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Lupus Carditis Volume 74, (Nº 1), 2000 Falcão et al Arq BrasUpdate Cardiol Lupus carditis volume 74, (nº 1), 2000 Lupus Carditis Creso A. Falcão, Norma Lucena, Izabel C. Alves, Ângela L. Pessoa, Emmanuelle T. Godoi Recife, PE - Brazil The kidney has usually been considered the most The kidneys are still the most studied organs in lupus, frequently affected organ in systemic lupus erythematosus, with the outcome of renal lesions traditionally considered a the heart and the pulmonary vessels, however, may also be mark of the evolution and prognosis of the patient involved. seriously involved. The diagnosis of a cardiac lesion may But it has been known for at least a century that the heart be occasionally difficult due to the common coexistence of can also develop serious lesions and may contribute in an multiple clinical problems, such as renal failure and infec- equally significant way to the morbidity and mortality of the tion, in lupus patients. Nevertheless, the specific cardiac in- illness 6-8. volvement (lupus carditis) requires suitable diagnosis be- In clinical practice and even according to the relevant cause in most of the cases immunosupressive treatment reports in the literature, it may ultimately be difficult to must be added to conventional therapy. Pericarditis, myo- differentiate the cardiac involvement due to SLE from the carditis, Libman-Sacks endocarditis, pulmonary arterial hy- one that occurs in SLE as a result of other causes 9, such as pertension and coronary artery disease are the main cardio- arterial hypertension and infectious endocarditis. It is vascular complications associated with autoimmune mani- known, however, that cardiac involvement is the third most festations in systemic lupus erythematosus. The so-called common cause of death in lupus, following infections and "lupus anticoagulant" and the anticardiolipin antibodies, renal failure 10. The aim of this report is to emphasize the which are also often called antiphospholipid antibodies, cardiac disease considered specific to SLE, which occurs have been associated different cardiac involvements in as a result of the autoimmune alterations caused by the di- systemic lupus erythematosus. sease. Cardiac involvement seems to be more easily detec- Systemic lupus erythematosus (SLE) is an autoim- ted during periods of activity of the illness 11 and is more mune disease of unknown origin characterized by inflam- customarily referred to as lupic cardiopathy or lupic carditis mation of multiple organs (kidneys, brain, heart, liver, (table I). lungs, joints, muscles, skin, etc.) with an evolution that is Since 1895, William Osler has been considered to be punctuated by remission and recurrence. The severity of the first to recognise cardiac lesions, in addition to the other the disease is variable, ranging from light and superficial complications of the multiform exudative erythema also clinical evidence, such as involvement of the skin and classified as inflammatory, such as cerebral, respiratory, arthritis, to quick fulminating forms with renal failure and gastrointestinal and renal involvement, as part of systemic serious disorders of the central nervous system 1,2. lupus 12,13. However Libman and Sacks focused attention on The name “lupus” comes from the Latin word “lupus”, a form of cardiac involvement they considered specific to which means wolf and refers to the classic erythematosus lupus. They reported post-mortem findings of a form of ulceration of the face. Early medical observations of the noninfectious endocarditis called atypical verrucous disease that date back at least seven centuries describe endocarditis in four patient with clinical data suggesting lupus as “a sickness that corrodes, bites and destroys” 3. SLE 14. Gross, from 1932 - 1940, described in detail the Systemic lupus erythematosus is the most common of the pathological findings of lupus carditis and concluded that the autoimmune systemic diseases 4 with an estimated preva- cardiac involvement of lupus was in fact a pancarditis 15,16. lence of 4 - 250 cases in 100,000 individuals in the general population, and occurs more frequently in females in the 16 As a whole, the cardiac involvement in SLE is estima- 17 - 55 year age group 5. The strongest, most characteristic ted to have a prevalence of 30 - 50% , or up to 52 - 80% 8 aspect of SLE is its autoimmune status, characterized by when pulmonary hypertension is included . Differences the development of multiple antibodies derived from the among several reports are probably due to the detection nucleus, cytoplasm and cell membranes 1,5. methods used. For example an approximate 57% frequency is found when two-dimensional echocardiography and Doppler are used 12. Patients with SLE may simultaneously Faculdade de Ciências Médicas – FESP - Universidade de Pernambuco present with more than one form of heart disease. Correspondência: Creso A. Falcão – Rua Padre Carapuceiro, 488/603 - 51020- In spite of the cardiac lesions considered specific to 280 - Recife, PE - Brazil. SLE that may presumably be due to the deposition of im- Received on 3/4/99 Accepted on 5/19/99 mune complexes in the pericardium, myocardium or vessel wall 12, and of various studies aiming to define an asso- 64 Arq Bras Cardiol Falcão et al volume 74, (nº 1), 2000 Lupus carditis Table I - Frequency of cardiac events in lupus or leaning forward), and by the presence of pericardial attrition on auscultation. The ECG evaluation shows peaked Cardiopathy Frequency (%) Method T waves and elevation of the ST segment which is not diffe- Pericarditis 24-49 Doppler-echocardiography 8 rent from that seen in other causes of pericarditis 8,10.12. The Myocarditis 40 Necropsy 16 inflammation may reach the sinoatrial node or the atrioven- Verruacous endocarditis 15-60 Necropsy 25 23 Pulmonary hypertension 14 Doppler-echocardiography 34 tricular node and provoke arrhythmias . Myocardial infarction 2 - 8 Clinic + ECG + enzymes 42 The pericardial fluid of patients with SLE frequently contains lupus erythematosus cells and low complement levels compared with that found in serum 12,17. Antinuclear ciation between lupus carditis and antiphospholipid anti- antibodies, anti-DNA antibodies, and rheumatic factor may 16 bodies 18-20, the pathogenesis of cardiac disease in SLE has also be found in the pericardial effusion . The eventual not yet been clarified. The model traditionally considered finding of antinuclear antibodies is considered virtually 10 for the pathogenesis of lupic carditis is quite similar to that pathognomonic of pericarditis associated with SLE . of other sites of lupic involvement. It is based on the idea M-mode and two-dimensional echocardiography are that a deposition of immune complexes and the activation of currently considered the main complementary methods the complement lead to acute inflammation, chronic or recur- used for the diagnosis of pericardial effusion. However, the rent in the vascular endothelium, pericardium, myocardium, absence of pericardial effusion on echocardiography endocardium, lead system and valvular leaflets, which may should not exclude the possibility of a clinically suspected 10 be supported by the common finding of immune complexes, pericarditis . complements and antinuclear antibodies in the affected tis- In the cases of pericardial constriction, computerized sues 10. It is also possible that the immune complexes found tomography (CT) and magnetic resonance imaging (MRI) in the tissues may not be pathogenic, but deposit there, are superior to echocardiography for the visualization of 10 passively, following a previous tissue lesion, in a way similar pericardial thickening and of the calcifications . However, to the immunocomplexes found in the derma - epidermis in spite of the high frequency of pericarditis and effusion, junction of the normal skin of some patients with lupus 21. cardiac tamponade and pericardial constriction rarely occur Moreover, it seems that heart disease in patients with during the evolution of SLE 8,12,17. lupus is found more commonly at autopsy than in clinical For symptomatic patients with a small effusion, treat- practice 12. The pathological findings usually observed on ment should include the use of nonsteroidal anti-inflam- necropsy of patients who died of SLE is pancarditis, affec- matory drugs, such as indomethacin with or without antima- ting the pericardium, myocardium, endocardium and coro- larial medication. For the more severe cases, or those that do nary arteries. The involvement of one layer of the heart, for not respond to the above measures, usually steroids are example the pericardium, may be predominant in one indivi- used in dosages that vary from 0.5-1mg/kg/day of predniso- dual but in another the heart may be diffusely affected. ne, according to the seriousness of the involvement. Peri- cardial drainage and pericardiectomy rarely need to be Pericarditis performed 8,10,12,21. Pericarditis is the most common form of cardiac invol- Myocarditis and cardiomyopathy vement in SLE 8,12,17. In a similar way to other forms of cardiac involvement in lupus, its prevalence is also greater in au- Myocardial disease in lupus has three separate causes: topsy samples, thus indicating that asymptomatic peri- the first and most frequent is myocarditis, acute or chronic, cardial involvement is quite common 8. Evaluating several which is the cause of a cardiomyopathy specific to lupus; clinical studies in the literature, Doherty and Siegel 16 found the second is myocardial ischemia, caused by coronary a 25.6% prevalence of pericarditis in 1,194
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