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 retrospectively evaluated the clinico-laboratory features of 11 children and adolescents with Systemic Lupus Erythematosus between the period of 2001 and 2006. All of them (100.0%) had renal involvement at the first visit in the hospital. Female to male ratio was 10:1. Skin and or mucosal involvement (90.9%), periorbital puffiness and or pedal edema (81.8%), fever (72.7%), hypertension (72.7%), and reticuloendothelial involvement (72.7%), were the commonest presentations. All patients had anemia (8.6 +/- 1.5 gm/dl), raised ESR (46.7 +/- 9.4 mm in first hour), proteinuria, and in disease activity as evident by raised ESR and positive anti-dsDNA antibody at the first visit. The mean duration of disease was 7.6 months and the average duration of disease activity was 63.18 days. Renal biopsy was performed in 8 patients: class IV lupus nephritis in 4 patients (50.0%), class II in 2 patients (25.0%), class III and V in patient (12.5%) each. Nephrotic range proteinuria and hypertension was observed in all patients of class IV and V of lupus nephritis. Class II and III lupus nephritis patients' were normotensive and had non-nephrotic range proteinuria. Three out of 11 patients (27.2%) expired. The commonest primary determinant of mortality was uncontrolled disease activity in 2 patients (66.6%). The third one had infection and developed disseminated intravascular coagulation. The mean duration of disease activity in patients who died (mean 30 days) was statistically lower than the survival group (75.6 days) (p < 0.01). Renal involvement during first visit and mortality could be attributed by late referrals and diagnosis at hospital.
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PMID:Systemic lupus erythematosus in childhood--a review of 11 patients at a single center in eastern Nepal. 1809 38

Patients with systemic lupus erythematosus (SLE) have accelerated atherosclerosis, but the underlying mechanisms are unclear. The size and number of lipoprotein particles may be better predictors of atherosclerosis than conventional cholesterol measurements. We measured lipoprotein subclasses by nuclear magnetic resonance spectroscopy (NMR), coronary artery calcification by electron beam computed tomography, and insulin resistance by homeostasis model assessment in 105 patients with SLE and 77 control subjects. VLDL particles were larger (50.0+/-8.5 versus 47.7+/-8.5 nm, P=0.01) and concentrations of large high-density lipoprotein (HDL) particles lower (10.1+/-5.3 versus 11.3+/-5.1 nmol/L, P=0.03) in patients with SLE than controls. In patients with SLE, small LDL concentration was associated with body mass index (rho=0.27), insulin resistance (rho=0.34), C-reactive protein (CRP; rho=0.30), and erythrocyte sedimentation rate (ESR; rho=0.20); all P<0.05. Large HDL concentration was inversely associated with insulin resistance (rho=-0.29), disease activity (rho=-0.23), and ESR (rho=-0.39); all P<0.05. VLDL concentrations correlated with CRP (rho=0.22), ESR (rho=0.24), disease damage (rho=0.20), and corticosteroid exposure (rho=0.29); all P<0.05. Neither the concentration of lipoprotein subclasses nor particle size was associated with coronary artery atherosclerosis. There were only minor differences in the NMR lipid profiles of patients with SLE and controls. Lipoprotein subclasses were associated with metabolic variables, inflammatory markers, and corticosteroid use but not with coronary artery atherosclerosis in SLE.
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PMID:Lipoprotein subclasses and particle size determined by nuclear magnetic resonance spectroscopy in systemic lupus erythematosus. 1842 45

