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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Antiphospholipid antibodies (APAb) are often found in systemic lupus erythematosus (LE) (secondary antiphospholipid syndrome), leading to arterial or venous thrombosis. In primary antiphospholipid syndrome other LE-associated symptoms are not detectable. We present the clinical course and therapy of three patients with this rarely reported disease, discussing immunopathology and therapy. Three young patients (28, 29 and 31 years) were seen at the university eye clinic, presenting severe occlusive vasculitis with vitreal haemorrhages and/or thrombosis. The first patient did not respond to any therapy (various immunosuppressives, plasma-pheresis, laser coagulation) and ended up with defective light perception in both eyes. The second patient also had light perception as final outcome in spite of acetylsalicylic acid, steroids and immunosuppression. The third patient established complete reperfusion on an arteriovenous occlusion after haemodilution and acetylsalicylic acid. Antibodies directed against phospholipids interfere with the blood clotting system in many ways (activation and aggregation of thrombocytes, endothelial function, coagulation cascade). The optimal therapy of this severe disease is unclear, suggesting that acetylsalicylic acid seems to be important, while the effect of immunosuppression or steroids is uncertain.
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PMID:[Retinal vasculitis and antiphospholipid antibodies]. 784 30

Results of immunological studies on skin biopsies of 5 patients with Sneddon's syndrome are reported. Also studied were coagulation factors and autoantibodies believed to play a role in this syndrome. Hemostasis was normal except for a mild increase of fibrinogen in one subject; lupus anticoagulant (LAC) and anticardiolipin antibodies were negative in all. The skin biopsies ruled out systemic vasculitis and vasculitis in association with connective tissue diseases. Sneddon's syndrome is a peculiar clinicopathological condition, probably with several etiologies, but is distinct from primary antiphospholipid syndrome.
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PMID:Sneddon's syndrome: clinical and immunohistochemical findings. 788 93

We report the first case of pathologic findings from brain biopsy in a patient with Sneddon's syndrome. The observations suggest that Sneddon's syndrome is not a vasculitis but is more comparable to the autoimmune vasculopathies such as the antiphospholipid antibody syndrome. Vascular thrombosis and emboli from cardiac sources are the likely causes of stroke in most cases. The success of warfarin in the treatment of antiphospholipid syndromes and the failure of immunosuppression and aspirin in the treatment of Sneddon's syndrome argue that warfarin anticoagulation may be the most appropriate intervention currently available.
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PMID:Sneddon's syndrome is a thrombotic vasculopathy: neuropathologic and neuroradiologic evidence. 864 99

The antiphospholipid antibody syndrome may develop both in patients with Systemic Lupus Erythematosus and as a primary disease, i.e. in the absence of both clinical manifestations and specific antibodies of Systemic Lupus Erythematosus. The primary syndrome is characterized by one of the following clinical manifestations: recurrent thromboembolic events, spontaneous abortions, immune thrombocytopenia, associated with laboratory signs such as the presence of anticardiolipin antibodies, lupus anticoagulant and/or antiphospholipid antibodies. Thrombosis by antiphospholipid antibodies is due to endothelial damage rather than to vasculitis. Several mechanisms have been postulated to explain the thrombotic effect of these antibodies. Anticoagulants, both dicumarol and heparin, associated with prednisone, are the classic therapy. High doses of immunoglobulins, plasmapheresis, immunoadsorbent plasmapheresis have also been used. Corticosteroids in bolus, possibly associated with cyclophosphamide also in bolus and plasmapheresis can be tried in more severe cases.
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PMID:[Antiphospholipid antibody syndrome]. 795 52

The HELLP syndrome (hemolysis, elevated liver enzymes and low platelets) is a thrombotic microangiopathic vasculopathy that presents in pregnancy. HELLP syndrome usually resolves with delivery of the fetus without sequelae and is often managed successfully with conservative care. To our knowledge, there has been no prior report of an association between HELLP syndrome and elevated titers of antiphospholipid antibodies (aPL). We describe 2 patients who each presented with HELLP syndrome that was refractory despite delivery of the fetus, corticosteroids, and anticoagulation. Both patients had elevated levels of anticardiolipin antibodies. Examination of skin and placental pathology revealed diffuse deposition of fibrin with small vessel thrombi, without evidence of vasculitis. Coincident with plasmapheresis, HELLP resolved in both patients. We conclude that there may be an association between the presence of aPL and a protracted HELLP syndrome in pregnancy; this may be a new manifestation of the antiphospholipid syndrome. Plasmapheresis appears to benefit these patients. Further investigation into this association is warranted.
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PMID:An association between refractory HELLP syndrome and antiphospholipid antibodies during pregnancy; a report of 2 cases. 796 86

