Article Text
Statistics from Altmetric.com
- Cardiomyopathy
- acute myocarditis
- pericardial effusion
- pericardial problems
- myocarditis
- peripheral vascular disease
- endocarditis
- heart muscle disease
- heart failure
- echocardiography
- EBM
- STEMI
- stable angina
- NSTEMI
- coronary artery disease (CAD)
- education
- smoking
- antihypertensive drugs
- pericardial disease
- pericarditis
- tamponade
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.
Main systemic inflammatory diseases
Vasculitides
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 pericardial fluid as exudate. Chronic inflammation may lead to fibrosis, calcification, and organisation of pericardial fluid, with a possible evolution towards pericardial constriction. The most common types of pericardial involvement in SIDs are represented by acute/recurrent pericarditis, and asymptomatic pericardial effusions. In non-selected series with acute pericarditis,3–5 w1 w2 a SID was responsible for acute pericarditis in 2–7% of cases (table 1), and in up to 10% for recurrent pericarditis.6
General management issues of pericardial involvement in SIDs
The management approach does not differ according to the presence or absence of a SID. If the aetiological search is inconclusive and a SID is present, pericarditis/pericardial effusion are probably correlated to the SID. The definitive diagnosis of pericardial involvement in SIDs requires the finding of specific histological lesions in the pericardium or characteristic pericardial fluid features2; nevertheless, in clinical practice the diagnosis is often presumptive, especially when pericardial fluid or tissue are not available, such as in mild forms with small to moderate effusions responsive to medical treatment.3
Presentation and diagnosis
There are several possible features of pericardial involvement in SIDs: (1) pericardial involvement usually reflects the activity of the systemic disease; (2) signs and symptoms of systemic involvement may be associated; (3) pericardial effusions are more common and larger than in idiopathic/viral cases.
Symptoms and signs that may induce suspicion that a systemic autoimmune disease may be present include fatigue, fever, weight loss, joint symptoms, skin abnormalities especially appearing after exposure to sun, photosensitivity, Raynaud phenomenon, alopecia, mucocutaneous ulcers, and dryness of the eyes.6 7 The usual screening tests in this setting include antinuclear antibodies (ANA) and rheumatoid factor, an autoantibody to the Fc portion of IgG. While the presence of high titres of antibodies (≥1/640) should arouse suspicion of an autoimmune disorder, low titres of antibody (≤1:80) with no signs or symptoms of disease are generally a non-specific finding, especially in women and the elderly, and in patients with organ specific autoimmune diseases.6
While ANA have a high sensitivity in systemic lupus erythematosus (SLE), in which a negative ANA makes the probability of the disease unlikely, the low sensitivity and specificity of ANA in other connective tissue diseases makes ANA a weak test for ruling in or ruling out other rheumatologic diseases.6 w3 w4 ANA may be found in several connective tissue diseases, chronic infections, and in 5–10% of normal subjects, and can be associated with several medications.6 w5 Nevertheless, it should be recognised that rheumatologic diagnoses are not based on a single test but require a combination of symptoms, signs, and laboratory findings. Thus, consultation with an expert and a careful history and physical examination are mandatory before an extensive and ‘blind’ use of diagnostic tests.6 7
The clinical diagnosis of pericarditis is based on simple criteria: typical chest pain, pericardial friction rub (figure 1), widespread ST segment elevation (figure 2), and pericardial effusion (figure 3).3 4 7 The pericardial chest pain that results is typically fairly sudden in onset and occurs over the anterior chest. Unlike pain from myocardial ischaemia, chest pain due to pericarditis is most often sharp and pleuritic in nature, with exacerbation caused by inspiration or coughing. One of the most distinct features is the tendency for a decrease in intensity when the patient sits up and leans forward, because of reduced pressure on the parietal pericardium, particularly with inspiration. However, dull, oppressive pain or radiation of the pain to the shoulders (particularly the trapezius ridges) may occur; in such cases it is difficult to distinguish pericarditis from other causes of chest pain. Differential diagnosis should include myocardial ischaemia, pulmonary embolism, aortic dissection, gastro-oesophageal reflux disease, and musculoskeletal pain.