This study aims to investigate the role of Antineutrophil cytoplasmic antibodies (ANCA) in patients with new-onset systemic lupus erythematosus (SLE). Sixty SLE patients, 28 of whom had lupus nephritis (LN), and 60 normal controls were enrolled; Serum ANCA was measured by enzyme linked immunosorbent assay (ELISA). The clinical and laboratory parameters of the patients were also recorded. Results show that twenty SLE patients were seropositive for ANCA, which was significantly higher than in normal controls. LN patients had significantly higher positive rate of ANCA than patients without nephritis. Compared with ANCA-negative patients, the ANCA-positive patients had significantly higher incidence of nerves system disorder, myocarditis, renal involvement and serositis. The positive rate of gamma-globulin, anti-dsDNA and anti-Sm antibodies were significantly higher in ANCA-positive patients. Elevated IgG and ESR, decreased serum C3/C4 appeared more often in ANCA-positive patients. In addition, serum ANCA level correlated positively with disease activity. Taken together, ANCA might be used as a potential complementary parameter to differentiate LN from SLE without nephritis. In addition, ANCA may serve as a useful marker of the disease activity of SLE.
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PMID:Anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus and lupus nephritis. 1852 49

The aim of this study is to characterize the clinical manifestations of postmenopausal systemic lupus erythematosus (SLE) patients. Of the 699 SLE inpatients, 20 postmenopausal and 70 menstruous SLE patients were evaluated and compared for the clinical manifestations. The mean age of onset was 55.05 years (range from 42 to 66) with a peak of 50-60 in postmenopausal lupus patients. The average time from SLE onset to diagnosis was 2.18 years. Arthritis was the most frequent initial manifestation in the postmenopausal group. Other common clinical manifestations and laboratory abnormalities include lassitude, fever, alopecia, malar rash, cardiac impairment and weight loss, and elevated ESR, decreased C3, ANA >or= 1:80, hypergammaglobulinemia and anti-RNP antibody positive. Compared with menstruous lupus patients, postmenopausal patients were more likely to have weight loss (P < 0.01), myalgia and myasthenia (P < 0.01), and less likely to have malar rash (P < 0.05), renal involvement (P < 0.01), leukocytopenia (P < 0.05) and positive ANA (P < 0.01). Thus, less disease severity and favorable prognosis were associated with postmenopausal SLE patients. Misdiagnosis and missed diagnosis were easy to make with their non-specific symptoms with fewer features suggestive of diagnosis.
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PMID:Less disease severity and favorable prognosis are associated with postmenopausal systemic lupus erythematosus patients. 1881 29

Systemic Lupus Erythematosus (SLE) is a disease of many faces and great mimicry. Women of child bearing ages are the potential candidates for this disease though it may occur in any age and sex group and the clinical manifestations do not vary with sex differentiation. Etiology of SLE is still to be defined and it has a prevalence of 27.7/100,000 population with higher rate of incidence in Asian and African women. This is a prospective study done from January, 2002 to December, 2006. The objective of the study was to observe the clinical profile and outcome of patients suffering from SLE. 33 patients were diagnosed following the ARA criteria. All the patients were investigated with ANA, anti ds-DNA antibody, routine blood and urine examination and follow up was done following SLE Disease Activity Index (SLEDAI) scoring system along with routine blood and urine examination. Mean age at presentation was 27.3 years with a standard deviation of 12.5. Among them, constitutional symptoms were present in 91% of the patients, hematological features were also present in 91% of patients where anemia was almost universal, one patient was suffering from autoimmune hemolytic anemia and 4 patients had thrombocytopenia. Mild leucopenia and lymphopenia was also observed in 33% patients. 60% patients show an ESR higher than 100 mm in 1st hour. Musculoskeletal and cutenous features were present in 81% and 75% cases respectively. 36% patients had some degree of renal involvement at the time of diagnosis. 6% patients had severe neuropsychiatric features. After appropriate management 52% patients leading a normal life with medication, 30% patients had a fluctuating course, 9% patients died and 9% lost follow up. Outcome was better in the patients who were diagnosed and treated early.
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PMID:Clinical profile, management and outcome of lupus. 1894 54

