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)

This paper details current and historic epidemiologic features of Fogo Selvagem (Endemic pemphigus foliaceus) in Brazil. The following features are described. a) The disease occurs in endemic fashion in regions of Brazil within the states of Goias, Mato Grosso do Sul, Parana, Sao Paulo, and Minas Gerais. It appears that the disease is spreading toward the northwest and west, involving the states of Mato Grosso, Para, Maranhao, Rondonia, Acre, and Amazonas. b) People at risk are young peasants or children of either sex or any race exposed to the local ecology in rural areas of endemic states. Although the disease has been described in urban centers, these occurrences are rare. c) Fogo Selvagem commonly appears in wild areas being colonized and disappears as these areas become urbanized. d) The majority of patients live in close proximity to rivers and within the 10-15 Km flying range of mosquitos or black flies (such as Simulium). It is hypothesized that a black fly, Simulium pruinosum may be the vector that precipitates the disease. f) There is a significant number of Fogo Selvagem in family units where multiple, genetically related individuals are affected. g) Finally, autoantibodies against lupus-associated antigens are not present in the sera of patients with Fogo Selvagem. Clinical examination of the skin, and serologic screening for pemphigus autoantibodies are specific parameters that can be used in the search for the etiologic agents that lead to autoimmune disease of the skin. To identify and prove an etiologic agent for this well-characterized autoimmune disease would be of tremendous importance to the understanding of autoimmune skin diseases, and potentially other organ-specific autoimmune disorders.
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PMID:Endemic pemphigus foliaceus (Fogo Selvagem): II. Current and historic epidemiologic studies. 264 12

During a survey period of 28 years a total of 449 patients suffering respectively from pemphigus, systemic lupus erythematous and chronic nephritis was admitted to the hospital. Of the 286 patients who received glucocorticoid treatment 28 were found to have steroid diabetes (9.8%). The incidences of steroid diabetes in these diseases were as follows: pemphigus 20% (7/35), systemic lupus erythematous 12.5% (14/112), chronic nephritis 5% (7/139). Two thirds of the diabetic subjects appeared asymptomatic, while the remainder showed polydipsia and polyuria. Renal glucosuria was seen in 3 cases and hyperosmolar hyperglycemia in 4. Blood glucose level in 10 out of the 21 remaining cases was 8.9 +/- 1.5 mmol/L and in the other 11 cases 14.8 +/- 2.4 mmol/L. Generally, the treatment of steroid diabetes is not intricate. Satisfactory improvement was seen in about 80% of the patients if a strict line of therapy against primary diabetes was oriented. In this series 18 patients did well under the treatment either with reduction of steroid or with use of oral hypoglycemic agents or insulin or both. Their blood glucose levels returned to normal and urine sugar disappeared rapidly. Four of the 5 deaths were caused by their primary diseases. One died of pemphigus complicated with infection and shock exhibiting an ante mortem blood glucose level as high as 31.7 mmol/L, obviously hyperosmolar status being the predisposing factor. Due to the fact that most of the steroid diabetic patients were clinically asymptomatic, delayed or misbranded diagnosis was not infrequently seen.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Steroid diabetes: an analysis of 28 cases]. 280 46

Immunotoxicologic studies have demonstrated that autoimmune responses and/or autoimmune diseases are induced in humans and experimental animals by chronic exposure to various chemicals. The present review is focused on seven groups of chemically induced human disorders, i.e. systemic lupus erythematosus, autoimmune hemolytic anemia, myasthenia gravis, pemphigus, glomerulonephritis, thyroiditis and hepatitis. Results obtained from studies of the available experimental counterparts of these diseases, i.e. those models obtained from the exposure of laboratory animals to various chemicals, are then analyzed. Finally, we present the lessons that can be derived from immunotoxicologic investigations regarding mechanisms of induction, heterogeneity of chemicals involved, humoral vs. cellular immune responses and genetic predisposition to chemically induced autoimmunity.
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PMID:Autoimmunity induced by chemicals. 304 16

