Background/Purpose: Recurrent pericarditis (RP) challenges pts and clinicians in terms of long-term management. Objective of this study was to evaluate the remission rate (drug-free for at least 6 months) and disease duration of RP. Secondary objectives were to assess the “improvement rate”, defined as discontinuation of immunomodulatory therapy (either corticosteroids or IL-1 antagonist), and to characterize the sub-group of patients managed with IL-1 inhibitors.

Methods: 370 pts with idiopathic or post cardiac injury RP were studied, male 49%, median age 49 yrs. Cox proportional hazards models were used to evaluate risk factors for disease duration (time to remission) and for IL1-pathway inhibitor discontinuation.

Results: The median follow-up (from first attack to last observation) was 4.9 yrs (IQR 2.8-8.4) (2590 patients-year). Median duration of disease was 3.8 yrs (IQR 2.1-7.5). CRP higher than 1 mg/dL (81%) and pericardial effusion (81%) characterized  first attack. A median of 4 recurrences (IQR 2-7) and a median hospitalization rate of 1.1/year (IQR 0.6-1.9) was recorded. At the last follow-up, 126 pts (34%, CI 28.7-40.4%) were free of any therapy for at least six months, with a median remission duration of 2.3 yrs (IQR 1-3.7). Remission rate was approximately 7% per yr. The disease duration was shorter for patients in remission (3.1 yrs, IQR 1.6-6.2) than for those still on treatment (4.0 yrs, IQR 2.2- 7.8) (p=0.02).  In multivariate analysis, “guidelines-based therapy” at first attack (p=0.02, HR=1.85, CI 1.25-2.73), and in particular colchicine use (p=0.038, HR=1.51, CI 1.02-2.23) were associated with a shorter course, whereas steroids use had a negative impact on disease duration (p=0.003, HR=0.53, CI 0.35-0.81).

Among the 289 pts treated with any type of immunomodulators, 33% were able to suspend any treatment at follow up, while 19% could discontinue only immunomodulators (”improvement rate”). In the subgroup of 96 pts managed with anakinra, at the last follow-up, 11% were free of any treatment, 5% were in treatment only with colchicine/NSAIDs, 80% were still on anakinra whereas 3% were on low-dose steroids only. The median disease duration was 4.6 yrs (IQR 3.4.-8.8) in patients who permanently discontinued anakinra and 5.2 yrs (IQR 2.7-7.7) in those still on treatment (p=0.005). Pericardial effusion at the first attack was associated with a lower rate of anti-IL-1 discontinuation. Among those still on therapy, median duration of therapy was: NSAIDs 3.6 yrs (IQR 1.1-5.7) (86 pts); colchicine 3.7 yrs (2-5.8) (185 pts); corticosteroids 3.3 yrs (1.1-5.7) (68 pts).

Conclusion: This is the largest series of patients with RP, with the longest follow-up. The disease course was long and quite impacting in term of recurrences and hospitalizations. Guidelines adherence from the very first pericarditis episode had a favorable role in term of remission and disease duration.

Moreover, this study includes the longest follow-up of patients with RP managed with anti-IL1 therapy. The fact that only 20% were able to stop anti-IL-1 over 4.6 yrs suggests that, once initiated, this disease requires a long-term treatment.  Our data refer to difficult-to-treat patients followed in tertiary referral centers and might not be generalizable.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/duration-of-disease-and-long-term-outcomes-in-patients-with-difficult-to-treat-recurrent-pericarditis-a-chronic-condition-treated-with-nsaids-colchicine-corticosteroids-and-anti-il-1-agents/