hs-CRP, NLR and PLR significantly correlated with the amount of PE. In the multivariate logistic regression model NLR (OR 1.363, 95% CI 1.026-1.809; p= 0.033) and hs-CRP (OR 1.090, 95% CI 1.030-1.152; p= 0.003) remained as independent predictors of severe PE in patients who have PE.Conclusion: hs-CRP, NLR and PLR may be used for disease activity monitoring, treatment response evaluation and risk stratification of patients with PE.Key Words: High-sensitivity C-reactive protein; neutrophil/lymphocyte ratio; pericardial effusion; platelet/ lymphocyte ratio İdiyopatik Perikardiyal Efüzyon ile İnflamatuvar Belirteçler Arasındaki İlişki ÖZETGiriş: İnflamasyonun kardiyovasküler hastalıklardaki rolü hakkındaki kanıtların gittikçe artmasıyla, çalışma-lar inflamatuvar markerların hastalık aktivitesi ve risk değerlendirmesindeki rolüne odaklanmıştır. Bu çalış-manın amacı inflamatuvar belirteçlerin idiyopatik perikardiyal efüzyonla ile ilişkisini ve hastalık aktivitesi ile olan korelasyonu olup olmadığının incelenmesidir. Hastalar ve Yöntem:Çalışma popülasyonu perikardiyal efüzyonu olan 107 hasta ile yaş ve cinsiyet olarak benzer 35 sağlıklı gönüllülerden oluşmaktadır. Tüm hastalar perikardiyal efüzyonun etyolojisine yönelik kapsamlı bir klinik değerlendirmeye tabi tutulmuştur. Bulgular: Plevral efüzyonu grubunda, yüksek sensitif C-reaktif protein (Hs-CRP), nötrofil/lenfosit oranı (NLR) ve platelet/lenfosit oranı (PLR) (herbiri için p< 0.001), sağlıklı gönüllülerin grubuna göre anlamlı olarak daha yüksekti. Perikardiyal efüzyonu olan hastalar efüzyon miktarına göre 3 gruba bölündü. Hs-CRP, NLR ve PLR perikardiyal efüzyon miktarıyla korele bir şekilde artmaktadır. Çoklu analizde, NLR (OR 1.363, 95% CI 1.026-1.809; p= 0.033) ve Hs-CRP (OR 1.090, %95 CI 1.030-1.152; p= 0.003), perikardiyal efüzyo-nu olan hastalarda ciddi perikardiyal efüzyonun bağımsız bir prediktörleridir.Sonuç: Hs-CRP, NLR ve PLR perikardiyal efüzyonu olan hastalarda, hastalık aktivitesi, tedavi monitörizas-yonunda ve risk sınıflamasında kullanılabilir.Anahtar Kelimeler: Yüksek sensitif C-reaktif protein;

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“…An autoimmune pathogenesis of IRP is suggested as autoantibodies including ANA (8) and anti-heart antibodies (9), are detected in 43.3% and 67.5% of subjects, respectively. However, the existence of an autoantibody does not necessarily mean that the autoantibody is pathogenic.…”

Section: Discussionmentioning

confidence: 99%

Acute Pericarditis as the First Manifestation of Familial Mediterranean Fever: A Possible Relationship with Idiopathic Recurrent Pericarditis

et al. 2014

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A 56-year-old man was admitted to our hospital due to periodic episodes of acute pericarditis. These episodes occurred monthly along with a high fever and elevation of the C-reactive protein (CRP) level. The patient became afebrile and his CRP level decreased following the administration of a non-steroidal antiinflammatory drug. A mutation analysis revealed the heterozygote of the familial Mediterranean fever (FMF) gene (E84K, G304R). This finding confirmed our diagnosis, and we treated the patient with colchicine. He responded to treatment and has been visiting our hospital without disease recurrence. FMF should be included in the differential diagnosis of repeated episodes of pericarditis.

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Anti-heart and anti-intercalated disk autoantibodies: evidence for autoimmunity in idiopathic recurrent acute pericarditis

Abstract: The detection of AHA and of AIDA supports the involvement of autoimmunity in the majority of patients with IRAP.

Cited by 145 publications

References 50 publications

Acute idiopathic pericarditis: current immunological theories

Acute idiopathic pericarditis: current immunological theories

The Relation of Inflammatory Markers to Idiopathic Pericardial Effusion

Acute Pericarditis as the First Manifestation of Familial Mediterranean Fever: A Possible Relationship with Idiopathic Recurrent Pericarditis

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