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 ability of sera from patients with systemic lupus erythematosus (SLE) and Felty's syndrome to induce increased adhesiveness of normal human neutrophils (PMN) was investigated. PMN from normal healthy donors were incubated in sera from 19 patients with active SLE, 12 with inactive SLE, 20 with Felty's, 24 with rheumatoid arthritis, and 34 normal persons. After incubation, the degree of adherence of the PMN to human endothelial cells in culture, their aggregation, and superoxide (O2-) generation were determined. Sera from patients with both active SLE and Felty's syndrome induced significantly increased PMN adherence to endothelial cells and PMN aggregation in vitro, compared with normal sera. This increased adherence to endothelial cells was maintained after heat treatment (56 degrees C for 30 minutes) of the sera. In O2- generation experiments, sera from patients with active SLE induced significantly increased O2- release from normal PMN using both fresh and heat-treated sera. Sera from Felty's patients demonstrated the same effect with heat-treated sera but not ith fresh sera. When sera from patients with active SLE and Felty's syndrome were used, all three parameters correlated significantly with each other in individual patients. In contrast, sera from the 12 patients with inactive SLE and 24 rheumatoid arthritis patients without Felty's failed to induce significant differences in the three parameters studied when compared with 34 normal controls. Fractionation of 3 SLE sera and 1 Felty's serum on Sephadex G-200 demonstrated that the adherence enhancing factor was present in both IgG and IgG-excluded fractions. The observed increased adhesiveness of PMN induced by SLE and Felty's sera may, at least in part, contribute to the neutropenia which is common in these diseases. Increased O2- release associated with PMN adherence may contribute to endothelial cell damage and vascular injury, which is also a common manifestation of these diseases.
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PMID:Increased endothelial cell adherence, aggregation, and superoxide generation by neutrophils incubated in systemic lupus erythematosus and Felty's syndrome sera. 629 13

A 58-year-old patient with neutropenia due to SLE developed spondylitis of the lumbar region caused by Candida albicans. The spondylitis was probably superinfected with Staphylococcus aureus. The initial one month's intravenous combination therapy with amphotericin B and flucytosine was discontinued because of fever reactions to amphotericin B, suspected myelosuppressive effect of flucytosine and insufficient clinical response. This therapy was followed by four months of oral ketoconazole and clindamycin with good results and without any side-effects.
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PMID:Hematogenous Candida spondylitis. A case report. 632 Jun 2

Activated complement components and immune complexes cause neutrophil (PMN) aggregation in vitro and in vivo, as in dialysis-induced neutropenia and adult respiratory distress syndrome. To investigate the possible role of PMN aggregation in systemic lupus erythematosus (SLE), we studied the capacity of 59 sera from 53 patients to induce aggregation of normal PMN in vitro. Neutrophil aggregating activity (NAA) was present in the sera of 26 of 28 patients with active SLE. The mean NAA in this group was significantly greater than that found in 13 patients with inactive SLE, 20 patients with rheumatoid arthritis, and 17 normal controls. In patients with SLE there was a positive correlation between disease severity and the quantitative measure of NAA. NAA did not correlate with serum C3 or C4 nor with the presence or absence of circulating immune complexes. High levels of NAA were particularly characteristic of central nervous system lupus. These data suggest that the formation of intravascular leukoaggregates may contribute to morbidity in SLE.
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PMID:Neutrophil aggregation induced by sera from patients with active systemic lupus erythematosus. 684 25

We applied a radiolabeled antiglobulin test to a study of patients with a variety of neutropenic disorders. After defining the nature of the interaction of radiolabeled anti-IgG with the neutrophil, we studied 16 patients with neutropenia of uncertain etiology and adequate bone marrow granulocyte precursors. Twelve of these 16 patients had increased neutrophil-associated IgG (PMN-IgG). Patients with the highest levels of PMN-IgG had the lowest neutrophil counts. The majority of patients with neutropenia and increased PMN-IgG had an underlying immunologic disorder that included immune thrombocytopenic purpura in 5 patients and autoimmune hemolytic anemia in 1 patient. In some patients, elevated PMN-IgG preceded other evidence for immunologic disease. The direct antiglobulin test helped to distinguish neutropenic patients with increased PMN-IgG both from patients with neutropenia due to a known nonimmune disorder and from nonneutropenic patients with rheumatoid arthritis or systemic lupus erythematosis. Each of four patients with increased neutrophil-associated IgG treated with systemic corticosteroids responded clinically with an associated fall in neutrophil IgG and a rise in the circulating neutrophil count. The radiolabeled antiglobulin test appears useful in defining a subpopulation of patients with neutropenia due to an underlying immunologic disorder.
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PMID:Granulocyte-associated IgG in neutropenic disorders. 697 94

