Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-four patients presenting an acute stroke with watershed cerebral infarct on CT scan or MRI were included in this retrospective study. Age was 63 +/- 14 years (mean +/- SD), and sex ratio was 2 men for 1 woman. Main clinical features were: in anterior location, lower limb weakness and frontal syndrome with transcortical motor aphasia in left lesions or spatial dyscalculia in right ones; in posterior location, brachiofacial weakness with constant quadranopsia and hypoesthesia, and Gerstmann syndrome in left lesion. There was no distinctive feature for subcortical and multiple infarcts. In bilateral infarcts, there were one pseudobulbar syndrome, and 2 pseudo brainstem syndromes with neuropsychological signs. Aetiologies were severe carotid artery disease in 14 cases, severe cardiopathy in 6, isolated cerebral angiitis in 1, essential thrombocythemia in 1, protein C deficiency with sickle cell disease in 1, and cholesterol emboli in 1 anatomical case. CBF performed in carotid artery occlusions or tight stenoses showed evidence of haemodynamic changes. Microembolic process can be proposed in the case with cholesterol emboli. Preventive treatment is discussed.
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PMID:Watershed cerebral infarcts: retrospective study of 24 cases. 135

The central nervous system (CNS) is clinically involved in approximately 40% of all systemic lupus erythematosis (SLE) patients. Minor psychiatric symptoms and abnormalities on neuropsychological testing are being detected with increasing frequency. This review summarizes current thinking concerning the diagnosis and pathogenesis of CNS lupus. The main symptoms of CNS lupus can be diffuse (generalized seizures, psychosis) or focal (stroke, peripheral neuropathies). Neuropsychiatric symptoms often occur in the first year of SLE, but are rarely the presenting symptoms of the disease. In studies on the pathology of CNS lupus, vasculopathy, infarcts and haemorrhages are often observed, whereas vasculitis is rare. Endocardial lesions and mural thrombi have also been reported in 33-50% of CNS lupus patients. In diagnostic imaging of the CNS, magnetic resonance imaging (MRI) scans often provide evidence for edema or small infarcts, both in focal and diffuse CNS lupus, whereas computerized tomography (CT) scans only show gross abnormalities. The first reports on position emission tomography (PET) scans in CNS lupus patients show decreased glucose uptake in the brain. The cerebral blood flow decreases during active diffuse and focal CNS lupus. The blood-brain barrier is somewhat more frequently impaired in diffuse CNS lupus. Intrathecal IgG and IgM production is observed in 25-66% of all CNS lupus patient. Various specificities of autoantibodies have been observed in CNS lupus. Of these, anticardiolipin (ACA) antibodies show a well-documented association with focal involvement of the CNS in SLE. These antibodies could cause thrombosis by interfering with the protein C pathway of fibrinolysis. In addition, they are associated with endocardial and valvular heart disease, which is often observed in SLE and which could cause embolism. The relation between ACA and diffuse CNS lupus is not yet clear. Low-avidity anti-DNA antibodies are also found in CNS lupus, possibly because of their cross-reaction with cardiolipin. Antineuronal antibodies and lymphocytotoxic antibodies have been associated with diffuse CNS lupus and abnormalities on neuropsychological testing. However, the population of these antibodies is rather heterogeneous and it has not been possible to assess a common target antigen. Therefore, it is still obscure whether there is also a second immune-mediated mechanism responsible for the development of the diffuse form of CNS lupus.
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PMID:Diagnosis and pathogenesis of CNS lupus. 186 69

A case report of a 61-year-old patient with hypersensitive vasculitis caused by ethylbiscumacetate is presented. After the implantation of the artificial aortic valve the patient was treated with medigoxine, furosemide, dipiridamole, disopiramide, potassium chloride and ethylbiscumacetate. Among these drugs furosemide and ethylbiscumacetate are reported to cause hypersensitive vasculitis. On the basis of the past medical history, clinical and histological findings furosemide was ruled out as a causative agent. The diagnosis was confirmed by the skin biopsy and finding of a normal biologic activity of protein C. The discontinuance of all previous drugs and administration of methylprednisolone led to improvement in the skin lesions of the patient. The need for continuous anticoagulant therapy was the reason for fepromarone to be given, achieving good anticoagulant effect, without any side effects. According to the criteria of Karch and Lasagna the degree of connection between drug use and side effects in this case belongs to the category "probable".
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PMID:[Hypersensitivity vasculitis caused by ethyl biscoumacetate]. 189 Sep 7