We report five cases of pancreatitis in systemic lupus erythematosus. Three patients died. The cause of death was directly related to pancreatitis in two cases. Several causes of pancreatitis may be suspected: vasculitis, thrombosis when associated to antiphospholipid syndrome, biliary calculi, infection, metabolic abnormalities or adverse effects of therapy. About seventy cases of pancreatitis have been reported in systemic lupus erythematosus in the literature Pancreatitis was the inaugural sign of lupus in six cases. In 12 cases, besides lupus, no other cause of pancreatitis was found. However, pancreatic vasculitis or thrombosis was rarely demonstrated even in post-mortem examinations. The role of corticosteroids in pancreatitis in lupus is controversial since the evolution of pancreatitis in lupus is generally good with corticosteroids. We think that diagnosis of pancreatitis in lupus should not implicate discontinuation or decrease of corticosteroids dosage, unless their responsibility is absolutely demonstrated.
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PMID:[Pancreatitis in systemic lupus erythematosus. Review of the literature apropos of 5 cases]. 805 27

A case of self-limited renal vasculitis during pregnancy in a patient with a history of recurrent fetal loss and with a positive cardiolipin immunoglobulin G antibody test is described. The renal disease manifested as an acute renal failure in the second trimester of the patient's third pregnancy, concurrent with severe pre-eclampsia. Vasculitis is a rare manifestation of antiphospholipid syndrome that has been described mostly in peripheral arteries. Renal vasculitis, however, has not yet been reported in association with this syndrome. The full spectrum of renal involvement in antiphospholipid syndrome is presently being determined, and we suggest that renal vasculitis be included in that spectrum.
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PMID:Primary antiphospholipid syndrome and self-limited renal vasculitis during pregnancy: case report and review of the literature. 807 77

The association of deep vein thrombosis with systemic necrotizing vasculitis is infrequent. Herein are referred two patients with microscopic polyarteritis and one patient with overlap syndrome who developed deep vein thrombosis which complicated in two cases with pulmonary embolism. The clinical features and the pathogenic mechanisms involved in this infrequent association are analyzed. In two patients the thrombosis was simultaneous with the diagnosis of vasculitis. In one patient deep vein thrombosis was the cause of hospital admission. Advanced age and thrombocytosis are factors associated to vasculitis, which may favor the development of thromboembolic disease. The presence of antiphospholipid syndrome was discarded. Although infrequent, this association should be considered because of the potential severe complications.
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PMID:[Deep venous thrombosis associated with necrotizing vasculitis]. 811 40

We report a patient who developed urticaria, angio-oedema and polyarthropathy secondary to the hypocomplementaemic urticarial vasculitis syndrome, a year prior to the onset of ulcerative colitis. Ten years later, primary sclerosing cholangitis and the antiphospholipid syndrome developed concomitantly. We believe this patient represents only the second reported case of idiopathic systemic lupus erythematosus (SLE) occurring in association with ulcerative colitis.
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PMID:Systemic lupus erythematosus in association with ulcerative colitis: related autoimmune diseases. 814 83

The aim of the study was to prove the incidence of antiphospholipid syndrome (APS) in systemic connective tissue disease (SCTD), especially in systemic lupus erythematosus (SLE) and to find out serologic markers of APS. Of 94 patients with SCTD followed up in the last three years 80 had SLE, 7 Sjogren's syndrome, 3 systemic vasculitis, 2 combined systemic connective tissue disease, 1 rheumatoid arthritis, 1 Sjogren's syndrome with vasculitis. Antiphospholipid antibodies (ApSt) were determined by ELISA method. In 31 of 80 patients with SLE (38.07%) antiphospholipid antibodies were proved. In lo of 31 (32.26%) signs of systemic lupus or the presence of APS markers have not been found, while 21 of 31 (67.74%) had some of clinical manifestations of APS (most commonly thrombocytopenia or vascular thrombosis) with evolution of the basic disease. Among our patients with SLE the most common finding was IgG antibody isotype-in 14 or 66.66% of cases. In 5 of 7 patients with Sjogren's syndrome ApAt were proved without clinical evolution of the basic disease and APS. Only 2 of 7 patients of this group showed, together with more increased ApAt, mild evolution of the basic disease and manifestation of APS (livado reticularis and recurrent venous thrombosis).
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PMID:[The antiphospholipid syndrome in systemic connective tissue diseases]. 819 29


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