The migration inhibiting factor (MIF) test was performed on 27 patients with chronic neutropenia using acute myeloblastic leukemia (AML) blasts and normal lymphocytes as antigens. Eight patients reacted positively to AML blasts, and the natural history of their disease differed from that of the remaining neutropenic patients: two of the eight patients developed AML and one showed evidence of preleukemic changes. Recurrent severe infections were common and one patient in this group developed systemic lupus erythematosus. The MIF test toward AML blasts may be regarded as a warning sign of a preleukemic state among neutropenic patients.
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PMID:Macrophage migration inhibition assay as a means of detecting lymphocyte sensitization to leukemic myeloblasts in neutropenic patients. 703

This paper presents the clinical features of 14 patients in whom systemic lupus erythematosus (SLE) was diagnosed for the first time after the age of 45 years. The onset was insidious and the diagnosis was delayed in most patients, the mean duration of symptoms before diagnosis being five years. Clinical features in this group of patients differed from classic descriptions of SLE in that there was a high incidence of neuropsychiatric disturbances and low incidence of serositis, while non-specific complaints of fever, weight loss, and malaise were often the only presenting clinical features. Factors associated with disease activity, such as elevated ESR, neutropenia, and thrombocytopenia were less frequency encountered than in younger patients. In 7 of 12 patients immunoglobulins and complement components were detected in basement membrane of normal skin. Diagnosis in this age group is difficult, and it is likely that SLE goes unrecognised in a number of older patients with non-specific complaints.
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PMID:Systemic lupus erythematosus in later life. 704 9

We have studied the infective complications in a group of 75 patients with immunologically-mediated disease who required high dose immunosuppression. There were 22 patients with anti-glomerular basement membrane antibody disease, 19 patients with systemic lupus erythematosus, 18 with wegener's granulomatosis and 16 patients with other forms of systemic vasculitis. The infection rate was 3.69 infections/patient, or 0.74 infections/patient/week of immunosuppression. Bacteria were the commonest infecting organisms (76.1 per cent); serious opportunist viral and fungal infections were less frequent (10.7 per cent) but opportunist pneumonias were an important cause of death. Sixteen patients died (21 per cent) and in 10 of these (62.5 per cent) death was considered to be primarily due to infection. Analysis of six aspects of host susceptibility to infection (age, renal function, dose of prednisolone, cyclophosphamide and azathioprine, and number of plasma exchanges) revealed no single factor as predisposing to infection in the whole group, but in 23 patients who suffered severe infective complications, renal impairment and increasing doses of prednisolone were associated significantly, particularly in combination (p = 0.06). Cyclophosphamide was associated with infection only in the presence of neutropenia, which was rare (13 infections in nine patients). The duration of plasma exchange was not related to the frequency of infection. Fifty patients needed an arteriovenous shunt to provide vascular access for haemodialysis or plasma exchange, and septicaemia occurred in 13; only two episodes of septicaemia were seen in patients without a shunt.
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PMID:Infection and immunosuppression. A study of the infective complications of 75 patients with immunologically-mediated disease. 711 69

The combination of idiopathic thrombocytopenic purpura and autoimmune hemolytic anemia is rare in childhood. Among 164 instances of ITP and 15 instances of AHA, 11 patients were found to have this combination. Three were found to have systemic lupus erythematosus, one had aplastic anemia, and seven had Evans syndrome. Neutropenia, at times associated with bacterial infections, occurred in four of the latter patients. Unlike most cases of ITP or AHA in childhood, the clinical course of Evans syndrome is usually chronic and relapsing. Treatment including corticosteroids, splenectomy, and immunosuppressive agents has been generally unsatisfactory. In view of the frequent presence of antibodies directed at red blood cells, platelets, neutrophils, and lymphocytes, immunopancytopenia may be a better term for this condition.
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PMID:Evans syndrome in childhood. 719 90

