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)

Clearly, many of the connective tissue disorders are intimately associated with either focal tissue evidence of immune complex deposition or markedly elevated levels of complexes during acute disease activity. In many diseases, such as systemic lupus erythematosus or rheumatoid arthritis, the immune complexes have been firmly established as basic elements in pathogenesis. In other disorders, however, the presence or measurable elevation of circulating immune complex materials may represent an epiphenomenon that reflects tissue injury secondary to the underlying inflammatory process. Much more precise definition of these problems awaits further longitudinal study.
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PMID:Immune complex-mediated rheumatic diseases: the evidence and the enigmas. 644 96

Several points can be made from analysis of the published cases of cutaneous mycobacteriosis and those in our series: 1) mycobacterial cutaneous infections are probably more common than is reported-we collected 34 cases over a 10-year period; 2) most patients with cutaneous infections caused by nontuberculous mycobacteria have significant underlying disease; 3) there is a relative lack of classic histologic features in patients with cutaneous mycobacteriosis, and there appear to be diverse forms of clinical presentation; 4) a high index of suspicion is needed in evaluating patients with possible cutaneous mycobacteriosis, and appropriate cultures must be done to establish the diagnosis. In attempting to provide a practical classification of cutaneous mycobacteriosis which includes infection by nontuberculous mycobacteria, we propose the following grouping, which uses simple terms, avoids confusing nomenclature, and incorporates pathophysiologic descriptions and prognostic information: 1) Mycobacteriosis caused by inoculation from an exogenous source. 2) Cutaneous mycobacteriosis caused by spread from an endogenous source. Contiguous spread originates most often with osteomyelitis, but also occurs through autoinoculation of the perirectal, oral, or vaginal skin as organisms are passed or expectorated from pulmonary or genitourinary tuberculosis. 3) Cutaneous mycobacteriosis caused by hematogenous spread. This group includes lupus vulgaris, nodules and abscesses, and acute disease with hemorrhagic pustules. Some mycobacterioses will be difficult to classify when inoculation or hematogenous spread cannot be ruled out. However, the system of classification we have proposed should help clinicians understand and diagnose the diverse forms of cutaneous mycobacterial infections.
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PMID:Cutaneous mycobacteriosis: analysis of 34 cases with a new classification of the disease. 678 10

Forty-one patients with systemic lupus erythematosus and glomerulonephritis were studied in a randomized drug trial. Thirteen patients received prednisone only (Group 1), 16 received oral cyclophosphamide and oral azathioprine (1 mg/kg body weight . d of each initially) (Group 2), and 12 were given boluses of intravenous cyclophosphamide (0.5 to 1.0 g/m2 body surface area every 3 months) (Group 3). The mean observation period was 42 months (range 1 to 6.5 years). Renal function deteriorated in four of 12 patients in Group 1 and three of 27 patients in Groups 2 and 3 (p = 0.114). By life-table analysis, 86% of the entire group survived 5 years after entry to the study. Marked hypertension, fluctuating changes in serum creatinine, erratic changes in levels of antibody to DNA, reduced C3 levels, increasing proteinuria or sustained hematuria, and flares of extrarenal disease activity occurred more commonly in Group 1. Infectious complications were not commoner in Groups 2 and 3. We conclude that any marginal benefits produced by the programs tested cannot be shown in this class of patients without markedly increasing the sample size. Our current studies involve more vigorous treatment of patients with more acute disease and less treatment during prolonged periods of relatively good health.
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PMID:Alternative modes of cyclophosphamide and azathioprine therapy in lupus nephritis. 704 43

Human herpesvirus-6 is a lymphotropic virus which infects susceptible individuals during the first year of life and usually causes life-long latency. In a variable percentage primary infections are followed by a short acute disease, exanthema subitum. Older individuals may suffer from infectious mononucleosis-like illnesses or from Kikuchi-Fujimoto's disease. In addition, there is a fairly wide spectrum of lymphoid and hematopoietic diseases or autoimmune disorders, which are associated with elevated titers of HHV-6 antibody, and from which replicating virus may be isolated. Such diseases include atypical polyclonal lymphoproliferation, Hodgkin's disease, chronic fatigue syndrome and systemic lupus erythematosus. The present article reviews the current knowledge of such associations.
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PMID:Clinical correlates of infection with human herpesvirus-6. 789 74