The objectives were to determine the prevalence and clinical significance of anti-cyclic citrullinated peptide (anti-CCP) antibodies in patients with juvenile idiopathic arthritis (JIA). Anti-CCP antibodies were checked by ELISA in 68 children with JIA, 38 males and 30 females with mean age of 10.6 (+/-4.02) years and disease duration of 3.7 (+/-1.8) years. Thirty-eight (56%) patients had polyarticular disease, 20 (29%) patients had oligoarticular disease and 10 (15%) patients had systemic onset disease. All patients had their antinuclear antibodies (ANA), rheumatoid factor (RF) and ESR checked and x-rays performed to look for erosions. Results were compared to those of 20 healthy children, 14 children with juvenile systemic lupus erythematosus (JSLE) and 30 adults with rheumatoid arthritis (RA). Anti-CCP antibodies were positive in 14/68 (20.6%) patients with JIA, all had polyarticular-onset disease. All patients with positive anti-CCP antibodies had RF-positive polyarthritis. Anti-CCP antibodies were negative in all patients with oligoarticular-onset and systemic-onset disease including 2 patients with extended oligoarthritis. Anti-CCP antibodies were negative in healthy and JSLE controls but were positive in 20/30 (66.5%) adults with RA. Anti-CCP antibodies correlated significantly with joint erosions in patients with JIA (p = 0.004) but no significant relation was found between anti-CCP antibodies and ANA positivity or raised ESR. These data confirm that anti-CCP antibodies are less prevalent in JIA than adult RA but are detectable in a significant proportion of RF-positive patients with polyarticular-onset JIA. The current study showed significant relation between anti-CCP positivity and erosive joint disease in JIA.
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PMID:Anti-cyclic citrullinated peptide antibodies in patients with juvenile idiopathic arthritis. 1899 Nov

Fever of unknown origin (FUO) characterizes febrile disorders that are accompanied by prolonged fevers of 101 degrees F or greater for 3 weeks or more that remain undiagnosed after comprehensive inpatient and outpatient diagnostic testing. At the present time, malignancies are the most common cause of FUOs. Among malignant FUOs, lymphomas are the most common. We present the case of a non-Asian young adult man who presented with FUO. He had no peripheral adenopathy or splenomegaly but was found to have anterior/superior mediastinal adenopathy and right paratracheal adenopathy. His diagnostic workup was negative for rheumatic/inflammatory and infectious diseases. Laboratory test results were unremarkable except for a highly elevated erythrocyte sedimentation rate and highly elevated serum ferritin level. Otherwise unexplained highly elevated serum ferritin levels in patients with FUOs suggest rheumatic and inflammatory disorders, for example, systemic lupus erythematosus flare or malignancy. The findings of mediastinal adenopathy combined with a highly elevated ESR and highly elevated serum ferritin levels indicate lymphoma as the most likely diagnosis. He also had polyclonal gammopathy on serum protein electrophoresis (SPEP). In a patient with FUO, negative blood cultures, and a heart murmur, polyclonal gammopathy on SPEP suggests atrial myxoma. Lymphomas are often associated with elevated alpha(1)/alpha(2) globulins on SPEP. Lymph node biopsy of the mediastinal nodes was negative for lymphoma but did not show characteristic emperiopolesis, pathognomonic of Rosai-Dorfman disease, a benign lymphoproliferative disorder. Rosai-Dorfman disease usually presents with massive bilateral cervical adenopathy but may present with lymph node involvement in other sites, as in this case. In patients with lymphadenopathy and a negative FUO workup, clinicians should consider the possibility of Rosai-Dorfman disease, particularly if accompanied by an otherwise unexplained highly elevated serum ferritin levels and polyclonal gammopathy on SPEP.
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PMID:Fever of unknown origin (FUO) due to Rosai-Dorfman disease with mediastinal adenopathy mimicking lymphoma: diagnostic importance of elevated serum ferritin levels and polyclonal gammopathy. 1915 May 34

We report the clinical profile, treatment and outcome of systemic lupus erythematosus in 70 patients between the age of 4-15 years. Fever (94.2%), arthritis (65.7%) and malar rash (57.1%) were the chief extra-renal manifestations. The ESR was raised in 98.5% patients, anemia was seen in 60% and direct Coombs test was positive in 58.3%. Antinuclear antibody was positive in all; anti-double stranded DNA antibody and low C3 levels were seen in 77.1% and 80%, respectively. Renal involvement was noted in 77.1% and included proteinuria (53%), hematuria (42.8%), hypertension (18.5% and elevated serum creatinine (8.6%). Renal histology showed class I nephritis in 3.7%, class II in 44.4%, class III in 4.3%, class IV in 44.4% and class V in 1.8%. On follow up 18.8 months later, 70% patients were in remission, 7.5% had active disease and 7.5% died. The characteristics of childhood lupus erythematosus were similar to those previously reported. The outcome was favorable in most cases.
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PMID:Clinical features and outcome of systemic lupus erythematosus. 1921 81

