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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Renal histology is increasingly used as a guide for therapy and prognosis in SLE but data in children are few and/or short-term. We assessed renal histological features in 19 children with SLE to determine whether these features are useful in predicting long-term outcome. Mean age at biopsy was 10 +/- 1.7 years old, male to female ratio was 1:2.8. Fourteen patients (73%) had diffuse proliferative lupus nephritis. Renal histology was evaluated using an activity index (AI) and chronicity index (CI). Clinical assessment of renal function at biopsy and outcome were graded according to urinalysis and serum creatinine. Renal function at biopsy correlated well with AI (p < 0.001) but not CI. At short-term follow-up (30 months), 3 patients had died from sepsis and another 2 reached end-stage renal disease. CI predicted poor clinical outcome, i.e. death or renal failure (p < 0.005) but AI did not. At long-term follow-up (mean 92.1 +/- 26.8 months) only one more patient reached end-stage renal disease. In others renal function assessment showed improvement or were stable. Neither CI nor AI correlated with clinical outcome. We conclude that although AI correlates well with renal function at biopsy and CI with short-term prognosis, neither can predict long-term outcome. Treatment may have altered the natural course of disease in these patients.
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PMID:Renal pathology and long-term outcome in childhood SLE. 980 81

Significant advances have been made in the capabilities to remove white blood cells (WBCs) from blood by both centrifugal and filtration techniques. New techniques have applications for both donor products (and their effects upon the recipients) and for selected disease therapeutics. The immunomodulatory effects of donor WBCs may be therapeutic, e.g., granulocytes harvested by apheresis may be used for the treatment of sepsis, or mononuclear cells collected by apheresis for peripheral blood progenitor (stem) cell transplantation or graft versus leukemia effect. In contrast, WBCs are removed from many transfusable components to decrease the immune effects in recipients. This has been accomplished primarily by the use of leukoreduction filters although newer adaptations of centrifugal equipment allow for the reduction of WBCs to target range of <1 x 10(6) WBCs/product. Therapeutic WBC removal by centrifuge has been used for treatment of the effects due to elevated levels of WBCs or platelets. More specific cellular immunotherapy has included lymphocytapheresis for the treatment of autoimmune diseases such as systemic lupus erythematosis (SLE). Various mononuclear cell fractions collected by apheresis have been used for lymphokine activated killer cells (LAK) and tumor infiltrating lymphocytes (TIL) cell therapy or autologous stem cell transplantation. The development of WBC adsorbent filters for therapeutic use has evolved as nonspecific filter materials have been demonstrated to show selective WBC removal, and filter columns permit therapeutic reductions in WBCs using online filtration therapy. Specific adsorption techniques, e.g., CD-34 selection, are in use in vitro and indicate directions for further developments in cellular immunotherapy.
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PMID:Apheresis techniques and cellular immunomodulation. 1022 38

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

Although the short- and medium-term (5-10 years) outcome of patients with lupus nephritis has been studied extensively, there are very few data on the second and subsequent decades. We studied outcome in 110 local patients investigated at a single centre before 1986, who all had potential follow-up of more than 10 years (actual 2-31 years, median 15.5 years). At last follow-up, 40 patients were dead and 70 alive, nine of whom were on maintenance dialysis or transplanted, actuarial survivals being 84%, 72%, 62%, 61% and 54% at 5, 10, 15, 20 and 25 years for the group as a whole. Survival was better in the cohort 1976-86 (n = 60) than in that from 1963-75 (n = 50) (90, 81 and 76% vs. 78, 56 and 43% at 5, 10 and 15 years, p < 0.001). Sepsis (12) and myocardial infarction (8) were the principal causes of death. Of living patients with renal function, 38% had normal urine and renal function, 11 were off all treatment (19%), 62% had persistent proteinuria and 18% had reduced but generally stable renal function. Renal failure, in those patients who developed it, occurred during the first decade and none of 67 patients actually followed more than 10 years subsequently went into renal failure. Induction treatment was with prednisolone, combined with azathioprine in more severe forms of nephritis, and from the middle 1970s to 1986, 30 with methylprednisolone and in 12 cases plasma exchange. Seventeen other patients were treated using oral cyclophosphamide during the 1960s. No patient received i.v. cyclophosphamide as induction therapy, although nine patients had this form of treatment later, largely because of non-compliance. Serious complications of lupus and/or its treatment occurred in 49%: sepsis in 32, ischaemic heart disease in 20, thrombosis in one and avascular necrosis of bone in eight. In contrast, fracturing osteoporosis occurred in only three, and cataracts requiring surgery and diabetes mellitus in none. The very long-term outlook of lupus nephritis, especially its more severe forms, has improved, but that with current management strategies only a minority of patients are able to stop treatment altogether, and the incidence of serious complications is high.
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PMID:The very long-term prognosis and complications of lupus nephritis and its treatment. 1039 9

A patient with systemic lupus erythematosus (SLE) developed a rectal ulcer and sepsis from colonic bacteria. At that time she had no other clinical manifestations of SLE. Histopathologic examination of the biopsies taken from the ulcer found evidence of vasculitis. Treatment with high-dose systemic steroids healed the ulcer clinically and endoscopically, but symptoms recurred when steroids were tapered. The patient was referred for surgery. This is a rare but dangerous complication of SLE and can be the only clinical manifestation of the disease.
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PMID:Rectal ulcers: a rare gastrointestinal manifestation of systemic lupus erythematosus. 1047 88

