The major manifestations are cardiac and cutaneous findings. In post-mortem studies, significant atherosclerosis was observed in >50% of deceased SLE patients regardless of the actual cause of death [53]. Cardiac damage may extend beyond the conduction system. There is no direct evidence that treatment with corticosteroids or cytotoxic therapy can prevent valvular damage; however, the decline in prevalence of Libman–Sacks lesions at autopsy following the introduction of corticosteroids supports a possible indirect beneficial role. Again, there is no evidence from clinical trials to support this proposal. Detection of CHB in the fetuses most commonly occurs in utero between 17 and 24 weeks of gestation [3]. Congenital heart block is characterized by interference with the transfer of the electrical nerve impulses (conduction) that regulate the normal, rhythmic, pumping action of the heart muscle (heart block). Please check for further notifications by email. In a decision analysis model, aspirin intake in 40-yr-old lupus patients was estimated to gain 3 months of quality-adjusted survival in APA-negative and 11 months in aPL-positive individuals [75]. Antibodies cross the placenta and damage different parts of the fetus’ body. It appears equally often in males and females.Heart block usually develops between 18 and 30 weeks of pregnancy. The pericardium can be involved by acute and chronic inflammatory changes; granular deposition of immunoglobulin and C3, demonstrated by direct immunofluorescence, support the role of immune complexes in the development of pericarditis. The most serious symptom is congenital heart block, which causes a slow heartbeat. Circulation. Lupus anticoagulant (LA) and anti-β2GPI are the risk factors for myocardial infarction in the study of SLE patients cohorts [65, 66]. Moreover, corticosteroids therapy seems to increase the serum concentration of cholesterol, lipoproteins and triglyceride, whereas hydroxychloroquine seems to reduce them in SLE patients. Immunol Cell Biol. Neuropsychological Phenotypes of Pediatric Anti-Myelin Oligodendrocyte Glycoprotein Associated Disorders: A Case Series. The heart in systemic lupus erythematosus, Manifestations cardiques du lupus erythemateaux aigu dissemine, Cardiac abnormalities in systemic lupus erythematosus: a prospective M-mode, cross-sectional and Doppler echocardiographic study, The heart in systemic lupus erythemathosus and the changes induced in it by corticosteroid therapy: a study of 36 necropsy patients, Possible association between anti Ro antibodies and myocarditis or cardiac conduction defects in adults with systemic lupus erythemathosus, Systemic lupus erythematosus presenting as an isolated congestive heart failure, Cardiac magnetic resonance imaging abnormalities in systemic lupus erythematosus: a preliminary report, Marked improvement of severe cardiac dysfunction after one course of intravenous immunoglobulin in a patient with systemic lupus erythematosus, Giant cell myocarditis: a rare cardiovascular manifestation in a patient with systemic lupus erythematosus, A hitherto undescribed form of valvular and mural endocarditis, Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients, Heart valve involvement (Libman–Sacks endocarditis) in the antiphospholipid syndrome, Evaluation of cardiac abnormality and embolic sources in primary antiphospholipid syndrome by transesophageal echocardiography, Assessment of cardiac structure and left atrial appendage functions in primary antiphospholipid syndrome: a transesophageal echocardiographic study, Close association between valvular disease and central nervous system manifestations in antiphospholipid syndrome, Accelerated atherosclerosis in systemic lupus erythematosus: implications for patient management. Correspondence to: Angela Tincani, MD, Reumatologia e Immunologia Clinica, Ospedale Civile, Piazza Spedali Civili 1, 25125 Brescia, Italy. Valve abnormalities associated with aPL are similar to those reported in SLE, varying from minimal thickening and/or vegetations to severe valve distortion and dysfunction. Heart involvement in neonatal lupus. All three layers of the heart—pericardium, myocardium and endocardium—can be involved by lupus; this section will focus on pericarditis and myocarditis because heart valve abnormalities are common lesions in either SLE and APS and will be discussed in next section. Cardiovascular disease in lupus patients: should all patients be treated with statins and aspirin? Neonatal lupus (NL) is an autoimmune disease that is passively transferred from the mother to the fetus. In histological studies of human carotid samples, β2GPI was shown to co-localize with T CD4+ lymphocytes in the subendothelial region of atherosclerotic plaques, supporting a possible role of antibodies in the disease progression [59]. Atherosclerosis treatment strategies in SLE and SLE-associated secondary APS include an aggressive control of all traditional risk factors including hyperlipidaemia, hypertension, smoking, obesity and diabetes mellitus, which should be performed by using both drug treatment and changes in lifestyle [73]. Patients with active lupus, especially children, have low high-density lipoprotein-C (HDL-C) and elevated very-low-density lipoprotein-C (VLDL-C) and triglyceride levels. Fetal/neonatal disease is independent of maternal disease: in fact, mothers may have SLE, Sjogren syndrome or other autoimmune symptoms, or may be entirely asymptomatic [3]. Heart block typically begins in utero during the second or third trimester. Springer Semin Immunopathol. Some reports demonstrate an association between anti-SSA/Ro antibodies and myocarditis [33]. Acquired congenital heart block. Congenital heart block is estimated to occur in 1% to 2% of babies born to mothers with anti-Ro/SSA and anti-La/SSB autoantibodies, which cross the placenta and can cause neonatal lupus … Congenital heart block is characterized by an interference with the transfer of the heart beat from the top to the middle (conduction system) that controls the rate that the heart be… Neonatal lupus syndromes are caused by maternal antibodies targeting proteins displayed on apoptotic blebs. The early diagnosis of the CHB and its potential complications (pericardial effusion, myocarditis) usually can avoid the deterioration of the fetal cardiac function. Description of 214 cases of autoimmune congenital heart block: Results of the French neonatal lupus syndrome. Pericardial involvement appears more frequently at SLE onset or during SLE relapses, although it can occur at any time of the disease [26]. Intravenous bolus of corticosteroid is necessary in more severe cases or if tamponade is present, while in patients with recurring pericarditis, chronic suppression with methotrexate, azathioprine or mycophenolate mofetil may be effective. [40] reviewed echocardiographic studies of primary APS patients: the four largest transthoracic echocardiography (TTE) studies reported 32–38% prevalence of valve lesions that most frequently involved left-sided valves, mitral more commonly, followed by aortic (whereas Libman–Sacks involves the tricuspid valve most often). Complete heart block (CHB) is the most serious manifestation of the neonatal lupus syndrome (NLS), a congenital syndrome in which maternal IgG anti-Ro/SS-A autoantibodies cross the placenta and injure an otherwise normally developing heart [2]. E-mail: Search for other works by this author on: Cardiac imaging techniques in systemic autoimmune diseases, A major histocompatibility complex class I-like Fc receptor cloned from human placenta: possible role in transfer of immunoglobulin G from mother to the fetus, Autoimmune associated congenital heart block: mortality, morbidity, and recurrence rates obtained from a national neonatal lupus registry, Neonatal lupus: review of proposed pathogenesis and clinical data from the US-based research registry for neonatal lupus, Monozygotic twins discordant for congenital complete heart block, Comparison of treatment with fluorinated glucocorticoids to the natural history of autoantibodies-associated congenital heart block: retrospective review of the research registry for neonatal lupus, Spectrum and progression of conduction abnormalities in infants born to mothers with anti-SSA/Ro-SSB/La antibodies, Isolated congenital complete heart block: longterm outcome of children and immunogenetic study, Congenital complete atrioventricular block, Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela, Maternal anti-Ro and anti-La antibody associated endocardial fibroelastosis, Risk of congenital complete heart block in newborns of mothers with anti-Ro/SSA antibodies detected by counterimmunoelectrophoresis: a prospective study of 100 women, The rate of recurrence of isolated congenital heart block: a population based study, Pulsed Doppler echocardiographic assessment of the fetal PR interval, Transplacental fetal treatment improves the outcome of prenatally complete atrioventricular block without structural heart disease, Neurotoxic effects of fluorinated glucocorticoids preparations on the developing mouse brain: role of preservatives, Two years infant neurodevelopment outcome after single or multiple antenatal courses of corticosteroidi to prevent complications of prematurity, Normal neuropsycological development of children with congenital heart block exposed or not, Questions about dexamethasone use for the prevention of anti-SSA related congenital heart block, Signs of first degree heart block occur in one-third of fetuses of pregnant women with anti-SSA/Ro 52 Kd antibodies, Outcome of pregnancy in patients with