Acute idiopathic pericarditis: current immunological theories

Abstract

Idiopathic recurrent acute pericarditis (IRAP) is a rare disease of suspected immune-mediated pathogenesis. It represents a diagnosis of exclusion. It is necessary to rule out infectious and noninfectious causes of pericardial inflammation, including systemic autoimmune and immune-related disorders, eg, Sjögren's disease, systemic lupus erythematosus. Since pericarditis may precede diagnosis of these disorders, IRAP diagnosis is often made after a long follow-up. According to the two main pathogenetic theories IRAP may represent an organ-specific autoimmune disease or an autoinflammatory disease (AInfD). The main evidence for autoimmunity in IRAP is provided by the detection of serum antiheart and antiintercalateddisk autoantibodies, and the response to anti-inflammatory or immunosuppressive therapy. The findings of familial forms and of proinflammatory cytokines in the pericardial fluid in IRAP would be in keeping with both organ-specific autoimmune disease and AInfD. In fact, AInfD are genetic disorders characterized by primary dysfunction of the innate immune system, due to mutations of genes involved in the regulation of the inflammatory response, in the absence of antigen specific T cells or autoantibodies. In AInfD there are active disease phases with raised non-cardiac specific inflammatory markers, such as C-reactive protein, as well as symptom-free intervals with possible C-reactive protein normalization. A minority of IRAP patients (6%) carry a mutation in the TNFRSF1A gene, encoding the receptor for tumor necrosis factor-alfa. This suggests that some IRAP patients may have an atypical or subclinical form of AInfD. Thus, IRAP may represent a syndrome with distinct pathogenetic mechanisms in different patients' subsets.

Idiopathic Recurrent Acute Pericarditis: A Cross Talk between Autoimmunity and Autoinflammation

“…However, the two potential mechanisms explaining IRAP, autoimmune and autoinflammatory, might be unconfirmed in a single patient. Evidence which might ascribe to autoimmunity the origin of IRAP is the presence of serum autoantibodies, while the presence of autoinflammatory gene mutations or a spontaneously occurring-remission of the pericardial inflammation might ascribe the origin of IRAP to autoinflammation [38]. In both autoimmune and autoinflammatory backgrounds we find hypercytokinemia in the pericardial fluid, familial clustering of IRAP, and a good response to empiric anti-inflammatory drugs.…”

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

“…However, the two potential mechanisms explaining IRAP, autoimmune and autoinflammatory, might be unconfirmed in a single patient. Evidence which might ascribe to autoimmunity the origin of IRAP includes the presence of serum autoantibodies, while the presence of autoinflammatory gene mutations or a spontaneously occurringremission of the pericardial inflammation might ascribe the origin of IRAP to autoinflammation [104,105]. In both autoimmune and autoinflammatory backgrounds we find hypercytokinemia in the pericardial fluid, familial clustering of IRAP, and a good response to empiric anti-inflammatory drugs.…”

Pericarditis Epistenocardica or Dressler Syndrome? An Autopsy Case

“…However, its advent of thrombolysis and widespread use of heparin have reduced the incidence of this syndrome [ 12 ]. When present, it arises 2 weeks after an MI presumably due to an autoreactive immune mechanism similar to postcardiac injury syndrome [ 10 , 13 ]. Typical symptoms include pleuritic chest pain with low-grade fever.…”