Pathogenetic mechanisms, new drugs, and old problems in idiopathic recurrent pericarditis: Comment on the article by Picco et al


Pathogenetic Mechanisms, New Drugs, and Old Problems in Idiopathic Recurrent Pericarditis: Comment on the Article by Picco et al

To the Editor:

We read with interest the recent article by Picco et al in which the authors present case reports of 3 children with recurrent pericarditis who were treated, during flares, with the interleukin-1β (IL-1β) receptor antagonist anakinra, with immediate response (1). High-dose nonsteroidal antiinflammatory drugs (NSAIDs) are recommended as the mainstay of therapy for recurrent pericarditis (aspirin 2–4 gm/day, indomethacin 75–225 mg/day, ibuprofen 1,200–3,200 mg/day) (2, 3). Although corticosteroids should be restricted in patients with pericarditis, when they are indicated, high doses have been recommended (2, 3). However, we have demonstrated that the lower doses commonly used by rheumatologists are safer and more effective (4). The results of previous studies have demonstrated that colchicine can reduce the recurrence of pericarditis (5–7); in a randomized controlled study, colchicine had reduced recurrences by half at 18 months (7). Picco and colleagues showed that idiopathic recurrent pericarditis can be successfully treated with anakinra (1), and we agree that this agent can represent a novel therapeutic tool, although its role needs to be more clearly defined. Anakinra should probably be reserved for patients who do not tolerate other therapies, and it also could be considered an alternative to corticosteroids in patients whose disease does not respond to high-dose NSAIDs plus colchicine and in the rare instances when the required dosage and duration of steroid treatment are unacceptably high. For anakinra, however, Picco et al describe dependence similar to that observed in patients taking steroids, and NSAIDs and colchicine probably remain the best option for avoiding this dependence.

As discussed by Picco and colleagues, idiopathic recurrent pericarditis shares several features with autoinflammatory diseases, such as recurrent episodes of apparently unprovoked serosal inflammation, leukocytosis, and familial occurrence (the last of which our group has described as occurring in 10% of relatives of pericarditis patients [8]). We have also found that the most frequent mutations linked to familial Mediterranean fever were absent in these patients (6). Dysregulation of IL-1 may be very important in this condition, and we agree that recurrent “idiopathic” or “autoreactive” pericarditis (2) may indeed be an as-yet unidentified autoinflammatory disease in a subset of patients.

Antonio Brucato MD*, Massimo Imazio MD†, David Spodick MD‡, Yehuda Adler MD?, * Ospedali Riuniti, Bergamo, Italy, † Ospedale Maria Vittoria, Turin, Italy, ‡ Worcester Medical Center, Worcester, MA, ? Sheba Medical Center, Tel-Hashomer, Israel.