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)

The antiphospholipid syndrome is characterized by venous and/or arterial thrombosis, recurrent foetal loss, thrombocytopenia and the presence of antiphospholipid antibodies. This study presents three case reports, which emphasize the multiform clinical features of the syndrome: one case of uncomplicated myocardial infarction in childhood and two prolonged, clinically elusive case histories, both with fatal outcome due to acute, multifocal, non-inflammatory vascular occlusion. It is important to distinguish between this syndrome and various vasculitis syndromes, although consensus on therapy for the antiphospholipid syndrome has not yet been achieved.
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PMID:[Antiphospholipid antibodies and occlusive vascular disease]. 825 17

The antiphospholipid syndrome is usually defined by the association of a clinical manifestation (recurrent venous and/or arterial thrombosis, recurrent spontaneous miscarriages) and a biological abnormality (anticardiolipin antibody, lupus anticoagulant). We retrospectively analyzed the records of 5 patients (4 females, 1 male, aged 30 +/- 12 years) with antiphospholipid syndrome, primary (n = 1) or secondary to systemic lupus erythematosus (n = 4), who developed malignant systemic hypertension with renal insufficiency, in the absence of lupus nephritis. Before the episode of malignant hypertension, all patients had normal systemic blood pressure and renal function. During malignant hypertension the systolic pressure was 206 +/- 39 mmHg and the diastolic pressure 130 +/- 25 mmHg, peak serum creatinine was 204 +/- 95 mumol/l, daily proteinuria was 1.1 +/- 0.8 gr, and complement serum levels were normal in all patients. Renal angiography found normal proximal renal arteries. Renal biopsy showed ischaemic glomeruli without proliferative lesions (n = 5), focal intimal fibrosis either isolated (n = 3) or associated with thrombosis (n = 2) of the intrarenal vessels, and the absence of vasculitis. Immunofluorescence study did not reveal typical lupus deposits. Patients were treated with antihypertensive agents, increasing doses of prednisone (n = 3), and anticoagulant (n = 2) or anti-aggregant therapy (n = 1). After a mean follow-up of 6.8 +/- 5.2 years, 4 patients were still alive with normal blood pressure and renal function, whereas 1 patient died of a probable catastrophic antiphospholipid syndrome. Patients with antiphospholipid syndrome, primary or secondary to systemic lupus erythematosus, may develop malignant hypertension with renal insufficiency and intrarenal vascular lesions, in the absence of lupus nephritis.
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PMID:Malignant hypertension in antiphospholipid syndrome without overt lupus nephritis. 827 82

The antiphospholipid antibodies (aPL), namely, the lupus anticoagulant and the anticardiolipin antibodies, are a family of autoantibodies directed predominantly against negatively charged phospholipids. Many studies have confirmed that patients with these antibodies are prone to repeated episodes of thrombosis, fetal losses, and thrombocytopenia. The association of aPL with these clinical events has been termed the antiphospholipid syndrome. Several skin lesions have been found in patients with this syndrome, including livedo reticularis, livedoid vasculitis, thrombophlebitis, cutaneous infarctions and gangrene of digits, ulcerations, lesions resembling vasculitis (nodules, macules), cutaneous necrosis/infarctions, subungual splinter hemorrhages, and, less commonly, discoid lupus and Degos' disease (malignant atrophic papulosis). In this article, we review the main immunologic and clinical aspects of this syndrome with special emphasis on the dermatologic features.
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PMID:Antiphospholipid syndrome. 842 86

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

Of a total of 800 patients with systemic lupus erythematosus, 37 were found to have developed avascular necrosis of bone (4.6%). Routine radiological screening of all joints was not performed and these 37 represented symptomatic patients only. Twenty-seven of these were positive for antiphospholipid antibodies (aPL) (73%). Both aPL positive and aPL negative patients had received large doses of oral steroids. The frequency of vasculitis and livedo reticularis, as well as arterial thrombosis was higher in the aPL positive group, but this did not reach statistical significance, perhaps because of the small numbers of patients involved. Although the difference in the frequency of thrombocytopenia was more marked in the aPL positive group (p < 0.08), once again, this was not statistically significant at the 5% level. Since the prevalence of aPL positivity in the general lupus population is about 30-40%, the presence of the aPL may be associated with an increased tendency to develop avascular necrosis. This is supported by recent reports of avascular necrosis occurring in patients with primary antiphospholipid syndrome in the absence of previous steroid administration.
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PMID:Avascular necrosis of bone and antiphospholipid antibodies in systemic lupus erythematosus. 847 66

