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)

A 17-year old-male presented with a 6-week history of weight loss, lassitude and calf pains. On examination he was very pale. Laboratory tests showed a very high erythrocyte sedimentation rate (155 mm in the first hour), anaemia (haemoglobin 10.1 g/dl), and a raised serum creatinine of 1.54 mg/dl. Microhaematuria (5-10 erythrocytes/microliter) and pronounced pyuria (500 leucocytes/microliter) were present, but the urine was sterile and there was no increase in albumin excretion. The serum IgG was raised to 75.7 g/l, suggesting an autoimmune disorder. Anti-nuclear antibodies (titre 1 : 1920) and anti-double-stranded DNA antibodies (31 U/ml) were present, while the serum complement C4 was decreased to 0.11 g/l. Renal histology showed an interstitial nephritis without glomerular involvement, while the bone marrow showed vasculitis accompanied by a prominent plasma-cell infiltrate. A diagnosis of interstitial nephritis associated with systemic lupus erythematosus was made, with asymptomatic cardiac and hepatic involvement. Renal function recovered rapidly with prednisolone therapy (initial dose 2 mg/kg.d). While glomerulonephritis is the most common lupus-associated renal disorder, isolated interstitial nephritis may occur in some cases, often with an absence of proteinuria.
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PMID:[Interstitial lupus nephritis]. 158 9

A 13-year-old girl presented with abdominal pain, fever, dysuria, incontinence and pyuria and was subsequently diagnosed as having systemic lupus erythematosus (SLE) with extensive gastrointestinal involvement and an associated interstitial cystitis. Despite aggressive therapy with high dose prednisone and cyclophosphamide she developed a small bowel perforation and subsequently died. The combination of bowel symptoms and interstitial cystitis seems unique to the population with SLE, while the separate complication of bowel perforation carries an extremely poor prognosis in this group of patients.
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PMID:Bowel perforation and interstitial cystitis in childhood systemic lupus erythematosus. 186 24

To assess the activity of lupus nephritis, 43 patients with systemic lupus erythematosus (SLE) were studied by gallium imaging. Delayed renal visualization 48 hours after the gallium injection, a positive result, was noted in 25 of 48 scans. Active renal disease was defined by the presence of hematuria, pyuria (10 or more red blood cells or white blood cells per high-power field), proteinuria (1 g or more per 24 hours), a rising serum creatinine level, or a recent biopsy specimen showing proliferative and/or necrotizing lesions involving more than 20 percent of glomeruli. Renal disease was active in 18 instances, inactive in 23, and undetermined in seven (a total of 48 scans). Sixteen of the 18 scans (89 percent) in patients with active renal disease showed positive findings, as compared with only four of 23 scans (17 percent) in patients with inactive renal disease (p less than 0.001). Patients with positive scanning results had a higher rate of hypertension (p = 0.02), nephrotic proteinuria (p = 0.01), and progressive renal failure (p = 0.02). Mild mesangial nephritis (World Health Organization classes I and II) was noted only in the patients with negative scanning results (p = 0.02) who, however, showed a higher incidence of severe extrarenal SLE (p = 0.04). It is concluded that gallium imaging is a useful tool in evaluating the activity of lupus nephritis.
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PMID:Appraisal of lupus nephritis by renal imaging with gallium-67. 387 88

Dermatomyositis is the connective tissue disease with the least renal involvement. Although some renal findings like proteinuria, hematuria, pyuria, progressive renal insufficiency, and glomerular and tubular calcium deposits with arteriolar fibrosis have been described, glomerulonephritides have rarely been associated with dermatomyositis, especially in childhood cases. We describe a 10-year old boy with the clinical picture of dermatomyositis who underwent renal biopsy due to microscopic hematuria demonstrating membranous glomerulonephritis with Clq deposition. Children with "full-house" membranous glomerulonephritis with deposition of Clq and the other immunoglobulins have been reported to present in the future with the clinical findings of systemic lupus erythematosus. However, laboratory evaluation of our patient for systemic lupus erythematosus was negative at the present time. Thus, we think this case should be followed up closely with special attention to the possible clinical and laboratory findings of systemic lupus erythematosus.
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PMID:Dermatomyositis with membranous nephropathy. 1143 94