The initial diagnostic evaluation of a patient with a suspicion of pericarditis/pericardial effusion should include physical examination, ECG, echocardiography, and chest x-ray (figure 4). Essential blood chemistry includes markers of myocardial lesion (eg, troponins) and inflammation (eg, erythrocyte sedimentation rate, C reactive protein).2 Evidence of elevated inflammatory markers is confirmatory but it is not required for diagnosis, because negative markers can be detected at the initial stage of the disease with early presentations or after empiric anti-inflammatory therapies to control pain.4
Aetiopathogenesis
The aetiopathogenesis of pericardial involvement in SIDs is thought to be immune mediated, although concomitant infections may play a role in some cases. SIDs may be either ‘aetiologic’ or ‘permissive’ of increased susceptibility to an unrelated primary cause (eg, viral).2
Imaging tests for different diagnostic purposes
Transthoracic echocardiography (TTE) is the imaging modality most often used for the initial evaluation and follow-up of pericardial diseases due to its wide availability, portability, lack of ionising radiation, and low cost. TTE provides semiquantitative estimation of pericardial effusion (figure 3) as well as assessment of its haemodynamic importance (figure 5). However, limitations of TTE include lack of accuracy to measure pericardial thickness, as well as to detect loculated effusions, and an inability to provide an accurate estimation of the entire pericardium and surrounding chest structures, because of the limited acoustic window. Moreover, echocardiography cannot assess the nature of pericardial effusion and masses because of the relative lack of tissue contrast. The technique is also strongly operator dependent.3 7 In contrast, CT and MRI provide a larger field of view, allowing detection of loculated pericardial effusion, pericardial thickening (figures 6 and 7) and masses, as well as associated chest abnormalities.w6 w7 CT attenuation measurements and analysis of MRI signals may enable the initial characterisation of pericardial fluid better than echocardiography. On CT, generally pericardial effusions are of low density in the range of 0–20 Hounsfield Units (HU). When the pericardial fluid contains higher amounts of protein, such as in the case of bacterial infections, or when the effusions are haemorrhagic, its density may rise to 50 HU or more. Inflamed pericardium may also show contrast enhancement.7
In CT imaging of the pericardium, difficulty may be encountered in differentiating fluid from thickened pericardial tissue. MRI is superior to CT in differentiating fluid, especially highly proteinaceous exudative effusions, from thickened pericardium.w8 CT, on the other hand, may detect minimal amounts of pericardial calcium. MRI has a superior ability to characterise pericardial effusions and masses with the use of a combination of T1 weighted, T2 weighted, and gradient recalled echo cine sequences without the use of either iodinated contrast material or ionising radiation.7 w8 Moreover, real-time cine MRI may disclose exaggerated ventricular interdependence in constrictive pericarditis.7 In patients with SIDs, the indications to undertake pericardiocentesis for diagnostic purposes are the same as those recommended for the general population (cardiac tamponade, suspicion of a bacterial or neoplastic aetiology, persistence of symptomatic moderate to severe effusion without response to medical treatment and a specific diagnosis).3 5
Diagnostic criteria and suggested diagnostic tests are essentially based on expert consensus, while there is a relative lack of data on sensitivity, specificity, and diagnostic accuracy of each test.