Rocky Mountain spotted fever (RMSF) takes its name from the characteristic rash that occurs as a consequence of vasculitis associated with rickettsial invasion of the endothelium. The authors examined sera from 14 patients with serologically confirmed RMSF for the presence of antibodies (IgG and IgM) reactive with human umbilical vein-derived endothelial cells and with the phospholipids cardiolipin (CL) and phosphatidyl serine (PS). Sera from 7 patients (50%) exhibited antiendothelial antibodies, and 10 (71%) patient sera reacted with CL and/or PS. Because such antibodies may interfere with or augment endothelial thrombosis-related activities, acute and convalescent sera were tested for their effects on endothelial PGI2 secretion and protein C activation. Acute sera from two patients and convalescent sera from four patients stimulated protein C activation. Additionally, sera from five acute and nine convalescent cases inhibited basal endothelial PGI2 secretion, but sera from two acute and three convalescent cases stimulated thrombin-dependent PGI2 secretion. These results demonstrated that, in a significant proportion of patients, RMSF was accompanied by the appearance of antibodies that bound to endothelial cells and to phospholipids; some of these antibodies may have altered anticoagulant endothelial functions.
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PMID:Serologic characterization of Rocky Mountain spotted fever. Appearance of antibodies reactive with endothelial cells and phospholipids, and factors that alter protein C activation and prostacyclin secretion. 202 28

Extensive cutaneous necrosis of the limbs with sudden onset was observed in 3 women suffering from benign systemic lupus erythematosus. All 3 had a circulating anticoagulant and a positive venereal diseases reference laboratory test without anticardiolipin antibodies. They were successfully treated with pulse methylprednisolone therapy and plasmapheresis. The necrosis-lupus anticoagulant relationship is discussed due to the absence of another possible etiology for cutaneous necrosis and the latter's histological aspect, i.e., thromboses of the dermal and hypodermal vessels without vasculitis. This cutaneous necrosis resembles that observed in congenital protein C deficiencies and favors the hypothesis of interaction between the lupus anticoagulant and the protein C-S complex.
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PMID:Cutaneous necrosis associated with the lupus anticoagulant. 250 25

We report six cases of protein S deficiency secondary to varicella. Five cases were complicated by thrombotic and vascular events, namely purpura fulminans and necrotic vasculitis, deep vein thrombosis and stroke. Two cases were associated with protein C deficiency and one case revealed a heterozygous factor XII deficiency. The underlying mechanism of this acquired protein S deficiency is unclear but could be related to a direct effect of zoster virus.
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PMID:Varicella and thrombotic complications associated with transient protein C and protein S deficiencies in children. 795 22

Little is known about the pattern of Deep Vein Thrombosis in Saudi Arabia. Over 4 year period, 62 cases with strong evidence of venous thrombosis were studied in King Abdulaziz University and King Fahad Hospitals to learn the pattern of deep vein thrombosis in Jeddah, Western Saudi Arabia. There were 32 females and 30 males. The mean age of the group was 36.0 years (range 6-90 years). One or more risk factors was/were detected in 40 patients. Among these 14 factors, age more than 50 years, obesity, vasculitis, malignancy and postpartum were the common factors encountered. In other 22 patients, no risk factor was found. However, extensive laboratory search diagnosed 9 rare disorders out of these 22 cases. Antithrombin III, protein C, protein S deficiencies in 5, 2, 1 patients, consecutively. The last patient had significantly shortened PTT. The other 13 (21.0%) patients were considered real idiopathic DVT. Extremities were involved in 54 patients compared to only 8 cases with inferior vena cava or visceral thrombosis. The upper limb was affected in only 10 patients unlike the lower limb which was more commonly affected n = 37.
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PMID:Pattern of deep venous thrombosis in Jeddah area, western Saudi Arabia. 837 13

Two girls, 22 months and 12 years of age, presented with repeated cerebral infarctions in association with primary antiphospholipid syndrome. The younger patient also suffered from protein C deficiency, while the other one had protein S and complement C4 deficiencies. All other causes of cerebral infarction were excluded; however, vasculitis remains a possibility in one patient. Both girls developed spastic tetraparesis as a sequela of the previous infarctions. The two patients were treated with aspirin and prednisone, with remission of the infarctions during the next 8 months of observation. A primary deficiency of protein C or S is proposed which would produce cerebral thrombosis with exposure of phospholipids; this thrombosis then, like antigens, would generate antibodies acting on the thrombin-thrombomodulin complex, exacerbating the thrombotic process. The association of complement C4 deficiency is an additional risk factor.
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PMID:Childhood stroke associated with protein C or S deficiency and primary antiphospholipid syndrome. 845 4