Hematuria or sterile pyuria as isolated urinary findings present a clinical dilemma for the treating physician. Our objective was to determine whether isolated hematuria and isolated sterile pyuria are associated with active systemic lupus erythematosus (SLE) with respect to renal and non-renal disease activity. This is a descriptive study from a large SLE cohort followed prospectively at the University of Toronto Lupus Clinic. All episodes of isolated hematuria and isolated pyuria between 1970 and 2000 were identified from our database. Isolated hematuria was defined as > 5 red blood cells per high power field; isolated sterile pyuria was defined as > 5 white blood cells per high power field in the absence of urinary infection and other renal manifestations. Non-renal disease activity (defined as nrSLEDAI > 1) was determined at first episode of isolated hematuria and pyuria. Renal disease activity was assessed by scoring renal biopsies within 3 months of detecting isolated hematuria or sterile pyuria. Thirty-four percent (323/946) of our cohort had at least one episode of isolated hematuria. Seventy-seven percent of these patients had concurrent non-renal disease activity. Of the 22 biopsies scored with isolated hematuria, 96% were abnormal (WHO > class I), including 52% with active nephritis. Twenty-three percent (215/946) had at least one episode of isolated sterile pyuria. Seventy-eight percent of these patients had concurrent non-renal disease activity. All 12 biopsies scored with isolated pyuria were abnormal (WHO Class > 1), including 75% with active nephritis. The appearance of isolated hematuria and isolated pyuria is associated with active renal and non-renal disease activity. An ongoing debate has emerged regarding the significance of isolated hematuria and isolated pyuria with respect to SLE disease activity. The results of this study suggest that isolated hematuria and isolated pyuria is associated with active renal and non-renal disease activity. Thus isolated hematuria and isolated sterile pyuria should be considered manifestations of active SLE.
Lupus 2001
PMID:Significance of isolated hematuria and isolated pyuria in systemic lupus erythematosus. 1143 77

Seventy female and three male Omani systemic lupus erythematosus (SLE) patients are described. At disease onset, 45 (62%) were under 20 years of age, and the remainder were between 20 and 44. Of all cases, 48% were familial. Over 5 years, the cumulative frequencies of autoantibodies was: antinuclear antibodies (ANA) 97%, anti-double-stranded DNA (anti-dsDNA) antibodies 92%, extractable nuclear antigen (ENA) antibodies 64%, antineutrophil cytoplasmic antibodies (ANCA) 58%, antiphospholipid (APL) antibodies 80%, and rheumatoid factor (Rf) 22%. Ribonucleoprotein (RNP) antibodies were found in 15/45 younger-onset and 2/28 older-onset patients (chi(2)=6.63, P<0.02). The mean SLE disease activity score (SLEDAI) was 13.5+11.4, and the cumulative frequencies of systemic involvement were: neurological 33.8%, vascular 10.4%, musculoskeletal 53.9%, renal 50.7%, dermal 80.5%, serosal 23.9%, immunological 95%, constitutional 31.3%, and haematological 26.0%. Linear regression analysis showed that high-titre ANA were predictors for pyuria (odds ratio [OR] 9.06, P=0.01). Antiextractable nuclear antigen antibodies were predictors for disease of the neurological (OR 26.3, P=0.008) and serosal (OR 27.7, P=0.005) systems, and anti-Sm antibodies for alopecia (OR 5.93, P=0.088) and hypocomplementaemia (OR 14.6, P= 0.016). Antibodies of known diagnostic utility may also give insights into the pathogenesis of SLE.
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PMID:Demographic, autoimmune, and clinical profiles of patients with systemic lupus erythematosus in Oman. 1267 78

Fungal infection of the genitourinary system is a relatively uncommon presentation. Cryptococcuria has rarely been recognized in clinical practice. Patients with positive urine culture for Cryptococcus neoformans from 1992 to 2003 were retrospectively reviewed. Sixteen patients were identified. Nine (56%) patients were male, with a mean age of 44 +/- 21 (range, 16-88) years. Fifteen (94%) patients had underlying conditions such as HIV infection, diabetes mellitus, hypertension, and/or systemic lupus erythematosus. Thirteen (81%) patients had cryptococcuria as a manifestation of disseminated cryptococcosis, and the rest had only isolated cryptococcuria. Urinary analysis revealed proteinuria (75%), pyuria (31%), and budding yeast (13%). Nine (56%) patients received antifungal therapy. Other patients were misdiagnosed or died before treatment. The mortality rate was 64%. In conclusion, cryptococcuria is not extremely rare and can present as a manifestation of disseminated cryptococcosis or isolated urinary tract infection.
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PMID:Cryptococcuria as a manifestation of disseminated cryptococcosis and isolated urinary tract infection. 1550 76

