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 expression of HLA class I was assessed on erythrocytes by haemagglutination with monoclonal antibodies to monomorphic epitopes on the heavy and light (beta 2-microglobulin) chains. Previously, enhancement of HLA class I expression was observed on erythrocytes of many patients with systemic lupus erythematosus (SLE) and chronic lymphatic leukaemia (CLL), and we have now tested erythrocytes from patients (and 130 normal controls) with other auto-immune diseases and renal and haematological disorders. The striking enhancement in patients with SLE and CLL was confirmed. A significant increase in expression was also observed in aplastic anaemia patients following bone marrow transplantation and in renal patients with primary glomerulonephritis who had received a transplant. No class I was expressed by erythrocytes from many patients with inherited haemoglobinopathies and high reticulocyte counts, which suggests that the enhancement in SLE patients cannot be accounted for by immature or young erythrocyte populations. The distribution of HLA-A and -B types in the patients with enhanced class I expression did not relate to those antigens previously detected more frequently on erythrocytes, B7(Bga), B17(Bgb), A28(Bgc), B8 or A10, and the enhancement was not associated with any particular HLA types.
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PMID:Erythroid HLA class I expression in 300 patients with haematological, renal and rheumatological disorders. 239 73

During the last few years the use of intravenous immunoglobulin (IVIG) has attracted increasing interest for the treatment of patients who do not have a classical humoral antibody deficiency syndrome. In certain situations this approach has revolutionized medical management, e.g. in immune thrombocytopenia. In other areas, such as in Kawasaki's syndrome, IVIG therapy have been shown to be highly beneficial in preventing long term disease sequelae by some investigators, but the field remains controversial. Conditions under which IVIG therapy has been shown to be of potential benefit are: (1) intractable childhood epilepsy; (2) autoimmune diseases, e.g. myasthenia gravis, systemic lupus erythematosus, idiopathic thrombocytopenic purpura, idiopathic neutropenia and aplastic anemia; (3) atopic allergy with IgG subclass deficiency including bronchial asthma; (4) in severe infections in combination therapy with antibiotics and as an antipyretic; (5) in Kawasaki's disease; (6) in multiple myeloma and chronic lymphocytic leukemia. Oral and intraventricular administration of IVIG have also been tried, the former for severe diarrhea and the latter to try to rescue the central nervous system from damage by a pathogen. Carefully controlled clinical trials are needed to establish the efficacy of gamma-globulin therapy in these and other conditions.
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PMID:New and controversial uses of intravenous gamma-globulin. 245 9

16 patients with transfusion-dependent, life-threatening bone marrow failure (14 with severe aplastic anaemia, 1 with systemic lupus erythematosus and 1 with pure red cell aplasia) were treated with cyclosporin-A (Cy-A) after either lack of response to conventional immunosuppression with antithymocyte-globulin/high-dose methylprednisolone for 95 to 1190 days (median 186.5) (group I, 8 patients) or as a primary treatment due to ineligibility for conventional immunosuppression (group II, 8 pat.). Cyclosporin-A was given orally to maintain trough levels of 200 to 300 ng/ml (RIA). In group I, 6 out of 8 patients responded 30 to 480 d (median 53) and are currently alive 627 to 1482 d (median 731) after initiation of Cy-A, respectively. In 3 of the responders Cy-A has been withdrawn, without relapse. In group II, 5 of 8 patients responded 26 to 170 d (median 63) and are currently alive 142 to 697 (median 420) d following initiation of Cy-A, respectively. These data indicate a place for cyclosporin-A in the management of patients with life-threatening bone marrow failure in whom a) immunosuppressive therapy with antithymocyte-globulin and high-dose methylprednisolone had failed and b) who are not candidates for vigorous immunosuppression or bone marrow transplantation, for medical or other reasons.
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PMID:Oral cyclosporin-A is effective treatment for untreated and also for previously immunosuppressed patients with severe bone marrow failure. 250 46