Systemic lupus erythematosus is characterized by the production of a broad spectrum of autoantibodies. Autoantibodies directed against endothelial cells (AECA) have been particularly well documented. We investigated associations between such antibodies, double-stranded DNA (dsDNAb), phospholipid (cardiolipin, ACA), and indices of activity and chronicity scored on renal biopsy specimens from 22 patients with acute lupus. AECA were present in 73% of these patients, and both the percentage of patients with AECA and the mean antibody titre fell significantly as patients entered remission. When patients already on immunosuppressive therapy were excluded from analysis (n = 7), only levels of AECA and DNAb (p = 0.02) correlated with histological evidence of active lesions and the presence of glomerular epithelial cell crescents; no correlation was found with chronic changes in the renal biopsies. Serum von Willebrand factor (vWf) and serum total protein S levels, two parameters reflecting endothelial cell function, were also measured during acute disease and remission. vWf concentrations were elevated during acute disease (m = 1.9 IU/ml, p = 0.02), but the values did not correlate with AECA titres. In contrast, total protein S levels were reduced (0.81 IU/ml vs. 0.97 IU/ml, p = 0.01) during active disease, but remained within the normal range (0.56-1.16 IU/ml). Furthermore, protein S levels were inversely related to levels of AECA (r = -0.4, p = 0.01). AECA were therefore present in most patients with acute lupus nephritis and were associated with histological evidence of active renal injury and serological evidence of endothelial cell dysfunction. These data provide indirect support for a pathogenic role for AECA in lupus nephritis.
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PMID:Antiendothelial cell antibodies in lupus: correlations with renal injury and circulating markers of endothelial damage. 826 73

Interstitial lung disease is a significant prognostic factor in connective tissue diseases. To further clarify clinical and immunological features of interstitial lung disease, we studied 894 consecutive patients with connective tissue diseases first evaluated between 1970 and 1993. Interstitial lung disease was found in 101 of 181 (56%) patients with systemic sclerosis, 92 of 183 (50%) with overlap syndrome, 35 of 76 (46%) with polymyositis/dermatomyositis, and 13 of 444 (3%) with systemic lupus erythematosus (p < 0.05, systemic lupus erythematosus vs. other connective tissue diseases). The presence of interstitial lung disease correlated with decreased survival in systemic sclerosis and in polymyositis/dermatomyositis, but not in overlap syndrome or in systemic lupus erythematosus. Interstitial lung disease in patients with connective tissue diseases was classified into two types: acute (n = 8) and chronic (n = 233). Among the 8 patients with acute disease, 4 (all with dermatomyositis) died of respiratory failure and 3 (all with systemic lupus erythematosus) responded to corticosteroids. Among the 233 patients with chronic interstitial lung disease, 20 had polymyositis/dermatomyositis with anti-aminoacyl tRNA synthetase antibodies and 40 had overlap syndrome with anti-U1 RNP antibodies. Respiratory failure in these patients was not frequent and occurred late in the course of the disease. As a cause of death, respiratory failure was associated with autoantibodies to topoisomerase I and aminoacyl tRNA synthetase but not anti-U1 RNP. Autoantibodies to aminoacyl tRNA synthetases were detected before the development of interstitial lung disease in 9 patients with polymyositis/dermatomyositis. We conclude that the clinical features of interstitial lung disease associated with connective tissue diseases vary with the type of connective tissue disease, and that analysis of autoantibodies can be useful in establishing a diagnosis and in forecasting the course and outcome.
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PMID:[Interstitial lung disease in patients with connective tissue diseases]. 875 18

The parent-into-immunocompetent-F(1) model of graft-vs-host disease (GVHD) induces immune dysregulation, resulting in acute or chronic GVHD. The disease outcome is thought to be determined by the number of parental anti-F(1) CTL precursor cells present in the inoculum. Injection of C57BL/6 (B6) splenocytes into (B6 x DBA/2)F(1) (B6D2F(1)) mice (acute model) leads to extensive parental cell engraftment and early death, whereas injection of DBA/2 cells (chronic model) results in little parental cell engraftment and a lupus-like disease. This study demonstrated that injection of BALB/c splenocytes into (BALB/c x B6)F(1) (CB6F(1)) mice resulted in little engraftment of parental lymphocytes and the development of lupus as expected. Injection of B6 splenocytes into CB6F(1) initiated an initial burst of parental cell engraftment similar to that of B6 into B6D2F(1). However, the acute disease resolved, and the CB6F(1) mice went on to develop chronic GVHD with detectable Abs to ssDNA, dsDNA, and extractable nuclear Ags. Limiting dilution CTL assays determined that B6 splenocytes have CTL precursor frequencies of 1/1000 against both CB6F(1) and B6D2F(1), whereas DBA/2 and BALB/c splenocytes have a CTL precursor frequency of 1/20,000 for their respective F(1)s. The Th cell precursor frequency for B6 anti-DBA/2 was 3-fold higher than that for B6 anti-BALB/c determined by limiting dilution proliferation assays. These results indicate the importance of adequate allospecific helper as well as effector T cells for the induction and maintenance of acute GVHD in this model, and presents an unexpected model in which initial acute GVHD is replaced by the chronic form of disease.
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PMID:Progression from acute to chronic disease in a murine parent-into-F1 model of graft-versus-host disease. 1106 62