A practical approach to the diagnosis and management of pericarditis and pericardial effusion is presented in figures 8 and 9. The recommended approach is consistent with the 2004 European Society of Cardiology guidelines,8 but goes beyond by incorporating published evidence after 2004.3
General therapeutic issues
The treatment of pericardial involvement, often asymptomatic in SIDs, requires collaboration with the rheumatologist. Nevertheless, for pericarditis related to a SID, non-steroidal anti-inflammatory drugs (NSAIDs) and corticosteroids at low doses (ie, prednisone 0.2–0.5 mg/kg per day), with the possible adjunct of colchicine, are the mainstays of medical treatment, as they are for idiopathic acute and recurrent pericarditis (table 2).3 9 Colchicine may reduce the risk of recurrences, but the effectiveness of this agent is not well established in SIDs. Use of colchicine as an adjunct to other anti-inflammatory therapies may be warranted, especially in patients who do not respond rapidly to NSAIDs and/or glucocorticoids.3 10
Pericardial drainage for therapeutic purposes is considered for cardiac tamponade, and for moderate to large symptomatic effusions unresponsive to medical treatment.3 10
Prognosis
The overall prognosis of pericardial involvement in SIDs is generally good. Pericardial effusion, usually mild, may be asymptomatic and does not have any adverse prognostic meaning. Recurrences of pericarditis may affect the quality of life and occur in 20–30% of cases, but severe complications such as cardiac tamponade and constrictive pericarditis are relatively rare complications.1 11
Pericardial involvement in specific diseases
Pericarditis in acute rheumatic fever
Pericarditis in rheumatic fever (RF) has become relatively rare in developed countries. RF is associated with a previous infection and has features of SIDs.2 Acute pericarditis is a sign of active rheumatic carditis, usually occurring at the initial episode in the first week following fever and arthritis. Pericardial rub is generally intense, and pericardial effusion is common, although cardiac tamponade is generally rare. Occasionally pericarditis may be the first sign of acute RF. On this basis, RF pericarditis without definite carditis should be suspected in the presence of fever and arthritis, usually preceding pericarditis, and serologic positivity for β-haemolytic streptococci in a young patient.2
Vasculitis
The vasculitides are defined by the presence of inflammatory leucocytes in vessel walls with reactive damage to mural structures. Loss of vessel integrity may lead to bleeding. Compromise of the lumen leads to downstream tissue ischaemia and necrosis. In general, affected vessels vary in size, type, and location in association with the specific vasculitic disorder. Vasculitis may occur as a primary process or may be secondary to another underlying disease. Classically, the vasculitic syndromes have been categorised by the predominant sizes of the involved blood vessels. The presence or absence of antineutrophil cytoplasmic antibodies is a more recent addition to the proposed classification criteria. The presence of vasculitis should be considered in patients who present with systemic symptoms in combination with evidence of single and/or multiorgan dysfunction. Although neither sensitive nor specific, common complaints and signs of vasculitis include fatigue, weakness, fever, arthralgias, abdominal pain, hypertension, renal insufficiency (with an active urine sediment containing red and white cells and occasionally red cell casts), and neurologic dysfunction. Pericardial involvement is relatively rare in large vessel vasculitis,w9–w16 while it is more common in medium to small vessel vasculitis (table 3). Pericardial involvement has been especially described in patients with Kawasaki disease,w17 w18 Churg–Strauss syndrome,w19–w21 and Wegener disease.w22–w24
Rheumatologic diseases
Pericardial involvement, especially asymptomatic, is relatively common in many rheumatologic diseases, in particular SLE, rheumatoid arthritis, systemic sclerosis, mixed connective tissue disease, and Sjögren's syndrome (table 3).
Systemic lupus erythematosus
SLE is probably the best known SID. SLE is a chronic inflammatory disease of unknown aetiology that can affect the skin, joints, kidneys, lungs, nervous system, serous membranes and/or other organs of the body.w25 Immunologic abnormalities, especially the production of a number of ANA, are a prominent feature of the disease.w26 The clinical course of SLE is variable and may be characterised by periods of remissions and relapses. Women, especially young women, are affected more frequently than men. The most common presentation pattern includes several constitutional complaints with skin, musculoskeletal, mild haematologic, and serologic involvement. However, some patients have predominantly haematologic, renal, or central nervous system manifestations. The pattern that dominates during the first few years of illness tends to prevail subsequently.w25
Cardiovascular involvement in SLE is relatively common, and all components of the heart (myocardium, pericardium, endocardium, and coronary arteries) can be affected. Accelerated atherosclerosis with coronary heart disease is a significant cause of morbidity and premature death. The greatest increase in relative risk (>50-fold greater) is among young women, who otherwise have a low risk of coronary heart disease. Venous thromboembolic complications may cause pulmonary hypertension and right heart failure. Other common types of cardiac involvement include valvular disease (most often mitral regurgitation and usually mild) and pericardial disease (usually manifesting as asymptomatic effusion or pericarditis).w27 Pericardial involvement is the most common type of echocardiographic abnormality found in SLE; usually asymptomatic, it is probably also the most frequent cause of symptomatic cardiac disease.12 Pericardial effusion occurs in >50% of patients (figure 3), and pericarditis may precede the clinical signs of the disease. Pericarditis, as with other types of serositis, most often occurs when SLE is active, and may be associated with other types of serositis (ie, pleural effusion, ascites). However, large effusions, suggestive of tamponade, and constrictive pericarditis are rare in SLE.5 11
The pericardial fluid may contain ANA, phagocytic cells containing nuclei (LE cells), low complement levels, and immune complexes similar to those seen in pleural effusions. However, in clinical practice, such findings in pericardial fluid add little to similar tests on the serum. The pericardium may reveal inflammatory lesions, usually with a predominance of mononuclear cells, but scarring may be the primary finding in healed disease.
