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)

We report the frequency of the finding of storage and hemophagocytic histiocytes in the bone marrow of patients with systemic lupus erythematosus with one or more hemocytopenias. The study was performed on bone marrows of ten patients with systemic lupus erythematosus during an episode of hemocytopenia. Four patients were not receiving any treatment and six had been taking oral prednisone (3.5 +/- 1.5 mg/day) for the previous 6 months. Hemocytopenias found were lymphocytopenia in five, thrombocytopenia in three, and neutropenia and anemia in two each. The bone marrow had variable cell content; megakaryocytes, the myeloid:erythroid ratio, as well as lymphocyte, plasma cell, and reticular cell contents were usually increased. Seven bone marrow samples showed storage histiocytes (sea-blue histiocytes and other histiocytes that contained unidentified intracytoplasmic material). These same samples revealed histiocytes phagocytosing erythoblasts, erythrocytes, polymorphonuclear neutrophils, lymphocytes or platelets. The presence of these histiocytes in all seven patients was related to a decrease in serum complement and with lupus disease activity as measured with the SLEDAI index (mean +/- SD = 2.1 +/- 1.5). The SLEDAI score of the remaining three patients, who had no histiocytes in their bone marrow, was 0, 0, and 1, respectively. The present study reveals that the bone marrow in patients with systemic lupus erythematosus and peripheral hemocytopenia contains storage and hemophagocytic histiocytes and the significance of these cells is discussed.
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PMID:Storage histiocytes and hemophagocytosis: a common finding in the bone marrow of patients with active systemic lupus erythematosus. 886 68

The hematologic manifestations of HIV infection and AIDS are common and may cause symptoms that are life-threatening and impair the quality of life of these patients. The most important of these manifestations are cytopenias. Anemia and neutropenia are generally caused by inadequate production because of suppression of the bone marrow by the HIV infection through abnormal cytokine expression and alteration of the bone marrow microenvironment. Thrombocytopenia is caused by immune-mediated destruction of the platelets, in addition to inadequate platelet production. The incidence and severity of cytopenia are generally correlated to the stage of the HIV infection. Other causes of cytopenia in these patients include adverse effects of drug therapy, the secondary effects of opportunistic infections or malignancies, or other preexisting or coexisting medical problems that may be prevalent in the HIV-infected population. Diagnosis of the mechanism and cause of the cytopenia may allow for specific management. Optimal management of the underlying HIV infection is essential, and mild cytopenia in asymptomatic patients may need no specific management. Supportive care for anemia includes the use of erythropoietin in addition to the judicious use of red blood cell transfusions. Therapy for neutropenia includes the use of the myeloid growth factors G-CSF and GM-CSF. Immune-mediated thrombocytopenia may be treated with a combination of zidovudine, corticosteroids, IVGG, and splenectomy. Platelet transfusions are sometimes needed for the treatment of thrombocytopenia caused by decreased production. Other hematologic manifestations such as hypergammaglobulinemia and lupus anticoagulants are commonly asymptomatic and usually require no specific therapy, but they can rarely cause morbidity and require specific interventions.
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PMID:Hematologic complications of human immunodeficiency virus infection and the acquired immunodeficiency syndrome. 909 37

We reviewed the side-effects of intravenous (i.v.) cyclophosphamide (CPM) pulse therapy in a group of 75 patients suffering from various autoimmune disorders (mostly systemic lupus erythematosus and vasculitis) who received a total of 451 i.v. CPM pulses, given on a monthly basis (mean +/- s.d. CPM dose per pulse: 764 +/- 217 mg; mean +/- s.d. follow-up period: 26.7 +/- 22.1 mon). Infection was the most common side-effect (30 episodes in 21 patients; 28% of the patients) but rarely required in-patient treatment (8 episodes in 7 patients; 9% of the patients). No relationship could be found between the occurrence of infection and the dose of CPM or of glucocorticoids. Other side-effects were rare. Only one patient suffered from neutropenia. Haemorrhagic cystitis was never observed nor did premature ovarian failure in the 25 female patients at risk. Four patients developed neoplasia and three died suddenly a few days after receiving a CPM pulse but the causal relationship between CPM therapy and these poor outcomes is speculative. Taken together, our data confirm in a large group of patients that i.v. CPM pulse therapy is relatively safe. In particular, the rate of severe infection requiring in-patient treatment is rare (1.8% of 451 pulses.).
Lupus 1997
PMID:Side-effects of intravenous cyclophosphamide pulse therapy. 910 32

Histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto's Disease (KFD), is a condition rarely associated with systemic lupus erythematosus (SLE). The diagnosis of KFD can precede, postdate or coincide with the diagnosis of SLE. Lymphadenopathy is a common clinical presentation of KFD and SLE, and is histologically indistinguishable in both conditions. We describe two cases of KFD associated with SLE. The diagnosis of KFD in one case was made several years before the diagnosis of SLE, and the other was simultaneous. Both showed large lymphadenopathy, but neither fever nor neutropenia. Lymph-node biopsy showed necrosis, with proliferation of histiocytes and immunoblasts, paucity of neutrophils and absence of hemathoxilin bodies. Both patients responded favourably to steroid treatment. Patients with KFD should be assessed for SLE and have long-term follow-up checking for development of SLE. KFD should be ruled out in SLE flare-up accompanied by lymphadenopathy.
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PMID:Histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto's disease, associated with systemic lupus erythemotosus. 932 32

The function of neutrophil can be evaluated by measuring oxidative metabolism using chemiluminescence, tetrazolium dye reduction or the others. Those results are not always satisfactory which would be caused by subtle difference in each preparation of the reagents and the lack of reproducibility. Recently, flow cytometric procedures for semi-quantitating superoxide production in neutrophils have been developed to evaluate their function. This procedure, which requires only small amount of whole blood, can easily and rapidly yield reproducible and reliable data. In this study, we optimized analytical conditions and then determined reference interval to evaluate neutrophil function of patients with various disorders. Optimal concentrations and incubation times of DCFH-DA and PMA were 5 mumol/l for 15 minutes and 25 micrograms/ml for 20 minutes, respectively. Production of superoxide in neutrophil was represented by relative fluorescence intensity(RFI) with assay coefficient of variance(CV) of 4.0-11.1%. Neutrophils had to be examined within 2 hours after venipuncture to obtain reliable data. Reference interval was determined as 170.4 +/- 58.7(mean +/- SD) RFI. Neutrophil function of patients with neutropenia, paroxysmal nocturnal hemoglobinuria(PNH), renal failure, systemic lupus erythematosus(SLE), myeloperoxidase deficiency, myelodysplastic syndrome(MDS), and diabetes mellitus were within the reference interval as evaluated by this method. Only neutrophils of chronic granulomatous disease, which is known to give clearly low superoxide production, showed actually decreased value. These results indicate that this procedure would be clinically useful for diagnosis of patient with impaired neutrophil function.
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PMID:[Determination of neutrophil function by measuring superoxide production with whole blood flow cytometry]. 939 45

Neutropenia is frequently observed in a variety of autoimmune disorders. As the mechanism of neutropenia in these disorders, the destruction of neutrophils by anti-neutrophil autoantibodies has been believed since elevated levels of neutrophil-associated IgG (NAIgG) have been described. However, no data exists to characterize the nature of NAIgG and show NAIgG is an anti-neutrophil autoantibodies. We investigated whether the elevated NAIgG in these patients consists of anti-neutrophil autoantibodies. The NAIgGs of 91 patients with autoimmune disorders including 50 patients with idiopathic thrombocytopenic purpura, 13 patients with systemic lupus erythematosus, 11 patients with Hashimoto's thyroiditis and 10 patients with Graves' disease were analyzed. The level of NAIgG was high in 36 of 91 patients. Elution studies were performed to determine whether NAIgG has a nature of autoantibodies. In model experiments, the ether eluate from neutrophils sensitized with neutrophil-specific alloantibody (anti-NA2) reacted with donor neutrophils, whereas the eluates from those with model immune complexes (ICs) failed. These data indicated that the ether elution technique is useful to determine whether NAIgG consists of anti-neutrophil autoantibodies. The NAIgG on patient's neutrophils was eluted with ether and the reactivity of the eluate with normal neutrophils was investigated. The eluates from 34 of 36 patients with various autoimmune disorders with elevated NAIgG level failed to react with donor neutrophils. These data indicated that the elevated NAIgG in the majority of these patients did not consist of anti-neutrophil autoantibodies, but possibly of ICs.
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PMID:Characterization of elevated neutrophil-associated IgG in various autoimmune disorders: not anti-neutrophil autoantibodies, but possibly immune complexes, bind to neutrophils. 955 Feb 87

Within a 6-year period from January 1991 to December 1996, 19 patients with Salmonella choleraesuis bacteremia were enrolled for clinical and microbiological analysis. Young children, the elderly and patients with hematological malignancy (36.8%), liver cirrhosis (26.3%), systemic lupus erythematosus (10.5%), chronic renal impairment (10.5%), and peptic ulcer (10.5%) were at high risk of this infection. The ratio of male to female was 3:1. Three cases (15.8%) were nosocomially acquired. Fever (89.5%), chills (57.9%) and anorexia (52.6%) were the most common clinical manifestations. Seven patients (36.8%) presented no gastrointestinal manifestations. Normal white blood cell count was noted in seven patients (36.8%), and neutropenia caused by underlying diseases or severe infection was found in six cases (31.6%). Various types of metastatic focal infections were found, such as septic arthritis, cutaneous infection, spontaneous bacterial peritonitis, and pneumonia. The severe immunocompromised status of patients and the high virulence of this pathogen may contribute to the high case fatality rate (21%). Higher resistance rate to commonly used antimicrobial agents was noted in ampicillin (94.7%), chloramphenicol (89.5%), and TMP/SMZ (63.8%). All strains of S. choleraesuis were susceptible to third-generation cephalosporins and fluoroquinolones. Generally, S. choleraesuis bacteremia should be taken into account in the differential diagnosis of sepsis in immunocompromised patients, even without gastrointestinal manifestations. The third-generation cephalosporins and fluoroquinolones may be the first choice for treatment of this invasive infections.
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PMID:Salmonella choleraesuis bacteremia in southern Taiwan. 1033 Jul 99