Venous thromboembolism (VTE) is a chronic rather than acute disease. After withdrawal of secondary thromboprophylaxis, many patients will experience a subsequent episode of thrombosis. Of these patients, approximately 5% will die from pulmonary embolism. The risk of recurrent VTE depends on the number of risk factors and their severity. High-risk patients, i.e. those with a natural coagulation inhibitor deficiency, recurrent thrombosis, active cancer, the lupus anticoagulant or compound clotting defects most probably benefit from indefinite oral anticoagulation. In these patients the risk of bleeding due to anticoagulant treatment seems to be outweighed by the risk of VTE. Patients with hyperhomocysteinemia or high factor (F) VIII plasma levels are also at an increased risk of recurrence. The optimal duration of secondary thromboprophylaxis in these patients is currently under investigation. Patients with the heterozygous F V Leiden mutation or the G20210A mutation in the F II gene do not require extended anticoagulation since their risk of recurrence is similar as in patients without the aforementioned mutations. Patients with VTE secondary to surgery or trauma have a relatively low risk of recurrence. In these patients short-term secondary thromboprophylaxis (6 to 12 weeks) is justified whereas patients with a first episode of spontaneous VTE should be treated with oral anticoagulants for a longer period of time (3 to 6 months).
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PMID:The risk of recurrent venous thromboembolism. 1223 19

Patients with systemic rheumatic diseases may be admitted to the ICU because of worsening of or development of a new manifestation of the rheumatic disease, infections caused by immunosuppression, or adverse effects of drugs used to treat rheumatic diseases. Sometimes an unrelated, acute disorder may become life threatening because of the underlying rheumatic disorder. Rheumatoid arthritis is the most common rheumatic disease seen in ICU patients, followed by systemic lupus erythematosus and scleroderma. These three conditions together account for up to 75% of rheumatic cases admitted to the ICU. The respiratory system is the organ system most commonly affected in the acute process, followed by the renal, gastrointestinal, and nervous systems. More than 50% of admissions result from infections, and 25% to 35% result from exacerbation of the underlying rheumatic condition. In about 20% of patients, the rheumatic disorder may be diagnosed for the first time in the ICU. An aggressive approach should be pursued to establish the diagnosis of either disease exacerbation or infection. Delay in instituting appropriate immunosuppressive or antimicrobial therapy may result in multiple organ system failure and a poor outcome. The mortality rate in patients with rheumatic disease exceeds that predicted by the APACHE II or SAPS II scores and is higher than that in nonrheumatologic ICU admissions. The mortality may exceed 50% in patients admitted for infection; the prognosis is comparatively better for patients with exacerbations of disease activity. Renal failure, coma, and acute abdomen are predictors of poor outcome. Early recognition of abdominal complications requiring surgical intervention may help reduce mortality.
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PMID:Rheumatologic diseases in the intensive care unit: epidemiology, clinical approach, management, and outcome. 1241 38

Systemic lupus erythematosus (SLE) is a CD4(+) T cell-dependent, immune complex-mediated, autoimmune disease that primarily affects women of childbearing age. Generation of high-titer affinity-matured IgG autoantibodies, specific for double-stranded DNA and other nuclear antigens, coincides with disease progression. Current forms of treatment of SLE including glucocorticosteroids are often inadequate and induce severe side effects. Immunological approaches for treating SLE in mice using anti-CD4 mAb's or CTLA4-Ig and anti-CD154 mAb's have proven to be effective. However, like steroid treatment, these regimens induce global immunosuppression, and their withdrawal allows for disease progression. In this report we show that lupus-prone NZB x NZW F(1) mice given three injections of anti-CD137 (4-1BB) mAb's between 26 and 35 weeks of age reversed acute disease, blocked chronic disease, and extended the mice's lifespan from 10 months to more than 2 years. Autoantibody production in recipients was rapidly suppressed without inducing immunosuppression. Successful treatment could be traced to the fact that NZB x NZW F(1) mice, regardless of their age or disease status, could not maintain pathogenic IgG autoantibody production in the absence of continuous CD4(+) T cell help. Our data support the hypothesis that CD137-mediated signaling anergized CD4(+) T cells during priming at the DC interface.
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PMID:CD137 costimulatory T cell receptor engagement reverses acute disease in lupus-prone NZB x NZW F1 mice. 1275 Apr


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