Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

When anti-SSA/Ro antibodies are present in sera of mothers with connective tissue diseases, the incidence of congenital heart block (CHB) has been reported to be 1-2% in live births. The risk of recurrence of CHB in a subsequent child remains limited to 10-16%. CHBs are usually complete but CHB of the first or second degree can also be observed. In some cases, CHB is associated with endocardial fibroelastosis. Late-onset cardiomyopathy may occur later in life in these children. The mortality of CHB, which is predominant in utero and in the first months of life, is an estimated 16-19%. A pacemaker is required in about 66% of cases. Curative treatment of CHB is based on fluorinated steroids (dexamethasone or betamethasone) that do cross the placenta in an active form. Guidelines are available but further studies are needed to optimize treatment.
Lupus 2005
PMID:Anti-SSA/Ro and anti-SSB/La antibody-mediated congenital heart block. 1621 62

The heart can be involved in vasculitides but the frequency of its involvement and the manifestations vary according to the vasculitis. Cardiovascular manifestations include cardiomyopathy (specific or resulting from myocardial infarctions), coronary arteritis (with risk of aneurysms, stenoses and thrombosis formation or rupture), pericarditis, valvulitis, conduction-tissue involvement (with heart blocks), arrhythmias (mainly supraventricular) and/or dissection of the aorta (and/or its proximal branches). As many of these manifestations are clinically silent, at least during their early stages, heart function should be systematically assessed in vasculitis patients, with at least ECG and echocardiography, and more invasive exploratory procedures when the former reveal abnormalities or symptoms become manifest. Specific cardiomyopathy has been identified as a factor of poor outcome in small and medium-sized vessel vasculitides (five-factor score). Therefore, in addition to symptomatic treatments, prescription of corticosteroids and immunosuppressants (mainly cyclophosphamide) is considered mandatory. This regimen has dramatically improved the overall prognosis of affected patients.
Lupus 2005
PMID:Cardiac involvement in small and medium-sized vessel vasculitides. 1621 74

We report a multicenter study of Chinese children in Hong Kong with systemic lupus erythematosus (SLE) nephritis. Children were included if: they fulfilled the ACR criteria, had significant proteinuria or casturia, were Chinese and younger than 19 years and had been diagnosed with SLE between January 1990 and December 2003. Investigators in each center retrieved data on clinical features, biopsy reports, treatment and outcome of these patients. There were 128 patients (eight boys, 120 girls; mean age: 11.9+/-2.8 years). About 50% presented with multisystem illness and 40% with nephritic/nephrotic symptoms. Negative anti-dsDNA antibodies were found in 6% of the patients. Renal biopsy revealed WHO Class II, III, IV and V nephritis in 13 (10%), 22 (17%), 69 (54%) and 13 (10%) patients, respectively. The clinical severity of the nephritis did not accurately predict renal biopsy findings. The follow-up period ranged from 1 to 16.5 years (mean+/-SD: 5.76+/-3.61 years). During the study five patients died (two from lupus flare, one from cardiomyopathy, two from infections). Four patients had endstage renal failure (ESRF) (one died during a lupus flare). All deaths and end-stage renal failure occurred in the Class IV nephritis group. Chronic organ damage was infrequent in the survivors. The actuarial patient survival rates at 5, 10 and 15 years of age were 95.3, 91.8, and 91.8%, respectively. For Class IV nephritis patients, the survival rates without ESRF at 5, 10, and 15 years were 91.5, 82.3 and 76%, respectively. The survival and chronic morbidity rates of the Chinese SLE children in the present study are comparable to those of other published studies.
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PMID:Lupus nephritis in Chinese children--a territory-wide cohort study in Hong Kong. 1663 24

A 32-year-old woman tested positive for lupus anticoagulant when she had fever of unknown origin at 18 weeks of pregnancy. Sixteen days after a normal delivery at 35 weeks, she developed dyspnea and was hospitalized with heart failure. Chest radiography showed severe pulmonary edema. Echocardiography showed dilation and diffuse hypokinesis of the left ventricle. The diagnosis was peripartum cardiomyopathy. The patient responded to diuretic and vasodilator therapy. Endomyocardial biopsy revealed mild myocardial degeneration and interstitial fibrosis. Heart failure due to coronary microthrombosis has been indicated in patients with antiphospholipid antibodies, suggesting such a relationship in this case.
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PMID:[Peripartum cardiomyopathy with antiphospholipid antibody: a case report]. 1676 33

Neonatal lupus is a rare disease, with a prevalence of 1:17,000-20,000 child births. The disease is attributed to autoantibodies such as anti-Ro and anti-La. These antibodies are a common finding in Systemic Lupus Erythematosus and Sjogren disease patients. The clinical manifestations include dermatological, hematological, hepatic, neurological and cardiac involvement. Cardiac disease may be presented as atrioventricular conduction block, cardiomyopathy or endocardial fibroelastosis. If the fetus's heart is affected, the death rates may be as high as thirty percent, and dilated cardiomyopathy may evolve. About 60% of infants will require a permanent pacemaker. Routine screening and early detection are crucial for the initiation of effective treatment. Nevertheless, preventive treatment for all mothers is not recommended due to the low prevalence of the disorder and the common side effects. This article reviews the current knowledge on pathophysiology, and treatment options in neonatal lupus erythematosus.
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PMID:[Neonatal lupus erythematosus--cardiac manifestations and atrio-ventricular conduction block]. 1825 46