Of the 120 systemic lupus erythematosus (SLE) patients treated by the authors, two have developed diffuse alveolar haemorrhage. The authors' objective is to present this rare, but severe manifestation. Patients 1 and 2 were 66- and 22-year old women, respectively. Both had SLE with multi-organ involvements including diffuse proliferative lupus nephritis. Before the diagnosis of the disease, both patients had experienced pneumonitis that resolved on corticosteroid treatment. Soon after the diagnosis, respiratory failure, haemoptoea and acute anaemia developed, accompanied by a rapid deterioration in the general condition. Chest radiographs revealed bilateral, diffuse, alveolar infiltrates. The pulmonary haemorrhage temporarily ceased in response to corticosteroid treatment, but both patients later died in consequence of active SLE and mixed bacterial and fungal sepsis. Post mortem examination demonstrated fibrosing alveolitis and alveolar bleeding in Patient 1, and an immune complex deposition-induced alveolocapillary inflammation with alveolar haemorrhage in Patient 2. Diffuse alveolar haemorrhage is a life-threatening manifestation of SLE. Its onset may be preceded by episodes of pneumonitis resolving on corticosteroid treatment. An active diagnostic workup, intensive observation and aggressive immunosuppressive treatment are the cornerstones of the management. The early detection and the active treatment of secondary infections are obligatory. The authors consider the most difficult challenge to be the optimum coordination of the above treatment modalities.
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PMID:[Diffuse alveolar hemorrhage in systemic lupus erythematosus]. 1069 87

Reactive haemophagocytic syndrome is characterized by activation and uncontrolled non-malignant proliferation of T lymphocytes and macrophages, leading to a cytokine overproduction, which accounts for the main biological signs. Children usually present with an acute febrile illness, fulminant pancytopenia and hepatosplenomegaly, posing a problem of differential diagnosis with severe sepsis. Hemopoietic cells are actively ingested by monocytes/macrophages in various organs, including lymph nodes, bone marrow, liver and spleen. This exarcerbation of the histiocytic system is currently classified among the reactional histiocytoses. It reflects an inappropriate host immune response. Most patients have a known underlying disease (hemopathy, lupus, systemic juvenile arthritis, HIV infection). In the few cases that occur in the apparent absence of any risk factors, investigations should be made to look for predisposing inherited diseases, such as familial lymphohistiocytosis or Purtilo's disease in boys. The treatment rests on immunosuppressive agents, followed by bone marrow transplantation, which can provide a definitive cure in genetic forms.
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PMID:[Reactive hemophagocytic syndrome in children]. 1076 6

A 14-year-old African-American girl was diagnosed with antiphospholipid-positive systemic lupus erythematosus (SLE) in July 1994. The course was complicated by nephrotic syndrome, sepsis, hemolytic anemia, acute renal failure, saphenous vein thrombosis, cutaneous vasculitis, mesenteric vasculitis, appendicitis, hemorrhagic cystitis, and avascular necrosis of the hips. In August 1997, she developed ovarian and fallopian tube complications secondary to SLE. Genitourinary complications of SLE, however, are uncommon, and ovarian vasculitis has not previously been reported as a complication of SLE. This report describes the course of an adolescent patient with SLE and focuses specifically on her genitourinary complications.
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PMID:Genitourinary complications of systemic lupus erythematosus. 1080 72

As a marker of in vivo B-cell activity, urine levels of free light chain (FLC) were measured twice weekly by radioimmunoassay (RIA) and correlated with disease activity over periods of 5-10 months in seven patients with systemic lupus erythematosus (SLE). In addition, RIA-measured urine albumin was used to track glomerular injury, and alpha1-microglobulin (alpha1-M) levels, 28- to 32-kDa protein, provided control measurements on excretion of low-molecular-weight proteins. As controls, urine FLC levels were obtained from healthy normals and in subjects with acute pharyngitis, sickle-cell anemia, and acute sepsis or pneumonia. The control results showed that with acute sepsis/pneumonia had marked increases in urine FLC, while pharyngitis and sickle-cell controls had normal FLC levels. In SLE, active patients receiving intravenous cyclophosphamide and high-dose steroids exhibited highly increased urine FLC that fluctuated widely during therapy and fell to normal range levels with disease remission. During active SLE, urine albumin often was increased, while alpha1-M levels remained in normal range. In contrast to the increased FLC of active disease, inactive patients on low-dose maintenance therapy had predominantly normal FLC levels throughout the collection period. These results support our hypothesis that longitudinal levels of urine FLC can be used to track disease-related B-cell activity in SLE. Furthermore, we suggest that the urine FLC of active SLE would share LC idiotype with the clonal associated in vivo secreted Ig, and thus permit the identification of these antibodies that are targeted to the culprit immunogen(s) responsible for the pathogenesis of SLE.
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PMID:Urine free light chains in SLE: clonal markers of B-cell activity and potential link to in vivo secreted Ig. 1082 64

Chronic inflammatory arthritis in childhood could be due to an obvious cause (e.g. sepsis, rheumatic fever, systemic lupus erythematosus etc.), or it could be idiopathic. After excluding those with obvious cause there still remains a large group of chronic inflammatory arthritis in childhood. This category has been variously called 'juvenile rheumatoid arthritis', 'juvenile arthritis', 'juvenile chronic arthritis', and more recently, 'idiopathic arthritis of childhood', The present article reviews the various classification criteria used for defining this group of disorders with emphasis on the common features as well as the major differences between these criteria. The major classes within this group with their characteristic clinical and laboratory features are also discussed.
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PMID:Juvenile rheumatoid arthritis--clinical manifestations. 1083 2


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