anti-SSA/Ro antibodies: a study of 165 pregnancies, with special focus on electrocardiografic variations in the children and comparison with a control group, Anti-SSA/Ro antibodies and the heart: more than complete congenital heart block? CAD is described with a prevalence ranging from 6 to 10%, and, in SLE patients, the risk of developing any CAD is 4–8 times higher than in controls [47, 48, 50, 51]. DISCUSSION. (See "Neonatal lupus: Epidemiology, pathogenesis, clinical manifestations, and diagnosis", section on 'Fetal surveillance for heart block'.) CHB, and 5-fold with neonatal lupus. It is relevant that oral feeding of the animals with human or bovine β2GPI was effective in reducing atherosclerosis as compared with control fed animals [63]. These alterations were known as Libman–Sacks endocarditis, a verrucous endocarditis of valve leaflets, papillary muscles and the mural endocardium, originally described in SLE patients [38]; later both clinical observations and experimental data showed a close linkage between aPL and cardiac valvulopathy and documented the responsibilities of antibodies in the valvulopathy genesis. The transfer of maternal Ro/SSA autoantibodies across the placenta can cause a range of transient and permanent conditions in a small proportion of exposed infants. However, up to now, endomyocardial biopsy remains the technique of choice in diagnosing myocarditis even if the procedure is invasive and subject to sampling error. Recently, other non-invasive investigations such as magnetic resonance, are employed for diagnosing myocardial involvement in SLE: T2 values sensitively indicated myocardial relaxation abnormalities, even at preclinical stage [35]. Complete heart block (CHB) is the most serious manifestation of the neonatal lupus syndrome (NLS), a congenital syndrome in which maternal IgG anti-Ro/SS-A autoantibodies cross the placenta and injure an otherwise normally developing heart [2]. 8600 Rockville Pike Large echo series have found frequencies of global hypokinesis between 5 and 20%. [11] have reported an endocardial fibroelastosis associated with CHB in 13 children born to anti-SSA/Ro or anti-SSB/La positive mothers. What influences patients’ opinion of remission and low disease activity in psoriatic arthritis? In addition to the classical complete and incomplete CHB, a high frequency of transient fetal first degree CHB has been recently reported, that in most of the cases is spontaneously reverted before or shortly after birth [22]. 2015;11(5):301-312. Recently, an association of SLE and giant cell myocarditis has been reported. For full access to this pdf, sign in to an existing account, or purchase an annual subscription. These observations support a possible role of autoimmunity in the genesis of atherosclerosis that may have clinical or subclinical features. CHB carries a significant morbidity and mortality (15–30%) most often in utero or in the first few months of life: in fact, 67% of all the recognized cases require a pacemaker insertion before reaching adulthood [3]. Gargouri L, Safi F, Maalej B, Mallek S, Turki F, Majdoub I, Akrout M, Abid D, Kamoun S, Mahfoudh A. Zuppa AA, Riccardi R, Frezza S, et al Neonatal lupus: follow-up in infants with anti-SSA/Ro antibodies and review of the literature. Veres and others [64] showed correlation between serum levels of aCL and anti-β2GPI antibodies and the incidence and severity of acute coronary syndrome, MI and stroke. When congenital heart block occurs in isolation, the majority of cases are associated with neonatal lupus and autoimmune antibodies against Ro/SSA and La/SSB antigens. Neonatal lupus syndromes are caused by maternal antibodies targeting proteins displayed on apoptotic blebs. Prophylactic antithrombotic therapy for patients with systemic lupus erythematosus with or without antiphospholipid antibodies: do the benefits outweigh the risks? Echocardiography represents the standard method to investigate pericardial abnormalities and is able to demonstrate mild effusion or thickening of pericardial layers, therefore, should be performed periodically in SLE patients. The more serious symptom of neonatal lupus is injury to the heart's electrical system, its valves or muscle. Methods: A survey was designed by the organizing committee of the 9th International Conference of Reproduction, Pregnancy and Rheumatic Diseases. Risk of heart block is 2%, risk of neonatal lupus is 5%.) Pericarditis is the most studied cardiovascular manifestation of SLE, although often not evident clinically, and it is included in the American College of Rheumatology (ACR) classification criteria for SLE. 1991;11(3):109-12. doi: 10.1007/BF00304497. CHB affects an estimated 1 i… Heart valve abnormalities (vegetations and/or thickening) are the most frequent cardiac manifestations of APS. Prophylactic therapy include anti-platelet and, in APS cases, anti-coagulant agents, as well as statins, folic acid, B vitamins and, as described above, possibly hydroxycloroquine (HCQ) that exerts evident anti-atherogenic properties. 