Efficiency and tolerability of rituximab therapy were assessed in 13 patients with Sjogren's syndrome and disease (10) (SLE-1, RA-2). Nine patients (SD-8, PA-1) presented with lymphomas and 4 with systemic manifestations of the disease. Complete and partial remission of lymphoma was achieved in 7 (78%) and 2 (22%) patients respectively. Beneficial effect of therapy on systemic manifestations of the disease was recorded in 3 (75%) of the 4 cases and only 1 patient had cryoglubulinemic glomerulonephritis resistant to rutiximab. Subjective improvement of glandular manifestations was reported by 12 (92%) patients. Objective improvement of salivation and lacrimation was documented in 7 (54%) and 6 (48%) patients who had residual secretion before therapy. Positive outcome of therapy in 12 patients was associated with complete depletion of CD20+ lymphocytes in peripheral blood. These cells were found in a patient who died despite the treatment. Rutiximab significantly decreased medians of ESR, IgG, IgA, IgM, gamma-globulin, and RF levels (p = 0.05-0.002). In 8 patients, therapy resulted in the disappearance of blood cryoglobulins. Intravenous premedication with 500 mg methylprednisolone prevented side effects of rutiximab. The study showed high efficiency and good tolerability of rituximab in combination with pulsed therapy with alkylating cytostatics and courses of polychemotherapy for the treatment of lymphoma and cryoglobuliemic vasculitis in patients with Sjorgen's syndrome and disease. Combination of rutiximab and pulsed therapy was more efficient than rutiximab monotherpy in patients with grade I-IIE MALT-type lymphomas. Rutiximab decreased systemic and glandular manifestations of Sjogren's disease and syndrome in 75 and 48-54% of the patients respectively.
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PMID:[First experience with the application of rituximab for the treatment of patients with Sjogren's syndrome and disease]. 1928 3

The aim of this paper is retrospective analysis of data from patients in whom the indication for cyclophosphamide (CF) pulse therapy was established in our department. Indications for CF pulse treatment were lupus nephritis (LN) alone or associated with central nervous system lupus. CF was administred in the dose of 500-1000 mg/m2 intravenously once monthly for the 6 months and once every 3 months thereafter. Patients were treated with adequate dose of glucocorticoids and other symptomatic therapy. The effect of applied therapy has been analysed by monitoring proteinuria, serum creatinine concentration, creatinine clearance, ESR, ANF titer and total complement hemolytic activity. Initial therapeutic procedure has been completed in 25/30 patients. The reasons for discontinuation in 5/30 patients were as follows: end-stage renal failure in spite of therapy (1), psychosis and lost of compliance (1), recurrent pancytopenia and subsequent sepsis (1), death non related to SLE (1) and failure to show at follow-up (1). Significant improvement of all control parameters was observed in the majority of patients in whom the therapy was completely conducted. 16/25 patients continued therapy for the next 18 months and in only 1/16 patients therapy was discontinued because of end-stage renal failure. In other 15/16 patients further improvement of control parameters was observed, although not so expressed as in the first 6 months. The most frequent complications were infections (16 infections were microbiologically proved and there were probably more infections). Alopecia (2), haematuria (1) and amenorrhoea (1) were also observed. Relatively low incidence of complications may be explained by careful patient monitoring. Considering that therapeutic success is defined not only by the improvement of renal function, but by stopping of further progression of renal failure, it can be concluded that intermittent CF pulse therapy showed good effect on LN in patients with clear indication.
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PMID:[Intermittent intravenous cyclophosphamide application in patients with systemic lupus erythematosus]. 1965 69


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