Clin Exp Rheumatol
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Patients with cyanotic congenital heart disease exhibit an increased incidence of thrombotic episodes and are frequently thrombocytopenic. We studied the sera of 15 patients with this type of heart malformation, searching for anticardiolipin antibodies. 3/15 had positive results. The three of them were adult females; two had thrombotic episodes and a false positive VDRL. Thus, cyanotic congenital heart disease may be another disease entity associated with the antiphospholipid syndrome.
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The innervation of normal rat synovium and adjacent bone, especially the communicatory nerve fibers between them, were investigated. ⋯ The role of nerves, especially the role of the communicatory nerves between the synovium and the bone marrow, should be considered in the pathophysiolosy of erosive arthritis.
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Comparative Study
Comparison of tests for the lupus anticoagulant and antiphospholipid antibodies in systemic lupus erythematosus.
A variety of laboratory assays are used to screen for the presence of the lupus anticoagulant. Six commonly used coagulation tests, and the ELISA assay for antiphospholipid antibody using three different substrate phospholipids, have been evaluated in 110 patients with systemic lupus erythematosus or lupus-like disease. One or more coagulation assays was abnormal in 41% (45/110) of the patients. ⋯ Approximately half (56%) of the patients with a positive clotting test had an abnormal antiphospholipid antibody assay. A similar proportion (58%) of the aPL positive patients had a prolonged coagulation test. The marked discordance between the coagulation assays and a positive antiphospholipid antibody test further complicates the laboratory definition of this abnormality, at least in patients with systemic lupus erythematosus.
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Editorial Comment
CD8 lymphocytes in polymyalgia rheumatica and giant cell arteritis.
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Case Reports
Macrophage activation syndrome and rheumatic disease in childhood: a report of four new cases.
A macrophage activation syndrome (MAS) developed in four children with chronic rheumatic diseases. The presentation included fever, hepatic and splenic enlargement, profound depression of blood counts, lowering of ESR, elevation of SGOT/PT and hypofibrinogenemia. The most characteristic sign of MAS was the presence in the bone marrow aspirate of well differentiated macrophages showing active haemophagocytosis with haematopoietic elements in their cytoplasm. ⋯ Its efficacy supports the central involvement of a T-cell dysfunction. It must be borne in mind that children with rheumatic diseases, especially the systemic form of juvenile chronic arthritis, are highly vulnerable to life-threatening macrophage activation, which appears to be more frequent than previously recognized. Very careful monitoring of apparently "innocent" drugs and intercurrent viral infections is thus required.