IL-1 beta receptor antagonist efficacy in the treatment of idiopathic recurrent pericarditis

Objective

To assess the efficacy of IL-1 beta receptor antagonist (IL-1RA) in the treatment of steroid dependent idiopathic recurrent pericarditis (RP).

Methods

Three patients (1 male, 2 females, aged between 13 and 16) were enrolled. RP was defined by "pericardial" pain and ≥1 of the following signs: fever, pericardial friction rub, electrocardiographic changes, echocardiographic evidence of pericardial effusion, elevated acute phase reactants.

Patients showed recurrent flares of pericarditis with malaise, precordial pain, tachyorthopnoea, sometimes fever. 2/3 developed a life-threatening cardiac tamponade needing pericardiotomy. None of them presented signs or symptoms consistent with a chronic rheumatic disease: antinuclear, anti-coxsackie and anti adenovirus antibodies were negative. Genetic analysis ruled out Familial Mediterranean Fever. Mean disease duration was 2 years and mean rate of recurrences was 4 yearly. Pericarditis seemed steroid dependent and poorly responsive to colchicine.

Results

IL-1RA administration was associated with a dramatic disappearance of symptoms and normalization of acute phase reactants. In all three patients IL-1RA was stopped after the achievement of a complete remission. In all cases, withdrawal was followed by a flare after a few weeks. The reinstitution of IL-1RA was associated with a new immediate response. All patients were mantained on IL-1RA treatment whilst steroids were tapered and stopped; at a mean follow-up of 4 months, none of them has experienced a disease relapse.

Conclusion

IL1-RA may be effective in the treatment of idiopathic recurrent pericarditis; moreover, this preliminary finding seems to suggest that these patients could be affected by a new, not yet identified, genetic autoinflammatory disease.