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)

Serum from a patient with inactive systemic lupus erythematosus (SLE) and ibuprofen-induced transient neutropenia was used as a probe to define further the control of human polymorphonuclear leukocyte (PMN) exocytosis and superoxide (O2-) generation. Thirty-minute preincubation of normal PMNs with 10-50% v/v of this serum, followed by washing, produced a specific dose-related suppression of leukotriene B4 (LTB4)-elicited beta-glucuronidase and lysozyme release of up to 45% and 30% respectively. If cells were not washed, the inhibition increased to 60% and 40%. Superoxide production stimulated by LTB4 was unaffected. The serum had no effect on formyl-met-leu-phe (FMLP) or phorbol myristate acetate-stimulated O2- or exocytosis. O2- and beta-glucuronidase release elicited by zymosan-treated serum (ZTS) were both decreased by 15%, but there was no increased inhibition seen if cells were not washed, or if the time of preincubation was increased from 7 to 30 min. In contradistinction, the serum inhibition of LTB4 exocytosis did show time dependence. Serum obtained when the patient was not leukopenic and sera from 6 normal controls, 2 patients with inactive SLE, 1 patient with SLE and chronic leukopenia, and 2 controls taking ibuprofen did not influence any PMN function. The serum inhibition of ZTS-induced functions was qualitatively similar to that observed when PMNs were preincubated and desensitized with ZTS in vitro. Selective inhibition of LTB4 exocytosis was not seen when PMNs were desensitized with LTB4 in vitro. These observations indicate that LTB4-elicited O2- and exocytosis can be independently and specifically regulated. The cellular site at which this serum factor acts is not clear, but the current studies strongly suggest that this inhibition is not due to in vitro deactivation by LTB4 activity.
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PMID:Independent regulation of leukotriene B4-elicited polymorphonuclear leukocyte exocytosis and superoxide generation by a serum factor. 303 8

SLE is a syndrome characterized by diverse clinical signs and symptoms, including rash, serositis, nephritis, central nervous system disease, thrombocytopenia, and leukopenia. These and other manifestations are present in patients with immune abnormalities indicative of B cell hyperactivity: hypergammaglobulinemia, increased amounts of antibodies reactive with self-determinants (characteristically including nuclear antigens), and increased numbers of circulating antibody-producing cells. It is this immune hyperactivity, inclusive of anti-self responses, which groups the patients with diverse clinical manifestations into a single syndrome called lupus.
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PMID:Pathogenesis of systemic lupus erythematosus. 304 91

The effects of short-course plasma exchange (PE) followed by tapering dose prednisone therapy was assessed in six children with systemic lupus erythematosus (SLE) and severe glomerulonephritis. All patients received pulse methylprednisolone therapy and three patients were treated with cytotoxic drugs prior to PE, but none had exhibited a good response. PE resulted in a rapid and sustained (greater than 1 year) remission of renal failure in the three patients with renal failure and severe glomerulonephritis. All six patients had severe nephrotic syndrome and five of six experienced a complete and sustained (greater than 1 year) remission post-PE (the sixth patient has greater than 4 month remission at the time of writing). Of interest was the high frequency of membranous [World Health Organization (WHO) Type V] and mixed membranous and diffuse proliferative SLE nephritis (WHO Type IV) on renal biopsy (4/6 patients). In addition, the severe anemia and leukopenia seen in most patients responded favorably to PE. Five of the six patients are currently managed on low-dose prednisone (0.25-0.5 mg/kg) every other day. One patient progressed to renal failure and dialysis more than 1 year post-PE. One patient required cytotoxic drug therapy post-PE (6 weeks). No significant complications were encountered; in fact, all patients eventually received their PE treatments as outpatients. We conclude that PE may provide a safe and effective therapeutic option for the treatment of severe progressive SLE nephritis in selected children who are unresponsive to steroid or cytotoxic drug therapy.
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PMID:Plasma exchange improves the glomerulonephritis of systemic lupus erythematosus in selected pediatric patients. 315 88

