Background/Purpose: Acute pericarditis is defined as at least 2 of the following criteria: typical chest pain (sharp and pleuritic, improved by sitting up and leaning forward), pericardial friction rub, suggestive EKG changes (widespread ST elevation or PR depression), new or worsening pericardial effusion. Pericarditis may recur in 15-30% of cases, and these children are frequently followed by pediatric Rheumatologists. In up to 68% of pediatric patients and in more than 80% of adult cases a specific etiology cannot be detected and pericarditis is considered idiopathic. Suggested explanations of recurrences include: insufficient dose and/or duration of non-steroidal anti-inflammatory drugs (NSAIDs), early corticosteroid treatment causing increased viral replication in pericardial tissue, too rapid tapering of corticosteroids, re-infection and exacerbation of the connective tissue disease.

Methods: Multicenter, retrospective cohort study including all consecutive cases of recurrent pericarditis of patients aged <18 years with at least 2 recurrences of pericarditis seen in referral centers in Italy. The study included 100 cases of recurrent pericarditis (median 13 years, range 1-17 years, 62 males).  

Results: Pericarditis was idiopathic or viral in 87.0% of cases, post-pericardiotomy in 9.0% of cases; Familial Mediterranean Fever was diagnosed in 2.0% of cases, and a systemic inflammatory disease in 2.0% of cases. The majority of children had fever and CRP elevation at disease onset (96% fever, 98% CRP elevation), and pericardial effusion (57%), while pericardial rub (25%) and EKG changes (42%) were detected in a smaller percentage of patients. Corticosteroid-treated patients experienced more recurrences, side effects, and disease-related hospitalization (for all p<0.05). After a median follow up of 60 months (6-360 months), 470 recurrences were recorded (median 3, range 2-25). Duration of the active disease was unpredictable in the single patient and largely variable. Overall, 97% of cases had additional recurrences. ANA testing is ongoing. Additional adverse events during follow-up included: readmission in 74% of cases and cardiac tamponade in 13% of cases (in the first attacks). None of our patients diagnosed as having an idiopathic recurrent pericarditis developed a systemic autoimmune disease; 78% children were treated with NSAIDs, 62% with steroids and in 62% cases colchicine was added. Refractory cases (17%) were treated with immunosuppressant drugs (azathioprine, methotrexate, IVIG, Plaquenil), and 7 (7%) with Anakinra.  Long term outcome was good, with no evolution in constrictive pericarditis, cardiomyopathy or systemic rheumatic diseases.

Conclusion: this is the largest ever published case-series of pediatric recurrent pericarditis. NSAIDs and colchicines remain the mainstay of the therapy while corticosteroids should be used with extreme caution, particularly in pediatric patients. In cortico-dependent cases anti-IL1 drugs proved to be very effective. The long term outcome is good.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/recurrent-pericarditis-in-children-and-adolescents-etiology-presentation-therapies-and-outcomes-in-a-multicenter-retrospective-cohort-of-100-patients/