Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The occurrence of hyperviscosity syndrome in a 22-year-old woman with systemic lupus erythematosus was reported. Ultracentrifuge study of her serum revealed the presence of intermediate complexes with sedimentation coefficients of 10.9S to 14.2S. The complexes were shown to consist of immunoglobulin G and immunoglobulin A and considered to be mainly responsible for the increased serum viscosity. This unusual protein abnormality responded remarkably well to the treatment with prednisolone.
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PMID:Hyperviscosity syndrome in a patient with systemic lupus erythematosus. 86 20

We report the first case of systemic lupus erythematosus with hyperviscosity syndrome as the presenting manifestation. A polyclonal increase in all immunoglobulins was observed. Ultracentrifuge studies showed 17-19S intermediate immune complexes (IC) composed primarily of autoreactive IgG demonstrated at acid pH. This was confirmed by chromatographic fractionation of sera, since the intermediate IC eluted as did purified heat aggregated IgG. Small amounts of IgM and IgA in the intermediate IC were also demonstrated by more sensitive techniques, i.e., nephelometry, polyacrylamide gel electrophoresis, and electrophoretic blotting. The decrease of serum viscosity was associated with the decrease of circulating IC after plasmapheresis or combined treatment of prednisone and cyclophosphamide. Serum viscosity bore no relationship to the amount of dissociated immunoglobulins.
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PMID:Hyperviscosity syndrome as the initial manifestation of systemic lupus erythematosus. 274 67

On the basis of an extensive study of references as well as of own experiences the present state of the plasma exchange therapy, its success, problems and tendencies are represented. In the 1st part the essential modifications of the technique (plasmapheresis/plasma filtration), problems of anticoagulation, kind and quantity of the substitution as well as tendencies to more specific separation methods are explained. The 2nd part gives a survey of all essential indications (Goodpasture's syndrome, myasthenia gravis, hyperviscosity syndrome, lupus erythematodes visceralis, intoxications) and shows the main complications.
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PMID:[Plasma exchange therapy (plasmapheresis/plasmafiltration). II. Indications and complications]. 654 91

Large-volume plasma exchange can now be rapidly and safely done using cell separator technology. Significant depletion of immunoglobulins and immune complexes can be achieved by repeated intensive plasmapheresis, but sustained depletion of these constituents requires concomitant immunosuppressive therapy. Plasmapheresis appears to work in some disorders by removing pathogenic antibodies, but other mechanisms of action have been postulated. It is the treatment of choice for thrombotic thrombocytopenic purpura and for the hyperviscosity syndrome due to macroglobulinemia. Apheresis can be useful in the treatment of many other disorders, most notably myasthenia gravis, glomerulonephritis associated with hemoptysis (Goodpasture's syndrome), refractory systemic lupus erythematosus, cryoglobulinemia and immune cytopenic disorders.
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PMID:Plasmapheresis in clinical medicine. 660 32

Plasma exchange can remove putative pathogenic autoantibodies and circulating immune complexes from the blood of patients with systemic lupus erythematosus (SLE). However, their efficacy has only been supported by noncontrolled and/or retrospective studies. Nonetheless, PE may still be of relevance in some selected SLE patients and as adjunctive therapy, in combination with corticosteroids (CS) and other immunosuppressant(s). We review herein the principal historical steps of the use of plasma exchange to treat SLE, based upon the main trials and case reports that have highlighted its most pertinent indications. Acute life-threatening manifestations and severe therapy-resistant manifestations, like refractory SLE renal disease, diffuse alveolar hemorrhage, neuropsychiatric SLE, thrombotic thrombocytopenic purpura, catastrophic antiphospholipid syndrome, hyperviscosity syndrome and cryoglobulinemia, are the indications for which plasma exchange might have a beneficial therapeutic role. Although few SLE patients undergo plasma exchange each year nowadays (10-20 per year in France), adverse events are very rare and recent advances in plasma exchange technologies, like immunoadsorption, might, in the future, counterbalance their cost and broaden their place in the therapeutic armamentarium for SLE.
Lupus 2005
PMID:Indications for plasma exchange in systemic lupus erythematosus in 2005. 1633 78

Efficacy of plasma exchange in patients with systemic lupus erythematosus has not been supported by the results of the first non-controlled and retrospective studies. Nonetheless, they remain relevant for some selected patients with life-threatening manifestations and/or severe therapy-resistant manifestations. They can be used as an adjuvant therapy in combination with corticosteroids and, when required, other immunosuppressant(s) for refractory renal disease, alveolar hemorrhage, some neuropsychiatric manifestations, thrombotic thrombocytopenic purpura, catastrophic antiphospholipid syndrome, hyperviscosity syndrome or symptomatic cryoglobulinemia. The use of newer technologies, like immunoadsorption, possibly in combination with recent biologics, might, in the future, offer some new perspectives for extracorporeal therapy of systemic lupus erythematosus.
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PMID:Plasma exchange for systemic lupus erythematosus. 1736 88