Systemic lupus erythematosus (SLE) is considered to be the quintessential autoimmune disease. It has not been possible to induce SLE in animal models by DNA immunization or by challenge with anti-DNA antibodies. We herewith report a murine model of SLE-like disease induced by immunization of C3H.SW female mice with a common human monoclonal anti-DNA idiotype (16/6 idiotype). Following a booster injection with the 16/6 idiotype, high levels of murine anti-16/6 and anti-anti-16/6 antibodies (associated with anti-DNA activity) were detected in the sera of the immunized mice. Elevated titers of autoantibodies reacting with DNA, poly(I), poly(dT), ribonucleoprotein, autoantigens [Sm, SS-A (Ro), and SS-B (La)], and cardiolipin were noted. The serological findings were associated with increased erythrocyte sedimentation rate, leukopenia, proteinuria, immune complex deposition in the glomerular mesangium, and sclerosis of the glomeruli. The immune complexes in the kidneys were shown to contain the 16/6 idiotype. This experimental SLE-like model may be used to elucidate the mechanisms underlying SLE.
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PMID:Induction of a systemic lupus erythematosus-like disease in mice by a common human anti-DNA idiotype. 325 23

A 17 year old girl with chronic relapsing pancreatitis since the age of 11 developed focal seizures, leukopenia, anemia and thrombozytopenia, signs suggesting lupus erythematodes. All immunologic investigations, including tests for Anti-DNS-Antibodies were negative, however. Therapy with prednisone and azathioprine resulted in clinical remission. Reduction of the immunosuppression lead to a severe crisis, which was controlled by plasmaseparation and cyclophosphamide. Subsequent analysis of circulating immune complexes revealed bound anti-DNS-antibodies.
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PMID:[Acute recurrent pancreatitis as the main symptom of lupus erythematosus disseminatus in childhood]. 325 73

In a clinical and serological study performed on a large series of patients with different connective tissue disorders, only sera from patients with systemic lupus erythematosus (SLE) contained two precipitin systems (DA1 and DA2) directed against nuclear proteins present in a total extract of human spleen. DA1 antibody appeared in 5% of SLE patients and was always associated with DA2 antibody which was found in about 22% of SLE patients. Sera monospecific for DA1 and DA2 antibodies gave a speckled staining pattern on both rat kidney and HEp2 cells. DA1-positive patients showed a higher incidence of photosensitivity (p less than 0.001), hepatosplenomegaly (p less than 0.001), hemolytic anemia (p less than 0.05), CNS involvement, vasculitis, and diffuse proliferative glomerulonephritis than DA1-negative ones. DA2-positive patients showed an increased incidence of leukopenia (p less than 0.05) and thrombocytopenia (p less than 0.01) than controls. While several indirect evidences suggest that DA1 precipitin might be identical to the previously described MA antibody, DA2 precipitin appears as a new system in SLE. The serological association between DA1 and DA2 antibodies might be analogous to the well known ones between antibodies to Sm and nRNP and to SSA/Ro and SSB/La.
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PMID:Detection of two associated precipitating autoantibodies (DA1 and DA2) in sera from patients with systemic lupus erythematosus. 326 26

Occasionally patients with overlapping features of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), termed "rhupus," have been encountered. We wanted to ascertain the frequency of such patients and determine whether they represent a unique overlap syndrome. Of approximately 7000 new patients evaluated over 11 years, we identified six patients who had, on the average, 6.7 American Rheumatism Association criteria for RA and 4.2 criteria for SLE. Criteria for RA included chronic symmetric arthritis with morning stiffness (six patients); subcutaneous nodules (two patients); positive rheumatoid factors test (four patients); and radiologic erosions (four patients). The criteria for SLE included malar rash (three patients); discoid lupus erythematosus (two patients); biopsy-proved nephritis (one patient); photosensitivity (one patient); leukopenia/thrombocytopenia (four patients); positive antinuclear antibodies or lupus erythematosus cell test (six patients); hypocomplementemia (two patients); and abnormal results from skin biopsy (two patients). During observations of up to ten years, the conditions of three patients were stable or improved, one died, and two were unavailable for follow-up. Patients usually did not have conditions that evolved to classic rheumatic disease patterns. Rhupus was not common and did not occur more frequently (0.09% prevalence among our patients) than expected from chance concurrence of SLE and RA (calculated at 1.2%). These observations confirm that rhupus indeed exists as a syndrome manifested by patients sharing features of probable coincidental concurrence of RA and SLE, but not as a unique clinical pathologic or immunologic syndrome. Appreciation of these patients with rhupus is important since their therapy and outcome differ from those having RA or SLE alone.
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PMID:'Rhupus' syndrome. 338 9

