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)

Two cases of cerebral transmural angiitis and ruptured aneurysm in patients with established systemic lupus erythematosus are presented. A 22-year-old woman with a 4-year history of systemic lupus erythematosus was found to have a ruptured cerebral aneurysm at the trifurcation of the middle cerebral artery. She died 10 days after admission because of the brain swelling. Necropsy showed remarkable brain edema and focal transmural angiitis at the site of the ruptured aneurysm. Another 29-year-old woman with a 3-year history of systemic lupus erythematosus had an acute subdural hematoma resulting from a ruptured aneurysm of the right anterior cerebral artery. She was surgically treated. The ruptured aneurysm was resected and examined histologically. The transmural angiitis was demonstrated. This report describes two radiographically and pathologically confirmed cases of central nervous system lupus producing focal angiitis of a cerebral artery with secondary aneurysm formation.
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PMID:Cerebral transmural angiitis and ruptured cerebral aneurysms in patients with systemic lupus erythematosus. 239 3

The blood-brain barrier separates brain interstitial space from blood and is formed by brain capillary endothelial cells that are fused together by epithelial-like tight junctions. Study of the blood-brain barrier traditionally has been a relatively arcane field, even for neurobiologists. However, advances over the last 10 years in understanding the transport physiology and cell biology of the brain capillary endothelial cell now provide insights into the pathogenesis of such problems as brain glucopenia, hepatic encephalopathy, therapeutic efficacy of alpha-methyldopa, brain edema in diabetic ketoacidosis, Alzheimer's disease, brain tumors, and lupus cerebritis.
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PMID:Blood-brain barrier: interface between internal medicine and the brain. 287 46

Changes in brain water and cerebral volume can lead to brain edema that may be one of the underlying causes of death in many neurological diseases. Cerebral water content is regulated by aquaporin 4 (AQ4) present in astrocytic end feet and around blood vessels. In systemic lupus erythematosus (SLE), magnetic resonance imaging (MRI) studies of the brain have demonstrated lesions with the prominent appearance of edema. Activation of complement may play a significant role in the pathogenesis of lupus cerebritis by causing inflammation that can lead to edema. In this study, the well-established MRL/lpr lupus mouse model was used to evaluate the role of complement in lupus cerebritis. IgG and C1q colocalized in perivascular deposits indicating that the blood-brain barrier was compromised. Both RNA and protein expressions of AQ4 were significantly increased in brains of MRL/lpr mice. Chronic administration of the soluble complement inhibitor, Crry-Ig, reduced inflammation as measured by decreased accumulation of IgG. In contrast to control MRL/lpr mice, AQ4 expression in complement inhibited MRL/lpr mice was not changed relative to untreated congenic controls. These results illustrate that complement activation in brains of lupus mice leads to enhanced AQ4 expression and inflammation. It is conceivable that increased AQ4 expression results in cerebral edema and hence complement inhibition may provide a new therapeutic option in inflammatory cerebral disorders such as lupus cerebritis.
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PMID:Administration of the soluble complement inhibitor, Crry-Ig, reduces inflammation and aquaporin 4 expression in lupus cerebritis. 1463 48

This case report concerns a 22-year-old woman who had been diagnosed with systemic lupus erythematosus (SLE). She had intermittent fever, butterfly erythema, photosensitivity, oral ulcers, and multiple arthralgia in the past seven years, but she did not adhere to regular treatments. The edema of the lower extremities and face aggravated in the recent two weeks, so she was admitted to our Department of Rheumatology and Clinical Immunology. Meanwhile, we found she had severe hypertension, the maximal blood pressure was 170/120 mmHg. The patient had high SLE disease activity (the disease activity index score was as high as 23) with blood involvement, acute renal insufficiency, multiple serous effusion and rash. After one week treatments of intravenous methylprednisolone 80 mg daily and other drugs, her conditions made some extent improvement. However, she suffered sudden epileptic attacks. No positive neuropathological signs were found, and the blood pressure was up to 190/130 mmHg before the onset of the seizures. Her cerebrospinal fluid (CSF) pressure was 330 mmH2O, the CSF protein level was normal value, and the white blood cell count was 0 cell/mm3, with no signs of infection. Cranial MRI showed vasogenic edema at bilateral parietal, occipito-parietal regions, and centrum ovale. We prescribed drugs of decreasing intracranial pressure, intravenous drugs of decreasing blood pressure and midazolam for sedation, without corticosteroid impulse therapy. She recovered consciousness in the next day, without epilepsy recurrence. We eventually diagnosed it as posterior reversible encephalopathy syndrome (PRES), according to the history, laboratory results, imaging featuresand clinical outcome. PRES is a disorder of reversible subcortical vasogenic brain edema in patients with acute neurological symptoms (eg, seizures, encephalopathy, headache, and visual disturbances). PRES is mainly caused by blood pressure changes or endothelial injury, which lead to breakdown of the blood-brain barrier and subsequent brain edema. Most patients have a favourable prognosis. SLE complicated with PRES is not rare, especially in patients with disease activity, hypertension, lupus nephritis and/or renal insufficiency, and use of cytotoxic drugs, early recognition and appropriate treatment remain important. Brainstem involvement, intracranial hemorrhage, renal insufficiency and high disease activity of lupus are risk factors for poor prognosis.
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PMID:[Posterior reversible encephalopathy syndrome in systemic lupus erythematosus: a case report]. 3056 90