Neonatal lupus is an uncommon condition associated with maternal anti-Ro autoantibodies. Findings may include cutaneous lupus lesions, third-degree heart block, cardiomyopathy, hepatobiliary disease, and/or thrombocytopenia or other hematologic cytopenias. It is common for only one organ to be affected, but any combination of organ involvement may occur. Recent studies have raised the possibility that the central nervous system may also be affected, but if it is, it is generally apparently asymptomatic. The most common severe manifestation of neonatal lupus is third-degree heart block, which usually begins during the second trimester of gestation. Attempts have been made to prevent the development of heart block, most often by treating the mother with systemic corticosteroids during pregnancy. There is not yet consensus as to the value of intervention during pregnancy. The neonatal lupus disease process is transient, although third-degree heart block, once established, is permanent. Cutaneous lesions tend to resolve completely and affected individuals tend to be healthy later in childhood. There does appear to be an increased risk for children who have had neonatal lupus to develop autoimmune diseases later in childhood or adulthood. The magnitude of that risk is uncertain. Mothers, who are often asymptomatic at the time of delivery of a baby with neonatal lupus, tend eventually to develop signs and symptoms of autoimmune disease.
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PMID:The clinical spectrum of neonatal lupus. 1879 91

A young woman who had a delivery history 3 months previously presented with dyspnea and orthopnea. Initial findings of physical examination, chest radiography, and echocardiogram showed typical congestive heart failure with severe left ventricular (LV) dysfunction. At first, we considered peripartum cardiomyopathy because she had given birth to a baby 3 months previously. However, even though we massively tried conventional drug therapy for 10 days, the patient still remained with refractory heart failure. We performed additional laboratory studies such as complement level and autoantibodies, of which the results supported systemic lupus erythematosus. We could make the diagnosis of acute lupus myocarditis and treated her with corticosteroid. The symptoms were dramatically disappeared and LV function also improved.
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PMID:Lupus myocarditis presenting as acute congestive heart failure: a case report. 1927 Aug 36

A case of a 29-year-old woman 18 days after delivery with catastrophic antiphospholipid syndrome secondary (CAPS) due to undiagnosed systemic lupus erythematosus, leading to cardiogenic shock is reported. Laboratory evaluation revealed increased anticardiolipin antibodies, lupus anticoagulant, antinuclear antibody and thrombocytopenia. Left ventricular ejection fraction was 20%, neurologic deficit and acute renal failure were also present. Cardiac involvement is common in CAPS, but cardiomyopathy due to microvascular thrombosis is rare. CAPS should be considered as a cause of acute heart failure in a women with systemic lupus erythematosus. In the presented case early therapy with anticoagulants, steroids, immunoglobulins and plasmaferesis was beneficial.
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PMID:[Catastrophic antiphospholipid syndrome complicated by cardiogenic shock - a case report]. 1965

Anti-Ro/SSA antibodies are associated with neonatal lupus (congenital heart block (CHB), neonatal transient skin rash, hematological and hepatic abnormalities), but do not negatively affects other gestational outcomes, and the general outcome of these pregnancies is now good, when followed by experienced multidisciplinary teams. The prevalence of CHB, defined as an atrioventricular block diagnosed in utero, at birth, or within the neonatal period (0-27 days after birth), in the offspring of an anti-Ro/SSA-positive women is 1-2%, of neonatal lupus rash around 10-20%, while laboratory abnormalities in asymptomatic babies can be detected in up to 27% of cases. The risk of recurrence of CHB is ten times higher. Most of the mothers are asymptomatic at delivery and are identified only by the birth of an affected child. Half of these asymptomatic women develop symptoms of a rheumatic disease, most commonly arthralgias and xerophtalmia, but few develop lupus nephritis. A standard therapy for CHB is still matter of investigation, although fluorinated corticosteroids have been reported to be effective for associated cardiomyopathy. Serial echocardiograms and obstetric sonograms, performed at least every 1-2 weeks starting from the 16th week of gestational age, are recommended in anti-Ro/SSA-positive pregnant women to detect early fetal abnormalities that might be a target of preventive therapy.
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PMID:Pregnancy outcomes in patients with autoimmune diseases and anti-Ro/SSA antibodies. 2001 31

'Neonatal' lupus erythematosus (NLE) describes a clinical spectrum of cardiac and non-cardiac abnormalities observed in neonates and foetuses whose mothers have the auto-antibodies anti-SSA/Ro (anti-Ro) and anti-SSB/La (anti-La). Of the cardiac abnormalities, congenital AVB is the most common cardiovascular abnormality found in affected foetuses and infants. Many other cardiovascular manifestations of NLE have been more recently recognized including atrial and ventricular arrhythmias and other conduction abnormalities, myocarditis, cardiomyopathy often with endocardiofibroelastosis and structural heart disease, particularly valvar lesions. In this report, the spectrum of cardiovascular manifestations observed in foetuses and infants with NLE are reviewed and the pathogenesis, diagnosis and clinical outcomes are briefly discussed.
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PMID:Spectrum of cardiac involvement in neonatal lupus. 2069 15


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