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)

The range of disorders affecting the cerebral veins and sinuses is increasing and now includes blood disorders, abnormalities in the patterns of blood flow, and infiltrative or inflammatory conditions, all of which may promote thrombosis. We describe 10 patients with cerebral venous thrombosis: two had protein S deficiency, one had protein C deficiency, one was in early pregnancy, and there was a single case of each of the following: dural arteriovenous malformation, intracerebral arteriovenous malformation, bilateral glomus tumours, systemic lupus erythematosus, Wegener's granulomatosis, non-Hodgkin's lymphoma. The recognition of such diverse aetiology may be importance since clinical features are non-specific, and may consist only of raised intracranial pressure, allowing confusion with 'benign intracranial hypertension'. The existence of effective treatment both for the thrombosis and for many of the underlying disorders makes early diagnosis essential. The prognosis of treated patients may be favourable.
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PMID:Cerebral venous thrombosis: new causes for an old syndrome? 214 67

Raised intracranial pressure in the absence of an intracranial mass or hydrocephalus (pseudotumor cerebri) has been described in association with many conditions including systemic lupus erythematosus (SLE). Papilledema is often listed in textbooks as one of the neurologic manifestations in SLE. However, the occurrence of this unusual neuroophthalmological presentation is infrequently encountered. We describe 3 women with SLE and pseudotumor cerebri who responded well to corticosteroid therapy.
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PMID:Pseudotumor cerebri in systemic lupus erythematosus. 227 Oct 55

We report a young woman with systemic lupus erythematosus complicated by pseudotumor cerebri which resolved with high dosage corticosteroid therapy. The cause of the raised intracranial pressure proved to be thrombosis of the torcular herophili and lateral sinuses. Despite laboratory evidence of a hypercoagulable state, the presence of a lupus anticoagulant could not be shown.
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PMID:Dural sinus thrombosis: a mechanism for pseudotumor cerebri in systemic lupus erythematosus. 355 88

In normal brain, the blood-brain barrier (BBB) is highly impermeable to K+ cations, their transport being controlled by ATPases situated in the endothelial cell membranes. 82Rb+ is a positron-emitting analogue of K+ with a half-life of 75 s. Using a steady-state model and positron emission tomography, quantitative extraction data for 82Rb+ transport across the BBB have been obtained both in normal human subjects and in a variety of conditions of cerebral pathology. A mean cerebral Rb extraction of 2.1% was found for normal subjects, corresponding to a mean value of 1.1 x 10(-6) cm s-1 for 82Rb+ cation permeability across the BBB. No increase in cerebral Rb extraction was observed for patients with diffusely raised intracranial pressure secondary to obstructive hydrocephalus and benign intracranial hypertension, or for patients with multiple sclerosis or cerebral systemic lupus erythematosus. Cerebral tumours that were enhanced on computed tomography scanning showed a significant increase in local Rb uptake. No correlation between tumour size, or grade of glioma, and tumour Rb extraction was found. Nonenhancing tumours showed no increase in local Rb extraction, and regions of perifocal tumour oedema also had Rb extraction values in the normal range. It is concluded that increased Rb extraction occurs only where tight junction integrity in the BBB breaks down locally, that is, in the microcirculation of enhancing tumours but not in that of perifocal regions of tumour oedema or nonenhancing tumours.
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PMID:Quantitative measurement of blood-brain barrier permeability using rubidium-82 and positron emission tomography. 633 92

