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Autoimmune Pericarditis

Autoimmune pericarditis is inflammation of the pericardium caused by systemic autoimmune or autoinflammatory disease. It presents as acute, recurrent or chronic pericarditis, often with pericardial effusion and pleural involvement (polyserositis).

Autoimmune and autoinflammatory conditions account for about 5 to 15% of acute pericarditis. They are the commonest cause of pericardial disease in:

  • systemic lupus erythematosus (pericarditis in 20 to 30% of patients over the disease course)
  • rheumatoid arthritis
  • systemic sclerosis
  • Sjogren syndrome
  • vasculitis
  • adult onset Still disease
  • familial Mediterranean fever
  • TRAPS (tumour necrosis factor receptor associated periodic syndrome)
  • sarcoidosis
  • inflammatory bowel disease

Post-cardiac injury syndromes (post-myocardial infarction, post-pericardiotomy, post-traumatic) are immune mediated and behave in a similar way.

Recurrence occurs in 15 to 30% of idiopathic pericarditis, and more often in autoimmune and autoinflammatory disease.

Pathophysiology.

Immune complex deposition, complement activation, autoantibody mediated injury and cytokine release (particularly interleukin-1 and tumour necrosis factor) inflame the pericardium and cause exudative effusion.

In autoinflammatory disease (idiopathic recurrent pericarditis, familial Mediterranean fever, TRAPS), activation of the NLRP3 inflammasome produces excess interleukin-1, which explains the response to interleukin-1 blockade.

Chronic or repeated inflammation may lead to fibrosis, thickening and constrictive physiology.

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