A non-complement-dependent antibody that inhibits bone marrow proliferation in vitro was detected in the serum of a patient with systemic lupus erythematosus and aplastic anemia. The patient recovered from the aplastic anemia following treatment with corticosteroids and plasmapheresis. The antibody could no longer be detected after this recovery. This case demonstrates that a rare, but potentially lethal, complication of systemic lupus erythematosus is aplastic anemia due to an autoantibody. Results of the experiments suggest a mechanism for, and treatment of, similar cases.
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PMID:An antibody that inhibits in vitro bone marrow proliferation in a patient with systemic lupus erythematosus and aplastic anemia. 275 21

The graft-versus-host reaction (GVHR) in both mice and humans can lead to the development of a broad spectrum of clinical and pathological symptoms. These symptoms are strikingly similar to those of a number of diseases of proven or presumed immunological origin, such as systemic lupus erythematosus (SLE), other collagen vascular diseases, lymphoproliferative disease, and aplastic anemia. The purpose of our investigation was to describe the immunological and pathological events that take place in the course of graft-versus-host disease (GVHD) and to gain insight into the cellular mechanisms underlying these events. To this end, a model was employed in which nonirradiated F1 mice were used as recipients of parental lymphoid cells. By pathological manifestations, 2 basic forms of GVHD can be distinguished in such non-irradiated F1 recipients: One is acute GVHD which is often lethal. It is characterized by a variety of suppressive (hypoplastic) pathological symptoms, including a severe hypoplasia of the lymphohemopoietic system accompanied by aplastic anemia and hypogammaglobulinemia. The other basic form is characterized by stimulatory symptoms, such as persistent lymphoid hyperplasia, formation of autoantibodies, and development of pathological symptoms reminiscent of SLE and other collagen vascular diseases. The suppressive pathological graft-versus-host (GVH) symptoms are caused by T suppressor/killer (TS/K) cells of the donor which react towards allogeneic class-I-structures of the F1 recipient's major histocompatibility complex (MHC). The stimulatory pathological GVH symptoms, by contrast, are caused by donor T helper (TH) cells which react toward the recipient's allogeneic class-II-MHC structures. The possible implications of these observations for the pathogenesis of a number of GVH-like diseases in humans are discussed. The hypothesis is advanced that some of these GVH-like conditions, which arise either e causa ignota or after exposure to certain viruses or drugs, are caused by T lymphocytes reacting against self-MHC structures on lymphohemopoietic cells that were rendered "foreign". By analogy to GVHD, it is conceivable that the development of either stimulatory or suppressive GVH-like symptoms in individuals exposed to a given virus or sensitizing drug depends not on the etiologic agent per se, but on whether the predominant response is made by the individual's TH or TS@K cells. This, in turn, might depend on whether the agent becomes immunogenic in combination with class-II or class-I alloantigens.
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PMID:Pathogenesis of graft-versus-host reactions (GVHR) and GVH-like diseases. 315 4

Soft tissue infections were seen in 25 patients with underlying malignancy and immunosuppressive disease. The primary disease included leukemia, lupus, aplastic anemia, lymphoma, carcinoma and myeloma. Infectious sites included the perianal area, gluteal, chest wall, extremity and the vulva. Eighty per cent of the infectious episodes occurred in patients who were granulocytopenic. Initial presentation was of local tenderness and redness. Fluctuation and discoloration were present in nine patients who were also hypotensive. Local drainage in five patients resulted in the death of two (20%). Overall, the mortality was 3/25 (12%). Wide debridement and drainage and appropriate antibiotic therapy resulted in the death of 1/20 (5%) patients. Hypotension, discoloration and fluctuation were found to be late signs in these patients. Soft tissue infections in the compromised host present subtly and progress to death if treatment is delayed. Temperature elevation and localized tenderness and erythema are indications for broad spectrum antibiotics and extensive intraoperative drainage and debridement.
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PMID:Soft tissue infections in the compromised host. 338 98