Markers of endothelial cell activation were measured in 28 patients presenting with various forms of limited or focal type cutaneous vasculitis. Plasma levels of tissue plasminogen activator antigen (t-PA:Ag), plasminogen activator inhibitor type 1 antigen (PAI-1:Ag) and PAI-1 activity, fibrin plate, von Willebrand factor antigen (vWF:Ag), tissue factor (TF) and soluble thrombomodulin (sTM) were measured. In comparison with the control group (n = 20) there was a significant increase in t-PA:Ag, vWF:Ag and TF (P < 0.05, Mann-Whitney U-test) in the cutaneous vasculitis group. This study confirms that measurable degrees of endothelial activation occur in cutaneous vasculitis. Cutaneous vasculitis includes a diverse group of clinical conditions, which are associated with inflammatory changes in cutaneous blood vessels with local fibrin deposition. The aetiology and pathogenesis of the majority of these entities remain unknown. Causative mediators are thought to include immune complexes, anti-endothelial cell antibodies, cytotoxic lymphocytes and viruses. Histologically, immune complexes and complement are frequently detected on the vessel wall, and serologically anti-endothelial antibodies are often detected in patients with vasculitis and in systemic lupus erythematosus (SLE) which correlate with the severity of cutaneous vasculitis, arthritis and nephritis. Lymphocyte-mediated toxicity to endothelial cells has been reported in a small number of patients with giant cell arteritis and Takayasu's arteritis. The vascular endothelium plays a central part in the control of haemostasis. Under physiological conditions endothelial cells present an anticoagulant surface to blood constituents, partially due to surface expression of heparan sulphate and thrombomodulin (TM). Heparan sulphate binds antithrombin III (ATIII), thereby accelerating inactivation of intrinsic coagulation enzymes. Thrombomodulin is an endothelial cell surface glycoprotein which promotes anticoagulation by forming a complex with thrombin which then activates protein C. Activated protein C together with a cofactor, protein S, inactivates FVa and FVIIIa. von Willebrand factor (vWF) is synthesized by endothelial cells, stored in Weibel-Palade bodies and released into the circulation upon endothelial stimulation. vWF mediates the binding of platelets to the subendothelium and is the carrier molecule for FVIIIC. The endothelium controls fibrinolysis by producing t-PA and its inhibitor PAI-1. Inflammatory cytokines such as interleukin-1 (IL-1) and tumour necrosis factor (TNF) activate endothelial cells, causing a shift from an antithrombotic to prothrombotic state, including expression of tissue factor, increased synthesis of PAI-1 and decreased expression of TM. Fibrin deposition and intravascular thrombosis are seen in cutaneous vasculitis syndromes, suggesting local endothelial cell activation. The aim of this pilot study was to assess whether perturbation of the endothelium in cutaneous vasculitis could be detected in the patients' plasma samples. If so, further studies to assess any correlation in levels of these markers with disease activity might prove useful in the future.
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PMID:Endothelial cell activation in cutaneous vasculitis. 868 65

We developed a novel assay using human thrombomodulin (TM), which detected overall abnormalities in the protein C anticoagulant pathway (PC pathway). This assay indicates the degree of inhibition of prothrombinase by TM, which is represented as the percentage of prothrombinase inhibition by 25 ng/ml of TM, termed PIP25 (Prothrombinase Inhibition Percentage). We examined PIP25 in plasma samples from patients with systemic lupus erythematosus (SLE) with or without lupus anticoagulant (LA), patients with Behcet's disease (BD), and patients with miscellaneous thrombotic vasculitis and compared these with the PIP25 of plasma samples from healthy volunteers in Japan. The PIP25S were significantly lower in SLE alone (35.5 +/- 12.8%, P = 0.036) and SLE with LA (33.0 +/- 13.3%, P = 0.030) and BD (33.3 +/- 13.4%, P = 0.010) than those in healthy volunteers (43.5 +/- 10.7%). There was no significance between healthy PIP25 and those with miscellaneous thrombotic vasculitis (44.2 +/- 8.4%, P = 0.823). These results suggest that the abnormalities of the protein C anticoagulant pathway were present in patients with SLE(LA) and BD.
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PMID:Abnormalities in the protein C anticoagulant pathway detected by a novel assay using human thrombomodulin. 930 62


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