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)

Hematologic alterations are frequent in SLE. The hematologic changes of 111 patients diagnosed as suffering from SLE are described, associating them to 89 clinical, biological and hystological variables. The most frequent alteration was normocytic and normochromic anemia. Coombs positive hemolytic anemia appeared in 10% of the cases. Approximately 20% of patients had leukopenia. Lymphopenia was observed during the active period of the disease. Mild thrombocytopenia is common, as well as quality changes of the platelets. The sedimentation rate is high in nearly all patients with SLE and this is a non-specific index of the disease activity. These changes have seldom been described in our area.
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PMID:[The hematological manifestations in 111 patients with systemic lupus erythematosus]. 191 69

This study presents 8 dogs of German Shepherd breed (6 males, 2 females, 2-5 years of age at onset of the disease) with a lupus like syndrome characterized by febrile polyarthritis, wasting, nephropathy, cutaneous lesions and high positive titres of ANA (antinuclear antibodies) of speckled type. The serum autoantibodies were further characterized by double immunodiffusion against ENA (extractable nuclear antigen), ELISA for Histone antibodies (Histon fraction H-24A and H-3S), indirect IF on rat-liver sections, non treated and RNase/DNase digested sections for DNP/RNP antibodies, and smears of a hemoflagellate C. luciliae for antibodies vs doubbel strained DNA, (dsDNA). Thus, the high ANA titres in these dogs represent varying types of autoantibodies against nucleoproteins of both DNA and RNA nature, associated histone antigens and non-histone antibodies (RNA and Sm) as well. Rheumatoid Factor titres in serum from these dogs were low or negative. Immunoglobulin deposits at dermo-epidermal junctions were demonstrated in some of the dogs with hyperkeratotic skin lesions. High concentration of serum-IgG was a constant finding in combination with anemia and in most cases leukopenia probably related to the chronic inflammatory process in these animals. Autoimmune hemolytic anemia (AIHA) or thrombocytopenia was not detected in these dogs.
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PMID:Serum auto antibodies and clinical/pathological features in German shepherd dogs with a lupuslike syndrome. 195 Aug 49

We describe a patient with X-linked chronic granulomatous disease (CGD) who developed systemic lupus erythematosus, which was characterized by photosensitivity, malar rash, glomerulonephritis, leukopenia, hypocomplementemia, antinuclear antibodies, and anti-double-stranded DNA antibodies, at age 3. The patient's mother is an asymptomatic carrier of CGD, and her other son (the patient's half-brother) also has CGD. Neither the mother nor the brother has clinical or serologic evidence of systemic lupus erythematosus. Previous cases of discoid lupus-like skin lesions have been reported both in carriers and in patients with CGD. Our patient represents the first reported case of an individual with convincing clinical, serologic, and pathologic evidence of systemic lupus erythematosus. The association between defective host defense mechanisms and autoimmune phenomena has been described previously in patients with Job's syndrome and in patients with B cell and T cell deficiency disorders, including the acquired immunodeficiency syndrome. The relationship between the known leukocyte defects in CGD and the pathogenesis of a lupus-like illness is unclear.
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PMID:Systemic lupus erythematosus in a boy with chronic granulomatous disease: case report and review of the literature. 198 66

Inherited deficiencies of classical pathway complement components are rare and associated with autoimmune diseases and with increased susceptibility to bacterial infections. We report the clinical evolution and studies of the complement system in a 17-year-old female patient of Swiss origin presenting with systemic lupus erythematosus (malar rash, photosensitivity, leukopenia and antinuclear antibodies), in whom the hemolytically active second complement component (C2) was less than 10% of the normal value and antigenic C2 was not detectable. Linkage studies showed that the patient is HLA-A25, B18 positive and has the slow factor B allotype BfS. Further immunological assessment revealed low IgG4 concentrations in the patient, who had the G2M(23) allotype. The asymptomatic first degree family members had half-normal C2 levels compatible with a heterozygous state of C2 deficiency. Therapy with hydroxychloroquine for 17 months and topical sunscreen preparations produced marked clinical improvement. During the 4 years of follow-up, the patient has been well and shown only an abnormal titer of antinuclear antibodies. No infections were observed. To the best of our knowledge, 99 cases of homozygous C2 deficiency have been described so far and are discussed here.
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PMID:[Systemic lupus erythematosus associated with homozygous C2 deficiency. Apropos of a case report and literature review]. 202 45