2008 Oct;154(1):30-7. doi: 10.1111/j.1365-2249.2008.03732.x. The addition of immunosuppressant such as azathioprine, cyclophosphamide or intravenous immunoglobulines (IVIG) may be helpful in the treatment of myocarditis [36]. 2015;11(5):301-312. Autoimmun Rev … Congenital heart block in neonatal lupus erythematosus. Antibodies to amino acid 200-239 (p200) of Ro52 as serological markers for the risk of developing congenital heart block. These cross the placenta and are available to the fetus in an active form [16], inhibiting the immune process in the fetal heart. Privacy, Help As described above, the role of corticosteroids in lupus-associated atherogenesis is rather controversial, as these agents may themselves be directly atherogenic, but they may also indirectly prevent premature atherosclerosis by controlling disease activity [48, 49]. Soltész et al. 2013;50(5):483-488. According to different reports, traditional risk factors were not different in APS and in the general population [48, 49]. Co-existent pleurisy, effusion or both are common [30]. In the past, cardiac manifestations were severe, often leading to death and they were frequently found in post-mortem examinations. The incidence of CHB in an offspring of a mother with anti-SSA/Ro antibodies is about 2%, while, if the mother already had a first affected child, the risk of CHB in a subsequent pregnancy rises to 18% [3, 12, 13]. Passively acquired autoimmunity and the maternal fetal dyad in systemic lupus erythematosus. When imaging techniques show in utero the presence of incomplete CHB, the suggested treatment of mothers is based on fluorinated steroids (dexametasone or betametasone). In the general population, the incidence varies between 1 in 15,000 to 1 in 22,000 live births. In SLE patients, the role of traditional and non-traditional risk factors for atherosclerosis is still debated. Oxford University Press is a department of the University of Oxford. Lindop R, Arentz G, Thurgood LA, Reed JH, Jackson MW, Gordon TP. FOIA Other non-traditional risk factors associated with the autoimmune-inflammatory pathogenesis of the disease or with immunosuppressive therapy must also be taken into account: among these SLE-related risk factors, besides cumulative dosage and/or length of corticosteroids therapy, disease duration, high score of activity or damage could contribute to the development of atherosclerotic plaque [26]. However, also segmental areas of hypokinesis can be indicative of the disease [34]. Congenital heart block is when the electrical signals controlling a baby's heart rate are disrupted and the heart beats more slowly than normal. … There are no typical findings on ECG, and cardiac enzymes may be normal. Complications of pericarditis, such as cardiac tamponade, constrictive pericarditis and purulent pericarditis are rare, and invasive procedures such as pericardiocentesis or pericardial window are rarely needed. Asymptomatic pericardial effusion is clearly more common than clinical pericarditis: in fact, 40% of unselected patients with SLE have pericardial effusion, detected using echocardiography. NLE and isolated fetal heart block together compose the neonatal lupus syndrome2. The reported prevalence of pericardial abnormalities, detected by echocardiographic studies, ranges from 11 to 54% [27]: this variability is partially attributable to the methods used to document pericardial disease and whether symptomatic or asymptomatic cases are included. Several autoantibodies, such as anti-phospholipid antibodies (aPL), anti-SSA/Ro antibodies and anti-endothelial cells antibodies, can mediate cardiac damage. Background - Neonatal lupus erythematosus is an uncommon acquired autoimmune disease caused by transplacental passage of maternal antibodies SSA/Ro, SSB/La or U1 ribonucleoproteins. XXIII. Gargouri L, Safi F, Maalej B, Mallek S, Turki F, Majdoub I, Akrout M, Abid D, Kamoun S, Mahfoudh A. Strandberg L, Winqvist O, Sonesson SE, Mohseni S, Salomonsson S, Bremme K, Buyon JP, Julkunen H, Wahren-Herlenius M. Clin Exp Immunol. Please enable it to take advantage of the complete set of features! Efficacy of the therapy can be assessed by serial echocardiographic studies or right ventricular endomyocardial biopsies. 1989 Aug;84(2):627-34. doi: 10.1172/JCI114208. More recently, some novel risk factors for atherosclerosis have been proposed and reviewed [58]; they include inflammatory markers (C-reactive protein, fibrinogen, interleukin-6), co-stimulatory molecules (CD40/CD40L), adhesion molecules, aPL including anti-cardiolipin (aCL) and anti-β2 glycoprotein I (anti-β2GPI); anti-oxidized low-density lipoprotein (anti-oxLDL), anti-oxidized palmitoyl arachidonoyl phosphocholine (anti-oxPAPC) and anti-hsp antibodies, homocysteine, lipoprotein(a) and HDL. The incidence of complete (third degree) congenital heart block is one in approximately 20,000 to 25,000 live births. 