G-CSF not only functions as an endogenous hemopoietic growth factor for neutrophils, but also displays pro-Th2 and antiinflammatory properties that could be of therapeutic benefit in autoimmune settings. We evaluated the effect of treatment with G-CSF in a murine model of spontaneous systemic lupus erythematosus, a disease in which G-CSF is already administered to patients to alleviate neutropenia, a common complication. Chronic treatment of lupus-prone MRL-lpr/lpr mice with low doses (10 microg/kg) of recombinant human G-CSF, despite the induction of a shift toward the Th2 phenotype of the autoimmune response, increased glomerular deposition of Igs and accelerated lupus disease. Conversely, high-dose (200 microg/kg) treatment with G-CSF induced substantial protection, prolonging survival by >2 mo. In the animals treated with these high doses of G-CSF, neither the Th1/Th2 profile nor the serum levels of TNF-alpha and IL-10 were modified. Despite the presence of immune complexes in their kidney glomeruli, no inflammation ensued, and serum IL-12 and soluble TNF receptors remained at pre-disease levels. This uncoupling of immune complex deposition and kidney damage resulted from a local down-modulation of FcgammaRIII (CD16) expression within the glomeruli by G-CSF. Our results demonstrate a beneficial effect of high doses of G-CSF in the prevention of lupus nephritis that may hold promise for future clinical applications, provided caution is taken in dose adjustment.
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PMID:Granulocyte-colony stimulating factor treatment of lupus autoimmune disease in MRL-lpr/lpr mice. 1052 19

SLE is associated with the production of autoantibodies to self-constituents. In particular, certain ribonucleoprotein particles are targeted. Despite the multitude of autoantibodies produced and the remarkable concentrations of these antibodies in the sera of SLE patients, there have been little data that the autoantibodies found in SLE are involved in the pathogenesis of disease or its manifestations. The present work demonstrates that anti-Ro (or SSA) is associated with granulocytopenia, binds the surface of granulocytes and fixes complement to this membrane surface. Binding is a property of anti-Ro Fab fragments and can be inhibited by 60-kD Ro. However, the antigen bound on the surface of granulocytes is a 64 000 mol. wt protein that is a novel autoantigen in SLE. As suggested by inhibition studies, sequence identity between 60-kD Ro and eight tandem repeats in the 64-kD antigen may be responsible for the observed serologic cross-reactivity. These data imply that anti-Ro antibodies that also bind the 64-kD protein mediate neutropenia in patients with SLE.
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PMID:Association of neutropenia in systemic lupus erythematosus (SLE) with anti-Ro and binding of an immunologically cross-reactive neutrophil membrane antigen. 1075 85

Thrombosis of upper extremity arteries is most commonly due to atherosclerosis of the proximal subclavian artery, trauma, or catheter-related injury. In the absence of an identifiable cause, a search for a hypercoagulable state is indicated. Hematologic manifestations of human immunodeficiency virus (HIV) infection and AIDS are frequent occurrences (Coyle TE. Med Clin N Am 1997;81:449-476). The most important of these are cytopenias (anemia, neutropenia, and thrombocytopenia). The incidence and severity of cytopenia are generally correlated to the stage of the HIV infection. In addition, various coagulation abnormalities have been reported in HIV-infected patients. Apart from thrombocytopenia, these have included a prolonged APTT due to the presence of lupus anticoagulant, an increased prevalence of protein S and heparin cofactor II deficiency, and hypoalbuminemia-related fibrin polymerization defects (Toulon P. Ann Bio Clin (Paris) 1998;56:153-160). HIV infection has also been associated with endothelial dysfunction. Although for the most part asymptomatic, elevated D-dimer levels have been found in HIV-infected patients, suggesting the existence of a prethrombotic state. In fact, clinical thrombosis eventuates in 2% of these patients (Toulon, 1988). Documented thromboses have involved both veins and arteries. We hereby present a patient who developed an acute thrombosis of his brachial artery as the initial manifestation of HIV infection.
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PMID:Acute brachial artery thrombosis as the initial manifestation of human immunodeficiency virus infection. 1081 96


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