Autoimmunity and autoinflammation as the yin and yang of idiopathic recurrent acute pericarditis.

[familial mediterranean fever]

Autoimmunity and autoinflammation are generally considered as mutually exclusive mechanisms of diseases but may concur to specific syndromes . Idiopathic recurrent acute pericarditis (IRAP ) is defined as the recurrence of pericardial symptoms at any point following the prior cessation of acute pericarditis , and the latency is generally 6 weeks . Manifestations of pericarditis such as pericardial friction rub , electrocardiographic changes , and pericardial effusion are less frequent in the subsequent episodes compared to the index attack , and in some cases the only clinical sign is represented by a suggestive chest pain . Several autoimmune diseases may manifest with pericarditis which is often related to viral infections , while postviral pericarditis may in turn display a nonspecific autoimmune background . Similarly , autoinflammatory syndromes such as familial Mediterranean fever and tumor necrosis factor receptor-associated periodic syndrome are characterized by self-limiting pericardial symptoms . Corticosteroids are generally effective , thus supporting the autoimmune nature of IRAP , but dramatic results are obtained with interleukin-1 blocking agents in corticosteroid-dependent cases , pointing to a pathogenic role for the inflammasome . Based on these observations , we submit that IRAP represents a paradigmatic example of the putative coexistence of autoimmunity and autoinflammation : the main aim of this review is to critically discuss the hypothesis as well as the current understanding of this enigmatic clinical condition .

Diseases
Validation

Diseases presenting "autoimmunity" symptom

acute rheumatic fever

benign recurrent intrahepatic cholestasis

epidermolysis bullosa simplex

familial mediterranean fever

inclusion body myositis

omenn syndrome

wiskott-aldrich syndrome

This symptom has already been validated