Idiopathic intracranial hypertension is a disorder of intracerebral pressure regulation and patients run the risk of permanent visual loss. Intracranial hypertension (IH) has been reported rarely in systemic lupus erythematosus (SLE). We reviewed the medical records of 127 patients with lupus nephritis (LN) who were followed up from 1987 to 1996 in our unit. There were six patients with IH which gave a disease prevalence of 4.7% in those with LN. All were females giving a disease prevalence of 5.2% for that sex, a high rate of occurrence of IH in patients with LN. Their age ranged from 22 to 34 y (27.8 +/- 3.6 y). Headache, vomiting and diplopia were the common presenting symptoms and had started 7.3 +/- 4.4 weeks prior to the diagnosis of IH. The cerebrospinal (CSF) opening pressure (413.3 +/- 77.0 mmH2O) was raised in all cases. Biochemical and cytological analyses of CSF were normal. The only abnormal radiological finding was partially empty sella in one patient on magnetic resonance imaging (MRI) (performed in three patients) or computed tomography (CT) (performed in all patients). All patients had serological evidences of active lupus disease at the time of diagnosis of IH. The renal histology was WHO type IV in four cases and III and V in one each indicating severe renal involvement. Laboratory evidences of procoagulant activity were found in the form of positive anticardiolipin antibody (aCL) in two patients, lupus anticoagulant (LA) in two and an otherwise unexplained isolated prolongation of activated partial thromboplastin time (APTT) in the other two. Clinically, one or more episodes of symptomatic venous or arterial thrombosis had occurred in all subjects. In addition to symptomatic measures, all subjects were treated with prednisolone, azathioprine, cyclophosphamide and plasmapheresis according to the protocol of our unit. One patient who did not receive plasmapheresis and cyclophosphamide had a relapse while all others recovered completely. None received anticoagulant therapy. Young females with serologically active lupus, severe forms of renal lesions, past history of venous or arterial thrombosis and laboratory evidences of procoagulant activity, appear to be at increased risk of IH. Thrombotic occlusion of the cerebral arteriolar or venous vascular bed eventually affecting the arachnoid villi and impeding CSF absorption is favoured compared to cerebral venous or sinus thrombosis as the pathogenic mechanism. Combined immunosuppression and plasmapheresis appeared to be beneficial in short and long term follow-up. We propose that patients with SLE and IH have definable risk and pathogenetic factors and are no more to be considered 'idiopathic'. The conditions calls for aggressive intervention which leads to an excellent outcome.
Lupus 1997
PMID:Treatable intracranial hypertension in patients with lupus nephritis. 930 63

We present the case of a 14-year-old girl who was admitted to the hospital with the complaint of horizontal diplopia for 48 hours. Initially, she was diagnosed with idiopathic intracranial hypertension. During hospitalization she developed fever, macular facial rash, and chest pain, and because of abnormal laboratory findings the diagnosis of systemic lupus erythematosus was established. She received immunomodulatory therapy, a combination of corticosteroids, and intravenous infusions of the monoclonal antibody rituximab, which augmented her clinical improvement. Intracranial hypertension secondary to systemic lupus erythematosus is a rare manifestation, especially as a presenting symptom. In addition, the fact that the patient developed an aggressive form of systemic lupus erythematosus during the initial period of hospitalization for idiopathic intracranial hypertension is also uncommon. Moreover, to our knowledge, we are not aware of any published case reports of intracranial hypertension secondary to systemic lupus erythematosus that was treated with rituximab.
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PMID:Diplopia due to systemic lupus erythematosus in a child: a rare clinical presenting sign of the disease. 2174 1

Clinical characteristics of systemic lupus erythematosus (SLE) patients complicated with cerebral venous sinus thrombosis (CVST) between 2000 and 2013 were analyzed through this retrospective, single-centre study. Of 4747 hospitalized SLE patients, 17 (0.36%, 12 females, average age 30) had CVST. Headache (88.2%) was the most common neurological symptom followed by nausea or vomiting (47.1%), conscious disturbance (41.2%), edema of eyelids or conjunctiva (35.3%), blurred vision or diplopia (35.3%), and seizure (35.3%). Increased intracranial pressure (ICP) occurred in 13 cases (76.5%). Magnetic resonance venography (MRV) detected thrombosis in the transverse (82.4%), sigmoid (52.9%), and sagittal (35.3%) sinuses, with frequent (70.6%) multiple sinus occlusions. Compared to SLE patients without CVST, SLE patients with CVST had a higher prevalence of thrombocytopenia and positive antiphospholipid antibodies and a higher SLE disease activity index (SLEDAI) score. 13 patients achieved improvement following glucocorticoids and immunosuppressants treatment, as well as anticoagulants, while 3 patients died at the hospital. CVST is relatively rare in SLE and tends to occur in active lupus patients. Intracranial hypertension is common but nonspecific clinical feature, so MRV evaluation is necessary to establish a diagnosis. Aggressive treatment for the rapid control of SLE activity combined with anticoagulants can improve the prognosis.
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PMID:Clinical Characteristics of Cerebral Venous Sinus Thrombosis in Patients with Systemic Lupus Erythematosus: A Single-Centre Experience in China. 2609 Apr 93