Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 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

Connective tissue disease (CTD) encompasses several disease or abnormal states characterized by inflammatory or degenerative changes in connective tissue. These disorders affect many organs, including the heart. However, the long-term survival of patients with cardiomyopathy and CTD is not known. The prognostic implications of different categories of CTD were studied in patients with cardiomyopathy. One thousand seven hundred patients with initially unexplained cardiomyopathy who underwent endomyocardial biopsy from 1983 to 2003 at The Johns Hopkins Hospital were evaluated. Seventy-one subjects were diagnosed with CTD. This cohort was followed for an average of 5.1 years. Among the different disorder categories within CTD, there was a significantly lower survival rate for subjects with undifferentiated CTD (adjusted hazard ratio for death 2.39, 95% confidence interval 1.27 to 4.48, p = 0.007) and a trend toward decreased survival in patients with systemic sclerosis (adjusted hazard ratio for death 1.75, 95% confidence interval 0.93 to 3.29, p = 0.081) compared with those with idiopathic dilated cardiomyopathy (DCM). No significant survival difference was observed between systemic lupus erythematosus and idiopathic DCM (p = 0.41). The long-term outcomes of subjects with CTD and myocarditis were not statistically different from the outcomes of those with CTD and no myocarditis (p = 0.71). In conclusion, the diagnosis of cardiomyopathy and either undifferentiated CTD or systemic sclerosis appears to be a poor prognostic indicator compared with the diagnosis of idiopathic DCM. Patients with systemic lupus erythematosus and cardiomyopathy have a similar prognosis to those with idiopathic DCM. Finally, the presence of myocarditis does not alter the survival of patients with CTD and cardiomyopathy.
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PMID:Comparison of survival among patients with connective tissue disease and cardiomyopathy (systemic sclerosis, systemic lupus erythematosus, and undifferentiated disease). 1765 38

Hydroxychloroquine- or chloroquine -induced cardiomyopathy is a rare but potentially fatal condition. Hydroxychloroquine and chloroquine are often used for long-term treatment of rheumatic diseases and for malaria prophylaxis. Hydroxychloroquine- and chloroquine-induced cardiomyopathy have well-described microscopic features, with the classic electron microscopic findings of myelin figures (myeloid bodies). We report on 2 new cases with novel findings. The first case, in a patient with systemic lupus erythematosus, was found to have megamitochondria in addition to myelin figures seen by electron microscopy. The second report describes the first case of hydroxychloroquine cardiomyopathy described in a patient with scleroderma. These novel findings will add to the present knowledge of hydroxychloroquine-induced cardiomyopathy in its pathology and its implication for treatment of rheumatic diseases.
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PMID:New clinical and ultrastructural findings in hydroxychloroquine-induced cardiomyopathy--a report of 2 cases. 1806 91

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

We report two cases of hydroxychloroquine-induced hyperpigmentation presenting in a 50-year-old Caucasian female (case 1) and a 78-year-old female (case 2), both receiving 400 mg per day. Case 1 had an arthritis predominant undifferentiated connective tissue disease, which was treated with hydroxychloroquine for 4-5 years. She presented with a mottled, reticulated macular gray pigmentation involving the upper back and shoulders. Case 2 had a history of systemic lupus erythematosus and rheumatoid arthritis, treated with hydroxychloroquine for 1.5 years. She presented to the hospital for treatment of constrictive cardiomyopathy and was noted to have a blue macular pigmentation involving the right temple. The biopsies from both patients showed superficial dermal, yellow-brown, non-refractile and coarsely granular pigment deposition. A Fontana-Masson stain highlighted some of these granules, while the Perl's iron stain was negative. Rare, previous reports of hyperpigmentation indicate the presence of both melanin and hemosiderin in patients being treated with antimalarial medication. To our knowledge, this staining pattern for hydroxychloroquine has not been previously reported in the literature and supports that hydroxychloroquine, in addition to chloroquine, binds to melanin.
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PMID:Hydroxychloroquine-induced hyperpigmentation: the staining pattern. 1872 67

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

Systemic lupus erythematosus (SLE) is a multisystem disorder with numerous potential adverse effects on the cardiovascular system. These complications likely develop in most patients with SLE at some time during the course of their disease, in part due to the decreased mortality associated with SLE as a result of modem medical management. Conduction disturbances have been reported in the literature to occur primarily from the progression of SLE and secondarily from pharmacotherapy used to treat SLE and may first be evident on the electrocardiogram in the emergency department (ED) setting. Electrocardiogram abnormalities such as borderline first-degree heart block may be clues to more significant cardiac disease brought upon by years of chronic inflammation, myocarditis, vasculitis, and fibrosis that are often the result of longstanding autoimmune disease. It is essential that patients with autoimmune disease be screened carefully in the ED setting for underlying myocardial disease, particularly given the increased potential for atherosclerosis, ischemia, arrhythmias, and myocardial conduction defects in these patients.
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PMID:Rapid progression of atrioventricular nodal blockade in a patient with systemic lupus erythematosus. 1892 71


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