Idiopathic relapsing-remitting steroid-dependent pericarditis with transient constriction: a case report

Constrictive pericarditis is an uncommon complication of idiopathic pericarditis long thought to be irreversible, often requiring pericardiectomy to ensure recovery. Recently, transient constriction has been described in the setting of long-standing pericarditis with active inflammation.

We report the case of a 16-year-old male with an idiopathic relapsing-remitting pericarditis, refractory to non-steroidal anti-inflammatory drugs (NSAIDs), who developed constrictive physiology. Despite proper tapering, the patient developed five relapses during steroid treatment, which led to IL-1 receptor antagonist starting. After a long treatment with anakinra, constrictive physiology resolved. Disappointingly, discontinuation of interleukin-1 receptor (IL-1R) treatment was followed by a relapse of pericarditis, in the absence of signs of constriction.

Our case sustains that constrictive physiology may be a transient phenomenon, when the inflammation is still active and fibrotic transition has not been completed. When NSAIDs and steroid prove to be ineffective, IL-1R antagonist may represent a potential treatment for constrictive pericarditis, although there remains little evidence on de-escalation of IL-1R antagonist treatment in pericarditis.