We describe a pair of monozygotic twins who are concordant for myasthenia gravis but discordant for systemic lupus erythematosus (SLE). SLE developed in twin 1 18 years post-thymectomy and has been characterized by recurrent transverse myelitis and optic neuritis. Twin 2 remains well post-thymectomy, except for a skin rash and persistent leukopenia. Both twins have developed autoimmune thyroid disease. We review genetic and environmental factors of importance in the pathogenesis of SLE and discuss the possible role of thymectomy in the etiology of the disease.
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PMID:A pair of monozygotic twins who are concordant for myasthenia gravis but became discordant for systemic lupus erythematosus post-thymectomy. 205 38

Two patients developed clinical and laboratory evidence of systemic lupus erythematosus (SLE) during treatment with valproate (VPA) preparations. The first patient, a 47-year-old man, had fever, malaise, and thrombocytopenia 1 month after VPA was added to phenytoin (PHT) and primidone (PRM). He developed high titers of antinuclear antibodies (ANA) and anti-DNA antibodies, and hypocomplementemia. After discontinuation of PHT and VPA, steroid and immunoglobulin treatment was required for 4 weeks before his condition improved. The second patient, a 28-year-old woman, had been followed for idiopathic leukopenia for 3 years and had previously experienced fever and lymphadenopathy from PHT. After 4 months of divalproex therapy, she developed confusion, joint pain, and a dramatic increase in seizure frequency. She also developed high titers of ANA and anti-DNA antibodies and hypocomplementemia, along with a further decrease in white blood cell (WBC) count. These responded to steroid therapy and withdrawal of divalproex. Three months later, reintroduction of divalproex was followed by a return of ANA in low titer, which resolved after discontinuation. We believe that VPA may have caused true SLE in these patients, one of whom was probably predisposed.
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PMID:Possible induction of systemic lupus erythematosus by valproate. 211 70

Although many reports have been made on the effectiveness of plasma exchange (PE) in active systemic lupus erythematosus (SLE), but there are no clear-cut criteria of indication for lupus nephritis (LN). In order to determine the criteria for indication of LN, 35 patients with LN treated by PE were studied with respect to renal function (F), proteinuria (P), immunological activity (A) and renal histology. The patients were divided into 4 groups according to the severity of renal function: F1 (stable chronic renal failure (CRF) or stable renal function n = 13), F2 (relapse type n = 9), F3 (rapidly progressive LN; creatinine clearance (Ccr) less than 40 ml/min n = 7) and F4 (acute renal failure; Ccr less than 10 ml/min n = 6). Proteinuria was also studied in 4 groups: P1 & P2 (without nephrotic syndrome (NS], P3 (acute type NS n = 15), and P4 (chronic type NS n = 7). These patients were divided into 3 groups to study immunological activity: A1 (chronic stage n = 6), A2 (relapse stage n = 12) and A3 (active stage n = 17). Comparison was made in each parameter. Renal histological classification according to WHO criteria of LN, activity score (AS) and chronicity score (CS) were evaluated and compared. As a result, the following indication was obtained. 1) ABSOLUTE INDICATION: 1. Rapidly progressive LN with high immunological activity; elevated serum creatinine (SCr) greater than 1.0 mg/dl/month or decreased Ccr from normal renal function to less than 40 ml/min within 1-2 months after onset. 2. Acute type NS within 1 year after onset. 3. histological AS greater than 20. 2) RELATIVE INDICATION: 1. Relapse LN with moderate immunological activity, decreased Ccr from normal function to 40-50 ml/min within 3-6 months, the rise in SCr of greater than 1.0 mg/dl/month. 2. Proteinuria is 1.0-3.5 g/day within 1 year after onset. 3. Such complication as CNS, serositis, thrombocytopenia and leukopenia, steroid resistance and/or severe side effects of steroid. 3) NO INDICATION: 1. CRF or stable renal function (Ccr greater than 50 ml/min). 2. Chronic type NS over 1 years with past history of NS and/or edema. 3. Low immunological activity and mild renal histology.
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PMID:[The criteria for indication of plasma exchange on lupus nephritis]. 221 18

