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Pericardial disease
Pericardial involvement in systemic inflammatory diseases
  1. Massimo Imazio
  1. Correspondence to Dr Massimo Imazio, Cardiology Department, Maria Vittoria Hospital, Via Cibrario 72, 10141 Torino, Italy; massimo_imazio{at}

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Systemic inflammatory diseases (SIDs) are inflammatory syndromes with involvement of at least two organ systems. Classical SIDs include vasculitis, connective tissue diseases, and granulomatous diseases (box 1).1 The term ‘connective tissue disease’ is increasingly used to designate genetic conditions that affect the structural integrity of connective tissue, such as Marfan syndrome, and not only systemic inflammatory conditions. On this basis, the term ‘rheumatologic disease’ or ‘systemic autoimmune disease’ is adopted instead of ‘connective tissue disease’ in this review.

Box 1

Main systemic inflammatory diseases


  • Large vessels: Takayasu arteritis, giant cell arteritis

  • Medium sized vessels: polyarteritis nodosa, Kawasaki disease

  • Small vessels: Churg–Strauss syndrome, Wegener disease

Rheumatologic diseases (also known as connective tissue diseases)

  • Systemic lupus erythematosus

  • Rheumatoid arthritis

  • Systemic sclerosis

  • Polymyositis and dermatomyositis

  • Mixed connective tissue disease

  • Sjögren syndrome

Granulomatous diseases

  • Sarcoidosis

Autoinflammatory diseases

  • Familial Mediterranean fever

  • TNF receptor-1 associated periodic syndrome (TRAPS)

Cardiac involvement is not uncommon in SIDs, although major cardiac problems rarely present. Cardiac involvement may affect the pericardium (pericarditis and pericardial effusion), the myocardium (myocarditis, cardiomyopathy, rhythm and conduction disturbances, heart failure), coronary arteries (acute coronary syndromes, ischaemic heart disease), the endocardium (valvular disease, thrombi), and major vessels (aneurysm formation, arterial and venous thrombosis).1 2

The reported frequency of cardiac involvement is variable, depending on the applied diagnostic method and patient selection. Technological improvements in imaging and the increasing spread of diagnostic methods have revealed higher frequencies of cardiac abnormalities in patients with SIDs than in older autopsy studies.

The pericardium consists of a double layered sac that provides mechanical protection, reduces the friction between the heart and surrounding structures, and limits the distension of the heart contributing to diastolic coupling of the ventricles. Normally, this function is achieved by the presence of a small amount of pericardial fluid (25–50 ml) produced by the visceral pericardium. Infectious and non-infectious noxa are usually responsible for inflammation of the pericardial layers, with increased production of …

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  • Competing interests In compliance with EBAC/EACCME guidelines, all authors participating in Education in Heart have disclosed potential conflicts of interest that might cause a bias in the article. The author has no competing interests.

  • Provenance and peer review Commissioned; internally peer reviewed.