2012 Mar;90(3):304-9. doi: 10.1038/icb.2011.108. Antibodies-SSA/Ro and anti-SSB/La antigens play a major pathogenic role in affecting the heart conduction tissue leading to the electrocardiographic abnormalities of the neonatal lupus syndrome and have been closely associated with endocardial fibroelastosis. The clinical spectrum of autoimmune congenital heart block. Prevention and treatment information (HHS), The neonatal lupus syndromes, which comprise transient hematologic and cutaneous disorders as well as the permanent manifestation of heart block, are considered to result from injury by passively acquired maternal autoantibodies. Comparison of enzyme-linked immunosorbent assay and immunoblot for measurement of anti-SS-A/Ro and anti-SS-B/La antibodies. The SS-A/Ro, SS-B/La, and RNP complex is a subgroup of antinuclear antibodies located within the nucleus of the cytoplasm and commonly found in patients with autoimmune disease. A fetus can go from having a normal sinus rhythm to complete & irreversible heart block & death in seven or fewer days, Dr. Buyon said. Description of 214 cases of autoimmune congenital heart block: results of the French neonatal lupus syndrome. Fetal Pulsed Doppler echocardiograms are usually performed weekly from 18 to 24 weeks gestation, with the American Heart … (ABSTRACT TRUNCATED AT 250 WORDS), National Library of Medicine The most serious complication … Neonatal lupus erythematosus with congenital heart block in twins. In contrast, the association of aPL and subclinical atherosclerosis features is still debated since it is supported by some works [67, 68] and denied by others [69, 70]. Your comment will be reviewed and published at the journal's discretion. [42] demonstrated cardiac involvement in 84% of primary APS patients and mitral regurgitation in 77.4%; interestingly, valve lesions were present in all stroke patients, confirming that the presence of cardiac valves pathology may be considered a risk factor for epilepsy, stroke and other CNS involvements, particularly in patients with primary APS [43]. Autoantibodies to SS-A/Ro and SS-B/La are mainly found in the sera of patients with Sjogren¨ syndrome and patients with systemic lupus … 1. Statins: immunomodulators for autoimmune rheumatic disease? [71] reported abnormal flow-mediated vasodilatation of the brachial artery and increased ccIMT in 46 patients with primary APS. The clinical detection of myocarditis ranges from 3 to 15%, although it appears to be much more common in autopsy studies (mainly done in the 1950s and 60s), suggesting the largely subclinical nature of lupus-associated myocarditis [26, 30]. The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices Despite the absence of official guidelines, many physicians with a focus on … It furthers the University's objective of excellence in research, scholarship, and education by publishing worldwide, This PDF is available to Subscribers Only. There are three different types of heart block: Varying degrees of heart block can occur (1st and 2nd degree) with 3rd degree being the most severe (complete heart block). The clinical edge of this phenomenon is coronary artery diseases (CAD) (myocardial infarction, angina, sudden death), while early endothelial dysfunction, abnormalities of circulation or atherosclerotic plaques, detected by different imaging techniques, identify the subclinical atherosclerosis expression. Etiological and Clinical Profile of Acute Nonbacterial Encephalitis in Children: A Single-Center Prospective Study. Complete heart block (CHB) is the most serious manifestation of the neonatal lupus syndrome (NLS), a congenital syndrome in which maternal IgG anti-Ro/SS-A autoantibodies cross the placenta and injure an otherwise normally developing heart . Non-organ-specific autoantibodies have been implicated in immune complex formation and deposition as the initial triggers for inflammatory processes responsible for Libman–Sacks verrucous endocarditis, myocarditis and pericarditis. Mothers frequently are healthy and unaware of their autoantibody status. In young women with SLE, the risk of MI is increased 50-fold [54]. Aspirin has been used for a long time to prevent CAD in the general population. The heart is frequently involved in SLE: very sensitive methods of cardiovascular investigation have found the prevalence of cardiac involvement to be >50% [25]. Some studies have shown that traditional cardiovascular risk factors are also more predictive in SLE patients than in age- and sex-matched