Antinuclear antibodies (ANA) are found in the majority of patients with systemic lupus erythematosus (SLE). We report here the only documented case, out of a series of 38 patients, in which SLE was diagnosed in spite of the fact that we failed to demonstrate any type of autoantibodies. A 25-year-old black woman presented with 6 of the 11 criteria of the American Rheumatism Association for classification of SLE, between August 1984 and April 1985, i.e. malar rash, photosensitivity, arthritis, pleurisy and pericarditis, renal insufficiency and nephrotic syndrome, anemia and leukopenia. Renal biopsy revealed mesangial glomerulonephritis, tubulonephritis and many tubuloreticular inclusions in the capillary endothelium highly suggestive of SLE. Four ANA determinations were performed during the 8 months of observation which were all negative, as were all other antibodies (anti-nDNA, -Sm, -RPN, -Ro, -La). The outcome was very favourable under prednisone and cyclophosphamide. In the rare cases of ANA negative SLE (5-10%) photosensitive dermatitis is the prominent feature and renal or central nervous system involvement is less frequent. Those patients usually have other types of autoantibodies (especially anticytoplasmic) which was not the case in our patient. This indicates that the absence of autoantibodies does not rule out SLE.
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PMID:[Disseminated lupus erythematosus without antinuclear antibodies or other autoantibodies]. 349 6

To determine whether patients with systemic lupus erythematosus undergoing long-term peritoneal dialysis have persistent clinical and serologic remissions, the clinical courses of eight patients with end-stage renal disease in whom peritoneal dialysis was begun at Rush-Presbyterian-St. Luke's Medical Center between 1981 and 1986 were analyzed. Patients were followed for a mean of 90.1 +/- 28.8 months before dialysis and 20.8 +/- 4.7 months after the initiation of dialysis. Disease activity was quantified for each individual in terms of "flares" per year before and after the initiation of peritoneal dialysis, the means of which were 0.66 +/- 0.46 and 0.94 +/- 0.28, respectively. Comparison of these rates showed no statistical difference. Seven of the eight patients had at least one flare while receiving peritoneal dialysis, all of which required prednisone therapy (mean 31.3 mg per day). The clinical manifestations included fever, rash, myalgias, anemia, leukopenia, serositis, and cerebritis. Eighty-eight percent of these flares had associated worsening of serologic results. Prednisone was discontinued in only one patient at any time during peritoneal dialysis. This experience reveals that patients with lupus continue to show clinical and serologic disease activity and require maintenance prednisone therapy while receiving long-term peritoneal dialysis.
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PMID:Persistence of clinical and serologic activity in patients with systemic lupus erythematosus undergoing peritoneal dialysis. 349 20

A 23-year-old woman with systemic lupus erythematosus had a severe hypersensitivity reaction to the drug ibuprofen. The reaction, documented by rechallenge during hospitalization, was characterized by marked hypotension, fever, rigors, conjunctivitis, meningitis, nausea, arthralgias, and leukopenia. In addition to illustrating the potential diagnostic confusion with infection or lupus flare, this case is of interest in that the reaction developed suddenly despite the patient's uneventful use of the drug for over two years. Collected cases are reviewed from the literature, and the common features are summarized.
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PMID:Severe systemic hypersensitivity reaction to ibuprofen occurring after prolonged therapy. 356 35


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