Rheumatoid arthritis
Rheumatoid arthritis is a chronic, systemic, inflammatory disorder of unknown aetiology that primarily involves the joints. The arthritis is usually symmetrical, may be remitting, and if uncontrolled may lead to destruction of joints due to erosion of cartilage and bone leading to deformity. The disease usually progresses from the periphery to more proximal joints, and in patients who do not fully respond to treatment, may result in significant disability within 10–20 years. Many organs may be involved, particularly in patients with severe joint disease. Patients with high titres of rheumatoid factor and active extra-articular disease may develop vasculitis. Patients with rheumatoid arthritis have an increased risk of coronary artery disease and heart failure. During the course of their disease <10% of patients have pericarditis, although up to one third have pericardial effusions, usually manifesting as asymptomatic pericardial involvement.13 Most patients with symptomatic pericarditis have a positive test for rheumatoid factor in sera. Pericarditis occurs most frequently in patients with active rheumatoid disease and other extra-articular manifestations. As a result, management should be aimed at the control of the disease. Myocarditis is uncommon.13
Systemic sclerosis
Systemic sclerosis (SSc) is also known as ‘scleroderma’. The term scleroderma is used to describe the presence of thickened, hardened skin (from the Greek ‘scleros’). The disease is characterised by excessive production of collagen resulting in fibrosis of the involved organs. SSc is characterised by the skin disorder associated with internal organ involvement. SSc is subcategorised further into diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) on the basis of the extent and distribution of skin involvement. LcSSc is commonly associated with the CREST syndrome (Calcinosis, Raynaud, Esophageal dysmotility, Sclerodactily, Telangiectasia). In lcSSc cardiac involvement is less common, although the most serious complication is the possible development of pulmonary hypertension (10–15% of cases).14 w28 w29
Malaise, fatigue, arthralgias, and myalgias are common symptoms in patients with SSc. Skin involvement is a nearly universal feature of SSc. It is characterised by skin thickening and hardening of variable extent and severity. The fingers, hands and face are generally the earliest areas involved. Oedematous swelling and erythema of the skin may precede skin induration. Raynaud phenomenon (RP) is a common clinical manifestation of vascular dysfunction in SSc. RP is considered primary when it occurs alone without evidence of any associated disorder. Secondary RP refers to the presence of the disorder in association with a related illness, such as scleroderma and SLE. Patients with symptomatic cardiac involvement due to SSc have a poor prognosis, with 2 and 5 year mortality rates of approximately 60% and 75%, respectively. Most commonly, cardiac complications are secondary to systemic or pulmonary hypertension, but primary cardiac involvement also occurs. The manifestations of primary cardiac involvement in SSc include pericarditis, pericardial effusion, myocardial fibrosis, heart failure, myocarditis associated with myositis, conduction disturbances, and arrhythmias. Symptomatic pericarditis occurs in 7–20% of patients with SSc, but pathologic evidence of pericardial involvement is observed in 70–80% at autopsy. Pericardial effusions may be small or large, and can develop rapidly, leading also to cardiac tamponade.14
Polymyositis and dermatomyositis
Dermatomyositis (DM) and polymyositis (PM) are classified as idiopathic inflammatory myopathies. DM and PM share the clinical feature of muscle weakness; DM is associated with a variety of characteristic skin manifestations.w30
Pericardial involvement is less common than for other connective tissue diseases (<10%) and may be manifested by acute pericarditis, pericardial effusion, and cardiac tamponade. Additional cardiac manifestations include arrhythmias and conduction disturbances.w30 w31
Mixed connective tissue disease