healthy subjects [56]; particularly, older age at diagnosis, hypercholesterolaemia and hypertension were the three most common predictors of CAD [57]. Despite clinical similarities, lupus myocarditis and giant cell myocarditis are histologically distinct entities, and the latter has a much more unfavourable prognosis [37]. Consequence of autoantibodies damage has been reported in several heart structures such as valves, myocardium, pericardium, conduction tissue and cardiac arteries in patients suffering from systemic lupus erythematosus (SLE), anti-phospholipid syndrome (APS), Sjogren syndrome and other autoimmune rheumatic diseases (ARD). Bethesda, MD 20894, Copyright Valvular disease, for the most part, is mild and asymptomatic; only rarely (4–6%) do aPL positive patients develop valve disease severe enough to require surgical treatment. The authors have declared no conflicts of interest. Maternal and fetal factors associated with mortality and morbidity in a multi-racial/ethnic registry of anti-SSA/Ro-associated cardiac neonatal lupus… Such post-natal progression of CHB has been described in the past by others and today justifies performing electrocardiogram in all children born to anti-SSA/Ro positive mothers [8]. Echocardiographic studies cannot definitely diagnose myocarditis, but global hypokinesis, in the absence of other known causes, is strongly suggestive. Accelerated atherosclerosis. Myocarditis, although mild, has to be treated immediately with high-dose steroids; in the most severe forms is necessary to use intravenous pulse corticosteroid followed by high oral doses. Epub 2012 Jan 17. According to echocardiographic studies, it is not clear if patients with SLE have valve disease more or less than in patients with primary APS; in addition, there is a discrepancy in the prevalence of valvular disease in SLE patients with or without aPL. Levesque K, Morel N, Maltret A, Baron G, Masseau A, Orquevaux P, et al. September 17, 2018 Congenital heart block (CHB) is a rare occurrence, affecting about 1-to-5% babies born to women with systemic lupus erythematosus (SLE) or Sjögren’s syndrome who … Surprisingly there are no clinically detectable abnormalities in the maternal hearts. Others found a correlation between aCL IgG antibody levels and ccIMT [46, 47]. This study also suggested that mitral valve thickening correlated with anti-cardiolipin antibodies (aCL) titre and aCL titre >40 GPL is a risk factor for thromboembolism, occurring in 25% of patients [40]. Background - Neonatal lupus … In most cases, the cause is not Maternal use of hydroxychloroquine is associated with a reduced risk of recurrent anti-SSA/Ro-antibody-associated cardiac manifestations of neonatal lupus… Recently, Nield et al. Mothers frequently are healthy and unaware of their autoantibody … Earlier development of carotid plaques were reported in SLE-associated APS in comparison with primary APS [46, 72]. Anti-phospholipid antibodies have been associated with thrombotic events in coronary arteries, heart valve involvement and intra-myocardial vasculopathy in the context of primary and secondary anti-phospholipid syndrome. Careers. Congenital heart block is a rare disorder that appears to affect males and females in equal numbers. Congenital Heart Block Izmirly PM, Costedoat-Chalumeau N, Pisoni CN, et al. Neonatal lupus and congenital heart block are more common in newborns of mothers with SLE who carry antibodies referred to as anti-Ro (or SS-A) and anti-La (or SS-B). Cerebral dysmaturation, ventriculomegaly, and lenticulostriate vasculopathy are … The most serious and the only permanent condition among these is CHB, which may manifest as a life-long slow ventricular heartbeat. Daily aspirin reduces the risk of MI and reduces CAD-related mortality; recent studies suggested that SLE patients might also benefit from aspirin prophylaxis. Hojnik et al. For Permissions, please email: journals.permissions@oxfordjournals.org. Cardiac involvement is one of the main complications substantially contributing to the morbidity and mortality of patients suffering from systemic autoimmune diseases. The most serious sequelae of NLE occur when autoantibodies attack the cardiocytes in the fetus during the second or third trimesters, causing an immune-mediated congenital heart block (CHB), … Both preclinical (carotid plaque) and clinical (myocardial infarction) atherosclerotic diseases are more prevalent in SLE patients than in the general population; clinically, atherothrombotic events, such as myocardial infarction (MI), have been recognized as risk factors for mortality [26, 47–51]; there may be a bimodal distribution of mortality risk factors in lupus: an ‘early’ peak in mortality is caused by disease activity and severity itself, as well as infections, while a ‘late’ peak is related to CAD [52].