Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P02749 (beta2-glycoprotein I)
 836 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Systemic lupus erythematosus is a complex immunological and rheumatological disease that has numerous complications. Central to the pathogenesis of systemic lupus erythematosus is immune complex formation and deposition in blood vessels and end organs. This is a case report of an autopsy of a patient with systemic lupus erythematosus, end stage renal disease, peripheral vascular occlusive disease, pancreatitis, and aortitis. The aortitis was found to be immune complex mediated with deposition of IgG, C3, as well as fibrinogen in the wall of the aorta as shown by immunofluorescence. The hypercoagulable state of the patient is discussed with particular emphasis on the role of anticardiolipin antibodies, antiphospholipid antibodies, and anti-beta-2-glycoprotein I in the pathogenesis. This case is unique in that the immune complex mediated aortitis has not been described in the literature over the past 25 years. We recommend that the diagnosis of immune complex mediated aortitis be considered in the differential diagnosis of aortitis, particularly in the background of a patient with systemic lupus erythematosus.
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PMID:Lupus aortitis: a case report and review of the literature. 874 62

We report a case of well-documented typhoid fever in a 30-year-old woman with inactive systemic lupus erythematosus with asymptomatic lupus anticoagulant and high-titer anticardiolipin antibody (aCL). Despite prompt eradication of the Salmonella typhi obtained with appropriate antibiotic therapy, multiple organ system dysfunction occurred. The central nervous system was involved, with ischemic infarcts in the occipital lobes. High-dose corticosteroid therapy failed to improve the neurologic manifestations, which responded to repeated plasmapheresis. A sharp fall in aCL and anti-beta2-glycoprotein I antibody titers was recorded before the start of plasmapheresis. At the same time, IgM and IgG antibodies to Salmonella group O:9 lipopolysaccharide became detectable; the IgM antibodies disappeared within 4 months, whereas the IgG antibodies remained detectable during the next 13 months. Despite treatment with high-dose corticosteroids and cyclophosphamide, rapidly progressive glomerulonephritis developed, leading to chronic renal failure. There is convincing evidence of a link between the S. typhi infection and the ensuing catastrophic syndrome in this patient, probably precipitated by bacterial antigens.
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PMID:Systemic lupus erythematosus-associated catastrophic antiphospholipid syndrome occurring after typhoid fever: a possible role of Salmonella lipopolysaccharide in the occurrence of diffuse vasculopathy-coagulopathy. 1032 64

The antiphospholipid syndrome (APS) is a disorder of hypercoagulability, characterised by thromboembolic events, repeated miscarriages and thrombocytopenia in association with circulating antiphospholipid antibodies. These antibodies are directed against epitopes on either oxidised phospholipids complexed with a glycoprotein, beta 2-glycoprotein I, or against the glycoprotein itself. Renal manifestations of the APS are varied and depend on the type of renal pathology present. The renal vasculature may be affected by either a small vessel, thrombotic microangiopathy process or by large vessel thrombosis. In patients with end stage renal disease, the prevalence of antiphospholipid antibodies may increase with time on dialysis. Anticardiolipin antibodies have been associated with a high incidence of haemodialysis access clotting, a major source of morbidity and hospitalisation in end stage renal disease patients. In renal transplant recipients, antiphospholipid antibodies may be associated with a higher incidence of primary graft non-function, particularly in patients without a history of pretransplantation haemodialysis. Complications of the APS during pregnancy span all trimesters and include intrauterine growth retardation, placental abruption, pre-eclampsia, preterm labour and recurrent fetal loss. As these women have a high risk of recurrent fetal loss, multiple treatment modalities have been investigated, including aspirin, heparin, prednisone and intravenous immunoglobulin. Various treatment strategies for the APS have been developed and are based on a combination of anticoagulant therapy with either warfarin or heparin, along with antiplatelet therapy with aspirin. Experimental treatments involving immunomodulatory therapy with intravenous immunoglobulin, apheresis and novel antibody therapy are being investigated with hopes of successful clinical applications.
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PMID:Nephrological and obstetric complications of the antiphospholipid syndrome. 1203 25