Mixed connective tissue disease (MCTD) is defined as a generalised connective tissue disorder characterised by the presence of high titre anti-U1 ribonucleoprotein antibodies in combination with clinical features commonly seen in SLE, SSc, PM, and rheumatoid arthritis. A diagnosis of MCTD usually requires several years before enough overlapping features have appeared, generally sequentially over time. Thus, in its early stages, MCTD is often referred to as an undifferentiated connective tissue disease. The early clinical features are non-specific and may consist of general malaise, arthralgias, myalgias, and low grade fever. A specific clue that these symptoms are caused by a connective tissue disease is the discovery of a positive ANA in association with the Raynaud phenomenon. All three layers of the heart may be involved in MCTD. A major cause of death in MCTD is pulmonary hypertension. This complication is caused by a bland intimal proliferation and medial hypertrophy of pulmonary arterioles. Pericarditis is a relatively common clinical manifestation of cardiac involvement, being reported in 10–30% of patients; pericardial tamponade is rare. Involvement of the myocardium is increasingly recognised.15
Sjögren syndrome
Sjögren's syndrome (SS) is a chronic autoimmune disorder characterised by lymphocytic inflammatory infiltration of the lacrimal and salivary glands with diminished function. SS occurs in a primary form not associated with other diseases and in a secondary form that complicates other rheumatic conditions. The most common disease associated with secondary SS is rheumatoid arthritis. In primary or secondary SS, decreased exocrine gland function leads to the sicca complex, a combination of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia).
In addition, a variety of other disease manifestations can occur in SS. Patients with primary SS often possess antibodies to the Ro/SSA or La/SSB antigens. Patients with antibodies to these extractable nuclear antigens are more likely to have adenopathy, peripheral neuropathy, cutaneous vasculitis, Raynaud phenomenon, and annular erythema (the lesions are polycyclic and maculopapular in nature, usually on the face, upper extremities, and trunk).
Cardiovascular involvement occurs in less than one third of cases, and is often asymptomatic. Pericarditis is the most common form of such involvement (figure 5).16
Behçet's disease
Behçet's disease is a relapsing inflammatory disease with recurrent aphthous stomatitis, genital ulcerations, and uveitis as the most typical manifestations. Clinical manifestations of Behçet's disease are due to vasculitis that may involve blood vessels of all sizes—small, medium, and large—on both the arterial and venous sides of the circulation. Large vessel vascular involvement occurs in approximately one third of patients with Behçet's disease. In these patients, perivascular and endovascular inflammation may lead to haemorrhage, stenosis, aneurysm formation, thrombus formation in both arteries and veins, and varices. Atherosclerosis does not appear to occur at an accelerated rate in Behçet's disease, as has been observed in autoimmune diseases such as SLE. Symptomatic cardiac disease is uncommon in Behçet's disease. Abnormalities that can occur include pericarditis, myocarditis, coronary arteritis, atrial septal aneurysm, conduction system disturbances, ventricular arrhythmias, endocarditis, endomyocardial fibrosis, mitral valve prolapse, and valvular insufficiency.17
Granulomatous diseases
Sarcoidosis is the most important disease of the group, presenting as a multisystem granulomatous disorder of unknown aetiology, typically affecting young adults. Lung involvement occurs in over 90% of patients. The ‘classic’ chest radiograph reveals bilateral hilar adenopathy. This finding, however, may be absent or, if present, may occur in combination with parenchymal opacities. Sarcoidosis can involve all organ systems to a varying extent and degree. The most prominent sites of extrapulmonary disease include the skin, eyes, reticuloendothelial system, musculoskeletal system, exocrine glands, heart, kidney, and central nervous system.w32 Clinical manifestations of cardiac sarcoidosis depend upon the location and extent of granulomatous inflammation. Cardiac involvement (about 25% of patients) may precede, follow, or occur concurrently with involvement of the lungs or other organs. Clinicians should consider the possibility of sarcoid heart disease in the evaluation of an otherwise healthy young or middle aged person with cardiac symptoms, or in a patient with known sarcoidosis who develops arrhythmias, conduction disease, or heart failure. Mild to moderate asymptomatic pericardial effusions are commonly detected. However, symptomatic pericarditis is rare during the course of the disease.18