A 30-year-old woman, who in the past was diagnosed as having systemic lupus erythematosus (SLE) and had been treated accordingly, consulted us with neuropsychiatric symptoms, including mutism and catatonia. ANA and anti-dsDNA in serum were negative, and she did not fulfill the ARA criteria for a diagnosis of SLE. However, in view of her history and the fact that she developed convulsions and a transient, unexplained period of leukopenia during admission, her current illness could be interpreted as a cerebral manifestation of SLE. After increasing the dosage of prednisone, she recovered almost completely from this episode. The literature concerning possible procedures to diagnose neuropsychiatric manifestations of SLE is reviewed; it turns out that especially the primary form of cerebral SLE often causes diagnostic problems.
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PMID:[Cerebral disseminated lupus erythematosus; brain-racking for patient and physician]. 225 54

The effect of sex hormones on the induction of experimental systemic lupus erythematosus (SLE) with a human anti-DNA (16/6 Id +) antibody, was studied. We found that injection of the pathogenic idiotype to BALB/c females and orchiectomized males treated with estrogen caused a rapid outburst of the disease 3 months after immunization, while nonestrogen treated mice developed the disease 5 months after immunization. The flare of SLE disease was characterized by raised levels of autoantibodies in the sera to dsDNA, histones, cardiolipin, Sm, RNP, SSA (Ro), SSB (La) and an emergence of high titers of mouse antibody carrying the 16/6 Id. These enhanced antibody levels were associated with an increase in erythrocyte sedimentation rate, proteinuria and leukopenia. Immunofluorescent studies confirmed the existence of immune complexes in the afflicted kidneys. Testosterone treated BALB/c females and orchiectomized males developed a classical response to the human anti-DNA antibody (16/6 Id +), but failed to develop fulminant SLE-like disease. Our data demonstrate the importance of sex hormones on the induction of experimental SLE-like disease in mice with no genetic tendency to autoimmunity.
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PMID:Sex hormone involvement in the induction of experimental systemic lupus erythematosus by a pathogenic anti-DNA idiotype in naive mice. 233 51

The influence of age on the prevalence of individual clinical manifestations of systemic lupus erythematosus (SLE) has not been adequately distinguished from racial or gender influences. Therefore, we examined variations in the clinical manifestations of SLE with age in a group of 361 patients. Multivariate regression techniques, including logistic regression and analysis of covariance, were used to identify clinical features associated with age, while controlling for important confounding factors, including race, gender, duration of followup, and treatment effects. Lymphopenia was found more frequently with increasing age, while malar rash, seizures, false-positive VDRL, thrombocytopenia (in whites), proteinuria (0.5-3.5 g/day), elevated antidouble stranded DNA antibodies, and hypocomplementemia were found less frequently. No age relationship was found for the prevalence of 16 of 24 clinical features examined, including the important disease manifestations of arthritis, serositis, psychosis, nephrotic-range proteinuria, renal failure, autoimmune hemolytic anemia, and leukopenia. The use of regression analysis allows the recognition of similarities and differences in cumulative clinical features of SLE due to age in isolation from the effects of other demographic factors.
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PMID:Age associated clinical manifestations of systemic lupus erythematosus: a multivariate regression analysis. 234 26


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