A case of rapidly progressive nephritis is reported in a female patient having systemic lupus erythematosus (SLE) with antiphospholipid syndrome. Clinical presentation of progressive lupus nephritis with intensifying renal insufficiency, arterial hypertension, hematuria, nephrotic syndrome was associated with unusual morphological manifestations of mesangiocapillary glomerulonephritis with advanced vasculitis. The authors attribute a malignant nephritis course atypical for patients with antiphospholipid syndrome to development of renal vasculitis. The discussion covers lupus genesis of vascular involvement, a probable triggering role of antibodies to phospholipids in impairment of endothelial cells.
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PMID:[The characteristics of kidney involvement in a female patient with systemic lupus erythematosus and the antiphospholipid syndrome]. 857 27

Systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) are closely related, but each has it own characteristic vasculopathy: vasculitis in SLE and thrombosis in APS, and either may be a serious or life threatening complication. When a patient has SLE associated with APS, systemic and cerebral vasculitis may coexist with disseminated coagulopathy. We report this complication in a 60-year-old woman who died from stroke and myocardial infarction, an occurrence seldom documented in the literature.
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PMID:Systemic and cerebral vasculitis coexisting with disseminated coagulopathy in systemic lupus erythematosus associated with antiphospholipid syndrome. 859 66

Idiotypic manipulation of naive mice has previously been used for induction of systemic autoimmune diseases (e.g., antiphospholipid syndrome, systemic lupus erythematosus, and Wegener's granulomatosis). The aim of this study focused on the utilization of this technique to induce the production of anti-endothelial cells Abs (AECA) and autoimmune vasculitis in a murine model. AECA were derived from a Wegener's granulomatosis patient's plasma. IgG was purified by absorption on a proteinase-3 affinity column resulting in the depletion of anti-neutrophil cytoplasmic Ab activity. The absorbed IgG fraction displayed a high titer of AECA as evidenced by a cyto-ELISA against unfixed human umbilical vein endothelial cells. BALB/c mice were actively immunized with the purified AECA. Three months after a boost injection with the human AECA, mice developed endogenous AECA (AB), but not Abs to proteinase-3, cardiolipin, or DNA. Histologic examination of lungs and kidneys revealed both lymphoid cell infiltration surrounding arterioles and venules; as well as deposition of Igs at the outer part of blood vessel walls. This experimental animal model of vasculitis, a product of our method of idiotypic manipulation, provides the first direct proof for the pathogenicity of AECA.
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PMID:Pathogenic role of anti-endothelial cell antibodies in vasculitis. An idiotypic experimental model. 864 46

The anticardiolipin or antiphospholipid antibody syndrome is characterized by an increased incidence of venous and arterial thromboses. This syndrome may occur in association with systemic lupus erythematosus or independently. Gastroenterological manifestations have included Budd-Chiari syndrome, hepatic infarction, esophageal necrosis with perforation, intestinal ischemia and infarction, pancreatitis, and colonic ulceration. We report a 39-yr-old man with antiphospholipid antibody syndrome complicated by adrenal insufficiency secondary to bilateral adrenal infarction who presented with severe epigastric pain. Endoscopic evaluation disclosed progressive gastric ulceration with necrosis in the distal body. Angiography revealed no vasculitis. Because of intractable pain despite intravenous anticoagulation and narcotic analgesia, the patient was taken to surgery, and an antrectomy with Billroth II gastrojejunostomy was performed. Histological examination revealed widespread vascular occlusive disease involving veins, small arteries, and arterioles present in all layers of the stomach and the perigastric fat consistent with the vasculopathy of the antiphospholipid antibody syndrome. Treatment with high intensity oral anticoagulation and corticosteroids resulted in clinical and endoscopic improvement. This case report extends the gastroenterological manifestations of the antiphospholipid antibody syndrome to include giant gastric ulceration and emphasizes the importance of anticoagulation in treatment.
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PMID:Giant gastric ulceration associated with antiphospholipid antibody syndrome. 912 46

We present a case of a 44-year-old female with systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome (APS). The patient died in the 15th year of the disease. The paraffin sections of the brain and spinal cord were examined using routine histological methods and immunohistochemistry with monoclonal antibodies against CD31, FVIIIAg and polyclonal antibodies IgG, fibrinogen and C3. Numerous thrombi-recent, organized and recanalized and focal vasculitis were seen. The immunopositive reaction in thickened vessel walls for CD31 and FVIIIAg indicated the process of the local incorporation of the thrombi. The immunoreactivity for fibrinogen, IgG and C3 suggests the immunological complexes formation in vessel walls, perivascular astrocytes and neurons.
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PMID:Antiphospholipid syndrome in systemic lupus erythematosus--immunomorphological study of the central nervous system; case report. 867 31


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