Autoinflammatory diseases
Autoinflammatory diseases are characterised by a primary dysfunction of the innate immune system, mostly caused by mutations of genes involved in the regulation or activation of the inflammatory response, without any apparent involvement of antigen specific T cells or significant production of autoantibodies. These disorders usually manifest in the paediatric population, although a limited number of patients experience disease onset during adulthood. The most common autoinflammatory syndromes include familial Mediterranean fever (FMF) and tumour necrosis factor receptor-1 associated periodic syndrome (TRAPS). Recurrent pericarditis is a common feature of both conditions, but it rarely occurs alone. Familial clustering may represent a clue for investigating mutations related to autoinflammatory diseases in these patients.19 20
FMF is an autosomal recessive disease largely restricted to certain ethnic groups and presenting with recurrent febrile serositis attacks. FMF has been described primarily in several ethnic groups originating in the Mediterranean littoral (Sephardic Jews, Armenians, Turks, North Africans, Arabs, and less commonly Greeks and Italians).19 w33
TRAPS is the most common autosomal dominant autoinflammatory disorder and is caused by mutations in the TNFRSF1A gene encoding the 55 kD receptor for tumour necrosis factor α (TNFα). Recurrent fever attacks with polyserositis and without alternative causes typically last at least 5 days and often continue for >2 weeks. Physical and emotional stress may trigger attacks. Particular hallmarks include the protracted duration of attacks, rash, eye and periorbital involvement in more than 80% of patients, and the nearly uniform presence of focal myalgias. Family history is commonly positive, although some carriers of TRAPS mutations may be entirely asymptomatic. The diagnosis of TRAPS is confirmed by genetic testing for common mutations in the TNFR1 gene. In addition, patients affected with recurrent pericarditis as the only clinical manifestation of TRAPS have been recently described.20 w34
Conclusions
Pericardial involvement is relatively common in SIDs, especially in rheumatologic disorders. Such involvement is presumed to be related to an autoimmune pathogenesis in most cases, although a concurrent infectious cause (especially viral) cannot be excluded in some cases. The treatment of pericardial involvement, often asymptomatic in SIDs, is essentially based on empiric anti-inflammatory therapy together with the management of the underlying systemic disease. The course is usually benign with possible recurrences, but rarely with serious complications such as cardiac tamponade or constrictive pericarditis.21
Systemic inflammatory diseases and pericardial involvement: key points
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.
Cardiac and pericardial involvement is not uncommon in SIDs, although major cardiac problems are rarely presenting manifestations.
The most common types of pericardial involvement in SIDs are represented by acute or recurrent pericarditis, and pericardial effusions.
The treatment of pericardial involvement, often asymptomatic, is essentially that of the underlying systemic disease.
Cardiac tamponade and constrictive pericarditis are possible but rare complications, while recurrent pericarditis is the most common complication.
Diagnostic criteria for pericarditis
Acute pericarditis (at least two of four should be present for the diagnosis):
Typical chest pain
Pericardial friction rub
Widespread ST segment elevation or PR depression
New or worsening pericardial effusion
Recurrent pericarditis. Recurrent pain and one or more of the following signs:
Fever
Pericardial friction rub
ECG changes
New or worsening pericardial effusion
Elevated markers of inflammation (ie, erythrocyte sedimentation rate, C reactive protein)
Vasculitis and pericardial involvement
The vasculitides are defined by the presence of inflammatory leucocytes in vessel walls with reactive damage to mural structures.
