Clues to detect tumor necrosis factor receptor-associated periodic syndrome (TRAPS) among patients with idiopathic recurrent acute pericarditis: results of a multicentre study

Cantarini, Luca; Lucherini, Orso Maria; Brucato, Antonio; Barone, Luca; Cumetti, Davide; Iacoponi, Francesca; Rigante, Donato; Brambilla, Giovanni; Penco, Silvana; Brizi, Maria Giuseppina; Patrosso, Maria Cristina; Valesini, Guido; Frediani, Bruno; Gargiulo, Mauro; Cimaz, Rolando; Paolazzi, Giuseppe; Vitale, A.; Imazio, Massimo

doi:10.1007/s00392-012-0422-8

Cited by 100 publications

(74 citation statements)

References 31 publications

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“…U części pacjentów okresowo występuje gorączka, co może być przejawem uwarunkowanych genetycznie chorób wywołanych mutacjami genów odpowiedzialnych za regulację odczynu zapalnego, bez udziału swoistych limfocytów T czy autoprzeciwciał [158][159][160][161]. Choroby te zwykle są rozpoznawane u dzieci, choć mogą się ujawnić dopiero w wieku dorosłym.…”

Section: Zajęcie Osierdzia W Przebiegu Układowych Chorób Autozapalnycunclassified

“…Najczęściej sstwierdza się: rodzinną gorączkę śródziemnomorską (FMF), w przebiegu której epizody zapalenia błon surowiczych trwają tylko 1-3 dni, a także zespół okresowych gorączek związanych z defektem receptora dla czynnika martwicy guza (TRAPS), w przypadku którego epizody objawowe trwają kilka tygodni. Mutacje odpowiedzialne za powyższe schorzenia rzadko obserwuje się u chorych na nawracające zapalenie osierdzia [158][159][160][161]. Choroby te mogą przebiegać z nadmierną ekspresją IL-1 [161].…”

Section: Zajęcie Osierdzia W Przebiegu Układowych Chorób Autozapalnycunclassified

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2015 ESC Guidelines for the diagnosis and management of pericardial diseases

Adler

Charron²,

Imazio³

et al. 2015

Kardiol Pol

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173

293

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Section: Zajęcie Osierdzia W Przebiegu Układowych Chorób Autozapalnycunclassified

2015 ESC Guidelines for the diagnosis and management of pericardial diseases

Adler

Charron²,

Imazio³

et al. 2015

Kardiol Pol

Self Cite

173

293

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“…Cantarini et al reported that 6% of IRP patients have TNFRSF1A mutations (16). TNFRSF1A codes tumor necrosis factor (TNF) α, and the mutation of TNFRSF1A causes TRAPS, a common autoinflammatory disease.…”

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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“…33 In a multicenter study features associated with detection of TRAPS mutations in IRAP included: positive family history for pericarditis and/or recurrent fever syndrome, relapses occurring years after the first year from disease onset, colchicine failure, and need of immunosuppressive agents. 43 …”

Section: Is Irap An Ainfd?mentioning

confidence: 99%

“…The anti-inflammatory effect mediated by colchicine may be beneficial both in autoimmune and autoinflammatory IRAP subsets, but the drug is not effective in TRAPS. 43 High corticosteroid doses (prednisone 1-1.5 mg/kg/daily) were successfully used in selected refractory cases in a small observational trial. 47 Corticosteroids could delay viral clearance and promote recurrences in viral forms.…”

Section: Irap Therapymentioning

confidence: 99%

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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Clues to detect tumor necrosis factor receptor-associated periodic syndrome (TRAPS) among patients with idiopathic recurrent acute pericarditis: results of a multicentre study

Cited by 100 publications

References 31 publications

2015 ESC Guidelines for the diagnosis and management of pericardial diseases

2015 ESC Guidelines for the diagnosis and management of pericardial diseases

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

Acute idiopathic pericarditis: current immunological theories

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