Since their initial description in 1957, the interferons (IFNs) have been increasingly used to treat a wide array of diseases. Acute adverse effects, i.e. 'flu-like' syndromes, hypo- or hypertension, tachycardia, headache, myalgias and gastrointestinal disorders, occur within the first hour or day after starting treatment. They are seldom treatment-limiting and are easily manageable. Sub-acute and chronic effects develop after several days, usually within 2 and 4 weeks of therapy. The most typical is neurological toxicity, including fatigue/asthenia, and behavioural and cognitive changes. Such symptoms may seriously impair quality of life and result in treatment discontinuation. Seizures have seldom been described. Other infrequent central nervous system adverse effects include vertigo, cramp and oculomotor nerve paralysis. Distal paraesthesias and peripheral neuropathy have been reported. IFN-associated autoimmunity is quite rare but a matter of concern. Biological or clinical manifestations usually require several months to become apparent. Autoantibodies have been shown to develop in most patients but have been inconsistently associated with clinical symptoms of systemic lupus erythematosus, rheumatoid-like arthritis and thyroiditis. Both hypo- and hyperthyroidism have been described but are usually reversible. Other infrequent autoimmune reactions include diabetes, pemphigus and worsening of multiple sclerosis. Although several patients present with a pre-existing autoimmune disorder, no predisposing factor has been clearly established. While hypotension and tachycardia are the most frequent acute cardiovascular complications, a few additional cases of cardiac arrhythmias and myocardial ischaemia have been reported after a short course or several weeks of treatment. These latter complications do not appear to be dose-dependent or age-related. Isolated cases of congestive heart failure have also been described. Mild proteinuria has been observed in 15 to 25% of patients, but acute renal toxicity is uncommon. A transient rise in serum aminotransferase levels is frequently noted during the first stage of therapy, especially in patients receiving the highest dosages. Direct hepatotoxicity is extremely rare. Autoimmune hepatitis, which is ill-diagnosed as chronic viral hepatitis, and de novo induction of autoimmune hepatitis, account for the majority of liver diseases. Haematotoxicity is relatively common but mild to moderate, and develops gradually during the first weeks of treatment. Neutropenia is the most common haematological toxicity, but is usually not dose-limiting and resolves rapidly upon drug discontinuation. Myelosuppression, autoimmune and immune allergic haemolytic anaemias and thrombocytopenias have seldom been described. Cutaneous adverse effects comprised nonspecific erythema and hair loss and, less frequently, vasculitis, local ulcerations at the site of injection and exacerbation of psoriasis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Clinical toxicity of the interferons. 751 63

Monthly intravenous cyclophosphamide (IVCY) has been a recommended therapy for severe lupus nephritis or neurological flare-ups in lupus patients. But the optimal treatment regimen and duration remains unknown. We report our experience in an open study of 37 patients treated with monthly IVCY. Thirty-four women and 3 men, mean age 35.5 with a mean disease duration of 59 months, with a mean 5.7 ACR criteria for SLE were analysed. 27 (group I) had lupus nephritis (OMS Class III or IV) and 10 had neurological involvement (group II). In group I, after six months of IVCY, a significant improvement was noticed in the UCH-Middlesex clinical index (2.9 pts vs 7.8), the proteinuria (3.12 g/d vs 5.4), complement and split fractions (CH50 98.4 vs 48.9%; C3 877 vs 600 mg/l; C4 177 vs 128 mg/l), the level of anti-DNA antibodies (67.5 vs 775 UI/ml) and the daily dose of steroids (22 vs 44 mg/d). Kidney biopsies showed a reduction of the activity index despite a slight increase of the chronicity index (4.1 vs 6.3 pts and 5.5 vs 3.6 pts). Those results were not maintained at medium and long term. Moreover five patients presented with worsening of renal function during IVCY treatment and two patients relapsed after the end of the treatment. In group II significant improvement was noticed at six months concerning the clinical index (1.77 pts vs 7.17) and the daily dose of steroids, 3 patients died because of cerebral vasculitis refractory to IVCY. Adverse effects are frequent: infectious (25 among 20 patients), hemorrhagic cystitis (2 events in 1 patient), gastrointestinal side effects were common (12/37 patients). Were also noticed: neutropenia (5/37), transient amenorrhea (4/28), drug induced menopausis (2/28). Overall mortality is important (7/37), uneffectiveness of IVCY was noticed in 5 patients, flares occurred in 8 patient during or after stopping treatment. IVCY seems efficacious if given at the very beginning of the flare. Its usefulness is obvious at six months among clinical and biological data in patients with severe lupus nephritis or neurological flare. It seems that long term outcome on the renal function is not modified.
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PMID:[Treatment of acute systemic lupus erythematosus with intravenous infusions of cyclophosphamide. Value and limitations]. 802 84


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