Classically, the vasculitic syndromes have been categorised by the predominant sizes of the involved blood vessels.
The presence of vasculitis should be considered in patients who present with systemic symptoms in combination with evidence of single and/or multiorgan dysfunction.
Rheumatologic diseases and pericardial involvement
Pericardial involvement (pericarditis, pericardial effusion) is common and generally benign in systemic lupus erythematosus (SLE); often it is asymptomatic and may also precede other SLE manifestations.
Pericardial involvement in rheumatoid arthritis is more commonly asymptomatic with pericardial effusion in one third of patients. Symptomatic pericarditis is three times less common than asymptomatic pericardial effusion.
Pericardial involvement, especially asymptomatic as pericardial effusion, is frequent in systemic sclerosis (>60%) and mixed connective tissue disease (30%).
Acute and recurrent pericarditis is the most common form of cardiac involvement in Sjögren syndrome.
Autoinflammatory diseases and pericardial involvement
An emerging cause of pericarditis, especially recurrent, is represented by autoinflammatory diseases.
Autoinflammatory diseases are characterised by a primary dysfunction of the innate immune system mostly caused by mutations of genes involved in the regulation or activation of the inflammatory response.
The most common forms include familial Mediterranean fever (FMF) and the tumour necrosis factor receptor-1 associated periodic syndrome (TRAPS).
FMF is an autosomal recessive disease largely restricted to certain ethnic groups and presenting with recurrent febrile serositis attacks.
TRAPS is the most common autosomal dominant autoinflammatory disorder; particular hallmarks include the protracted duration of attacks, polyserositis, rash, eye and periorbital involvement in more than 80% of patients, and the nearly uniform presence of focal myalgias.
You can get CPD/CME credits for Education in Heart
Education in Heart articles are accredited by both the UK Royal College of Physicians (London) and the European Board for Accreditation in Cardiology—you need to answer the accompanying multiple choice questions (MCQs). To access the questions, click on BMJ Learning: Take this module on BMJ Learning from the content box at the top right and bottom left of the online article. For more information please go to: http://heart.bmj.com/misc/education.dtl
▸ RCP credits: Log your activity in your CPD diary online (http://www.rcplondon.ac.uk/members/CPDdiary/index.asp)—pass mark is 80%.
▸ EBAC credits: Print out and retain the BMJ Learning certificate once you have completed the MCQs—pass mark is 60%. EBAC/ EACCME Credits can now be converted to AMA PRA Category 1 CME Credits and are recognised by all National Accreditation Authorities in Europe (http://www.ebac-cme.org/newsite/?hit=men02).
Please note: The MCQs are hosted on BMJ Learning—the best available learning website for medical professionals from the BMJ Group. If prompted, subscribers must sign into Heart with their journal's username and password. All users must also complete a one-time registration on BMJ Learning and subsequently log in (with a BMJ Learning username and password) on every visit.
References
- ↵Updated review on all types of cardiac involvement in systemic inflammatory diseases.
- ↵Comprehensive review on pericardial involvement in vasculitides and connective tissue diseases.
- ↵Most recent review on the management of pericardial diseases with a focus on controversial aspects.
- ↵First prospective cohort study on the diagnostic, therapeutic and prognostic role of C reactive protein in acute pericarditis.
- ↵
- ↵Prospective cohort study on the prevalence and clinical significance of antinuclear antibodies in recurrent pericarditis.
- ↵Most recent review on the aetiological search in acute and recurrent pericarditis.
- ↵European guidelines on the management of pericardial diseases.
- ↵
- ↵Most recent review on the medical treatment of pericardial diseases; in two parts.
- ↵
- ↵Review of cardiac involvement in systemic lupus erythematosus.
- ↵Review of cardiac involvement in rheumatoid arthritis.
- ↵
- ↵
- ↵
- ↵
- ↵
- ↵
- ↵
- ↵
Footnotes
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.