Aetiology and Classification of Pericardial Diseases

(1)

Cardiology Department, Maria Vittoria Hospital and Department of Public Health and Pediatrics University of Torino, Torino, Italy

2.1 Overview and Epidemiology

The pericardium may be affected by all kinds of aetiological agents, although specific aetiologies are much more common in clinical practice and may have specific regional changes according to the prevalence of infectious diseases, especially tuberculosis (Table 2.1) [1–9].

Table 2.1

Aetiology of pericarditis in major reported published series

Aetiology	Reported frequency (%)
Idiopathic	15 % (Africa) to 80–90 % (Europe)
Infectious pericarditis
Viral (e.g. Coxsackie, EBV, CMV, HIV, Parvovirus B 19)	Largely unknown (30–50 % in Marburg experience, Germany)
Bacterial:
Tuberculosis	1–4 % (Italy, Spain, France), up to 70 % (Africa)
Purulent	<1 % (Europe) to 2–3 % (Africa)
Other infectious causes	Rare (largely unknown)
Non-infectious pericarditis
Neoplastic aetiology	5–9–35 % (in tertiary European referral centres)
Autoimmune*	2–24 %
Other infectious causes	Rare (largely unknown)

* including systemic inflammatory diseases and post-cardiac injury syndromes

The pericardial may be affected alone or be involved in a systemic disease or a localization of another pathological process affecting primarily another organ or tissue.

Tuberculosis is the major cause of pericardial diseases all over the world being the most common aetiology in countries with a high prevalence of tuberculosis, such as developing countries (Fig. 2.1) [2, 3, 6, 8].

Fig. 2.1

Prevalence of tuberculosis per 100,000 people is highest in sub-Saharan Africa, and it is also relatively high in Asia. From Wikipedia, with data from the World Health Organization (2009) Global tuberculosis control: epidemiology, strategy, financing (image in the public domain)

A high prevalence (>60 % of cases) has been reported in sub-Saharan Africa, and tuberculous pericarditis is especially associated with HIV infection in these settings [6]. On the contrary, tuberculous aetiology accounts for less than 5 % of cases in developed countries, such as Western Europe and North America, where there is a low prevalence of tuberculosis [2–4, 7]. In these countries, most cases are labelled to be “idiopathic” and are presumed to be viral in most cases (>85 %). The epidemiological background is essential to develop a rational, cost-effective, diagnostic evaluation since the aetiology search should be especially focused to exclude major aetiologies in order to avoid many diagnostic tests with a very limited diagnostic yield [2–5, 9]. Immigration may change the current aetiological spectrum in the next few years, and thus a careful consideration of these aspects as well as ethnic issues is warranted.

A simple and commonly used classification divides pericardial aetiologies into infectious and non-infectious (Table 2.2).

Table 2.2

Main aetiologies of pericardial diseases. A simple classification is to divide into infectious and non-infectious causes

Infectious causes

Viral (common): enteroviruses (coxsackie viruses, echoviruses), herpesviruses (EBV, CMV, HHV-6), adenoviruses, parvovirus B19 (possible overlap with aetiologic viral agents of myocarditis)

Bacterial: Mycobacterium tuberculosis (common, other bacterial rare), Coxiella burnetii, Borrelia burgdorferi, rarely – Pneumococcus spp., Meningococcus spp., Gonococcus spp., Streptococcus spp., Staphylococcus spp., Haemophilus spp., Chlamydia spp., Mycoplasma spp., Legionella spp., Leptospira spp., Listeria spp., Providencia stuartii

Fungal (very rare): Histoplasma spp. (more likely in immunocompetent patients), Aspergillus spp., Blastomyces spp., Candida spp. (more likely in immunocompromised host)

Parasitic (very rare): Echinococcus spp., Toxoplasma spp.

Non-infectious causes

Autoimmune (common):

Systemic autoimmune and auto-inflammatory diseases (systemic lupus erythematosus, Sjögren syndrome, rheumatoid arthritis, scleroderma), systemic vasculitides (i.e.: eosinophilic granulomatosis with polyangiitis or allergic granulomatosis, previously named Churg-Strauss syndrome, Horton disease, Takayasu disease, Behçet syndrome), sarcoidosis, familial Mediterranean fever, inflammatory bowel diseases, Still disease

Neoplastic:

Primary tumours (rare, above all pericardial mesothelioma)

Secondary metastatic tumours (common, above all lung and breast cancer, lymphoma)

Metabolic:

Uremia, myxedema, anorexia nervosa, other rare

Traumatic and Iatrogenic:

Early onset (rare):

Direct injury (penetrating thoracic injury, oesophageal perforation)

Indirect injury (non-penetrating thoracic injury, radiation injury)

Delayed onset:

Pericardial injury syndromes (common) postmyocardial infarction syndrome, postpericardiotomy syndrome, post-traumatic, including forms after iatrogenic trauma (e.g. coronary percutaneous intervention, pacemaker lead insertion and radiofrequency ablation)

Drug-related (rare):

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