{"orpha":251307,"name":"Idiopathic recurrent pericarditis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Idiopathic relapsing pericarditis"],"definition":"Idiopathic recurrent pericarditis is a rare autoinflammatory syndrome defined as recurrence of pericardial inflammation of unknown origin following the first episode of acute pericarditis and a symptom-free interval of 4-6 weeks or longer. Recurrent attacks of chest pain may be the sole presentation or the chest pain may be accompanied by pericardial friction rub, electrocardiographic or echocardiographic changes, pericardial effusion and increased C-reactive protein. Cardiac tamponade is a rare, life-threatening complication.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251307/","orphanet_url":"https://www.orpha.net/en/disease/detail/251307","names":{"fr":{"name":"Péricardite récurrente idiopathique","definition":"Un syndrome auto-inflammatoire rare défini par la récurrence d'une inflammation péricardique d'origine inconnue après un premier épisode de péricardite aigüe, et un intervalle sans symptôme de 4-6 semaines ou plus. Les attaques récurrentes de douleur thoracique peuvent être l'unique présentation, ou la douleur thoracique peut s'accompagner d'un frottement péricardique, d'anomalies à l'électrocardiogramme ou à l'échocardiogramme, d'une effusion péricardique et d'une augmentation de la protéine C-réactive. La tamponnade cardiaque est une complication rare, mettant en jeu le pronostic vital."},"de":{"name":"Perikarditis, idiopathische rekurrente","definition":"Die idiopathische rezidivierende Perikarditis ist ein seltenes autoinflammatorisches Syndrom, das als Wiederauftreten einer Perikarditis unbekannten Ursprungs nach der ersten Episode einer akuten Perikarditis und einem symptomfreien Intervall von 4-6 Wochen oder länger definiert ist. Wiederkehrende Brustschmerzattacken können die einzige Präsentation sein oder die Brustschmerzen können von perikardialem Reiben, elektrokardiographischen oder echokardiographischen Veränderungen, Perikarderguss und erhöhtem C-reaktiven Protein begleitet sein. Die Herztamponade ist eine seltene, lebensbedrohliche Komplikation."},"es":{"name":"Pericarditis recurrente idiopática","definition":"La pericarditis recurrente idiopática, es un síndrome autoinflamatorio poco frecuente que se define en base a la presencia de inflamación pericárdica recurrente de origen desconocido después de un primer episodio de pericarditis aguda y un intervalo asintomático de 4 a 6 semanas o más. Los episodios recurrentes de dolor torácico pueden ser el único signo de presentación, aunque el dolor torácico puede ir acompañado de roce pericárdico, cambios electro- o ecocardiográficos, derrame pericárdico e incremento de la proteína C reactiva. El taponamiento cardíaco es una complicación poco frecuente potencialmente mortal."},"it":{"name":"Pericardite idiopatica ricorrente","synonyms":["Pericardite recidivante idiopatica"],"definition":"La pericardite idiopatica ricorrente è una malattia autoinfiammatoria rara, caratterizzata dalla ricorrenza dell'infiammazione pericardica ad eziologia ignota, a seguito di un primo episodio di pericardite acuta e dopo un intervallo di 4-6 settimane o più in cui il paziente è asintomatico. Gli attacchi ricorrenti di dolore toracico possono essere l'unico sintomo d'esordio; il dolore toracico può anche associarsi a sfregamento pericardico, alterazioni elettrocardiografiche o ecocardiografiche, versamento pericardico e aumento dei livelli della proteina C-reattiva. Il tamponamento cardiaco è una complicanza rara e potenzialmente letale."},"nl":{"name":"Idiopathische recidiverende pericarditis","synonyms":["Idiopathische terugkerende pericarditis"],"definition":"Idiopathische recurrente pericarditis is een zeldzaam auto-inflammatoir syndroom dat wordt gedefinieerd als recurrentie van pericardiale inflammatie met ongekende oorsprong volgend op de eerste episode van acute pericarditis en een symptoomvrij interval van 4-6 weken of langer. Terugkerende aanvallen van pijn in de borst kan het enige presenterende symptoom zijn, of de borstpijn kan gepaard gaan met pericardwrijven, elektrocardiografische of echocardiografische veranderingen, pericardiale effusie en een toename van C-reactief proteïne. Harttamponade is een zeldzame, levensbedreigende complicatie."},"pt":{"name":"Pericardite recorrente idiopática","synonyms":["Pericardite recidivante idiopática"]},"pl":{"name":"Idiopatyczne nawracające zapalenie osierdzia","synonyms":["Idiopathic relapsing pericarditis"]},"cs":{"name":"Idiopatická rekurentní perikarditida","synonyms":["Idiopatická relabující perikarditida"]},"uk":{"name":"Ідіопатичний рецидивуючий перикардит","synonyms":["Ідіопатичний рецидивуючий перикардит"],"definition":"Idiopathic recurrent pericarditis is a rare autoinflammatory syndrome defined as recurrence of pericardial inflammation of unknown origin following the first episode of acute pericarditis and a symptom-free interval of 4-6 weeks or longer. Recurrent attacks of chest pain may be the sole presentation or the chest pain may be accompanied by pericardial friction rub, electrocardiographic or echocardiographic changes, pericardial effusion and increased C-reactive protein. Cardiac tamponade is a rare, life-threatening complication."},"zh":{"name":"特发性复发性心包炎","synonyms":["特发性复发性心包炎"],"definition":"特发性复发性心包炎是一种罕见的自体炎症综合征，定义为急性心包炎首次发作后复发的原因不明的心包炎，无症状间隔4-6周或更长时间。反复发作的胸痛可能是唯一的症状，或者胸痛可能伴有心包摩擦、心电图或超声心动图改变、心包积液和C反应蛋白升高。心包填塞是一种罕见的危及生命的并发症。"},"tr":{"name":"İdiyopatik tekrarlayan perikardit","synonyms":["İdiyopatik tekrarlayan perikardit"]}},"xrefs":[{"source":"ICD-10","code":"I09.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"BB2Y","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0016662","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707790","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":324936,"name":"Unclassified autoinflammatory syndrome","classifications":[196]},{"orpha":324953,"name":"Unclassified autoinflammatory syndrome of childhood","classifications":[231]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}