To evaluate the correlation between measurements of antinuclear antibodies serum levels by enzyme immunoassay (ANA-EIA), and the degree of systemic lupus erythematosus disease activity. To retest the performance of the test compared to measurement of antinuclear antibodies by immunofluorescence (ANA-IIF). Eighty-five sera from 71 patients with SLE were tested. Demographic, clinical, laboratory, and SLEDAI status were collected. The sera were tested for ANA-EIA and by ANA-IIF at 1:40 and 1:160 dilutions. Serum levels of ANA-EIA were compared to the overall SLEDAI score and to each of its components. A SLEDAI score of > or =6 was considered clinically significant. The sera of fifty-one healthy volunteers served as controls. Serum levels of ANA-EIA were significantly higher in patients with a SLEDAI score of > or =6 compared to the group of patients with a SLEDAI score of <6 (P = 0.004). High serum levels of ANA-EIA correlated significantly with elevated anti DS-DNA antibodies (P < 0.001), low C(3) or C(4) levels (P < 0.001), pyuria (P < 0.011), arthritis (P = 0.019), and new rash (P = 0.019). Levels of ANA-EIA were significantly higher in patients tested positive by IIF compared to those who tested negative. Higher serum levels of ANA-EIA correlated with clinically significant disease activity in patients with SLE. Higher serum levels of ANA-EIA also correlated with some single items of the SLEDAI. The results also reiterated the validity of ANA-EIA testing in patients with SLE. Further longitudinal studies are needed in order to test the hypothesis that serum ANA-EIA levels might reflect fluctuations in disease activity.
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PMID:Antinuclear antibodies measured by enzyme immunoassay in patients with systemic lupus erythematosus: relation to disease activity. 1763

The objective of this study was to assess isolated pyuria in an unselected systemic lupus erythematosus sample, and to determine factors potentially associated with this manifestation. We studied patients followed in our lupus clinic, defining isolated pyuria as more than 10 white blood cells per high power field in the absence of hematuria, proteinuria, casts, or bacteriuria. We assessed the effects of various demographic and clinical factors on the occurrence of isolated pyuria, using univariate logistic regression analyses. We also performed multivariate models which included sex, age, race/ethnicity, systemic lupus erythematosus duration, non-renal systemic lupus erythematosus disease activity, systemic lupus erythematosus damage, number of non-renal and renal American College of Rheumatology criteria ever present, pre-existing hypertension, and current drug exposures. Of 264 subjects, 66 were excluded (43 had bacteriuria or a contaminated urine culture and 23 had no concomitant urine culture); 27 of the remaining 198 (13.6%) had isolated pyuria. Sixteen of 27 patients with sterile pyuria had previous American College of Rheumatology criteria for renal involvement (hematuria, casts, and/or proteinuria) compared to 62/171 patients without sterile pyuria (unadjusted odds ratio = 2.55; 95% confidence interval = 1.11-5.85). Our univariate analyses also suggested a trend towards higher non-renal disease activity in patients with isolated pyuria. Independent associations were not evident in adjusted analyses. Isolated pyuria was observed in a significant number of our systemic lupus erythematosus sample. Although the differential diagnosis for isolated pyuria is broad, this manifestation may be correlated with lupus activity even in the absence of hematuria or proteinuria. Lupus (2010) 19, 793-796.
Lupus 2010 Jun
PMID:Isolated pyuria in systemic lupus erythematosus. 2030 45

Bullous systemic lupus erythematosus is a rare subset of systemic lupus erythematosus that is even rarer in pediatric patients. We report a case of a 12-year-old girl who presented with a vesiculobullous eruption on her face, neck, trunk and genital and oral mucosa, as well as anemia, sterile pyuria, ANA (1:1280, speckled pattern) and positive anti-Sm and anti-RNP. Pathological examination suggested dermatitis herpetiformis, and direct immunofluorescence revealed IgG, IgA and fibrin in the epithelial basement membrane zone. We present a typical case of bullous systemic lupus erythematosus and emphasize the importance of clinical and histopathological differential diagnosis with dermatitis herpetiformis.
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PMID:Bullous systemic lupus erythematosus: differential diagnosis with dermatitis herpetiformis. 2206 82


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