Over a period of nine years we observed 52 children with acute neurological symptoms which were caused by a cerebrovascular disease. Fourteen patients had congenital vascular malformations, most frequently AV-angiomas (9 patients). A Sturge-Weber-Syndrome and a venous angioma were found in two cases and one patient had an aneurysm of the middle cerebral artery. Thirty-eight patients had acquired cerebrovascular diseases such as ischaemic infarctions (22), intracranial haemorrhages without vascular malformations (14) and thromboses of the dural sinus (2). The cerebral infraction was a complication of a congenital heart disease in 8 children, two others suffered from chronic renal insufficiency and were on haemodialysis. Two children had a trauma of the internal carotid artery and in one patient a large haemorrhagic infarct was caused by hypernatremic dehydration. In 9 patients (6 females, 3 males) no obvious aetiology of the infarct could be found. However, in most of these cases a nonspecific febrile illness preceded the neurological manifestations. The thrombosis of the dural sinus occurred in a 6-week old previously healthy infant and in a 3-year old boy as a complication of a nephrotic syndrome. Intracranial haemorrhages (without cerebrovascular malformations) occurred in 14 patients, mainly as a complication of haematological diseases (acute lymphatic leukaemia, severe aplastic anaemia, haemophilia A, lupus erythematodes). Four children had spontaneous intracerebral haemorrhages without obvious causes. The prognosis for survival was good in children with infarcts, but persisting neurological deficits were more severe than in children with haemorrhages. At the acute stage the lethality was higher in children with intracranial haemorrhages.
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PMID:[Cerebrovascular diseases in childhood--etiology, clinical aspects and prognosis]. 395 16

We describe two female patients with systemic lupus erythematosus (SLE) who developed severe aplastic anemia. Although each patient had received multiple medications including diphenylhydantoin, the relationship to these drugs to the development of marrow aplasia was unclear. After administration of an oral androgen (oxymethalone) and corticosteroids, there was complete hematologic recovery. Both patients relapsed when oxymethalone was withdrawn, and both recovered when androgen therapy was reinstituted, with or without high-dose prednisone. In both patients, there was complete reversal of pancytopenia despite the presence of initially severe marrow aplasia (less than 10% cellularity). However, in both cases, prolonged androgen therapy (2 months) was required before hematologic improvement occurred. Androgens are known to stimulate hematopoiesis in man, and they appear to influence immune function in a mouse model of SLE. Thus androgens may be particularly useful in the treatment of SLE-associated aplastic anemia.
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PMID:Aplastic anemia complicating systemic lupus erythematosus: response to androgens in two patients. 646 36

Hematologic complications of systemic lupus erythematosus (SLE) usually involve peripheral destruction of blood elements. We report a case of SLE-associated aplastic anemia in which an IgG complement-dependent antibody, obtained from the patient's disease-phase serum but not remission-phase serum, suppressed growth of granulocyte-macrophage progenitor cells from bone marrow of normal donors in vitro. Therapy with plasmapheresis and immunosuppression resulted in lasting remission.
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PMID:Serum inhibitor in systemic lupus erythematosus associated with aplastic anemia. 673 7

An immunoenzyme assay for detection of platelet antibodies and suitable for routine use is described. Purified rabbit IgG anti-human IgG antibodies are conjugated to beta-galactosidase with meta-maleimidobenzoyl-hydroxysuccinimide ester as a bifunctional reagent and o-nitrophenyl-beta-galactopyranoside as a substrate to evaluate the enzymatic activity of the labeled antiglobulin. The sera of 26 patients suffering from various diseases (acute leukemia, aplastic anemia and systemic lupus erythematosus) and 40 control subjects were assayed with the enzyme-labeled reagent and, for comparison, with an indirect immunofluorescence technique. Half of these patients had never been transfused. Platelet antibodies were detected by both assays in all the transfused patients except one, and in 3 out of 13 non-transfused patients. The sera of all the control subjects were negative. Quantitation of platelet antibodies was obtained by a sensitive antiglobulin absorption technique. A method for standardization of the reagents allowing comparison of results obtained in the same patient at different times and suitable for long-term follow-up studies is also described.
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PMID:An immunoenzymatic assay for the detection and quantitation of platelet antibodies: the platelet beta-galactosidase test (PGT). 679 85


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