Based on epidemiologic data, a current hypothesis states that Fogo selvagem (FS) may be triggered by environmental factors present in endemic areas of Brazil. Because the appearance of new cases is limited to those areas, we wanted to ascertain if the presence of the pemphigus autoantibodies was restricted to the patients. To further delineate the restriction of the autoantibody response in these patients we also investigated the presence of lupus-associated autoantibodies. Using indirect immunofluorescence (IF) we tested the sera of patients with FS (n = 196), their relatives (n = 138), their cohabitants (n = 13), and normal donors from endemic (n = 38) and non-endemic areas (n = 44) for pemphigus autoantibodies. Antinuclear antibodies (ANA) and anti-nDNA antibodies were determined by indirect IF against Hep-2 cells and Crithidia lucilliae, respectively. Autoantibodies against nRNP, Ro/SSA, La/SSB, and Sm were assayed by double immune diffusion in agarose gels. FS autoantibodies were present in the sera of all patients with active disease (n = 196, 100%, titers greater than 40 to 2560), but were not found in any sera from normal individuals in endemic or non-endemic areas. The titer of the FS autoantibody showed a rough correlation with the extent and activity of the disease. Furthermore, lupus-associated autoantibodies were not present in any of the tested samples. We conclude the FS antiepidermal autoantibodies are specific serologic markers of the disease and are not present in unaffected individual from the endemic areas. As such, they provide an important marker that should be useful in ongoing epidemiologic studies aimed at identifying putative etiologic agent(s).
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PMID:Serologic abnormalities in patients with endemic pemphigus foliaceus (Fogo selvagem), their relatives, and normal donors from endemic and non-endemic areas of Brazil. 329 5

D-Penicillamine is effective in the treatment of Wilson's disease, cystinuria and rheumatoid arthritis. However, it may have adverse side-effects by inducing a spectrum of diseases such as myasthenia gravis, lupus-like disease, IgA deficiency and pemphigus vulgaris. A case of D-penicillamine-induced pemphigus is presented. The clinical aspects, pathogenesis, immunology and therapy of D-penicillamine-induced diseases are discussed.
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PMID:[Penicillamine-induced pemphigus]. 331 68

There were 72 patients (19 with hepatic failure, 10 with fulminant hepatitis, eight with paraquat poisoning, eight with rheumatoid arthritis, five with myasthenia gravis, four with hyperlipidemia, four with systemic arteriosclerosis including brain infarction, three with pemphigus vulgaris, two with multiple myeloma, two with systemic lupus erythematosus, two cases non-specific Ig-G antibody, two cases medication with an anticancer drug, one with multiple sclerosis, one with Crohn's disease with amyloid kidney and one with chronic myeloblastic leukemia) treated by plasma exchange in the Kidney Center, Tokai University School of Medicine from Jan. 1983 to Dec. 1986. We performed plasma exchange using fresh frozen plasma in 40 cases and Lactate-Ringer's solution containing albumin (4.0-5.0%) in 20 cases as the replacement fluid. In 17 cases, we performed double filtration plasma exchange with the recycle system and no replacement fluid. Although PE therapy did not constitute a basic therapy for hyperlipidemia, pemphigus vulgaris, rheumatoid arthritis, myasthenia gravis, and systemic lupus erythematosus, it was effective in relieving severe clinical symptoms. At the present time, conventional plasma exchange does not improve the survival rate of patients with hepatic failure and fulminant hepatitis. Developments of a new artificial liver support apparatus and identity of many toxic substances in hepatic failure are necessary. No hypotension, hypovolemic shock or other significant complications were experienced.
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PMID:Clinical reports on plasma exchange in the Kidney Center, Tokai University School of Medicine. 344 83

In an 80-year-old woman with pemphigus erythematosus, we demonstrated ANA as well as anti-DNA antibodies in the serum. This finding supports the argument that this skin disorder represents a combination of a disease of the pemphigus group with systemic lupus erythematosus.
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PMID:[Pemphigus erythematosus--detection of anti-DNA antibodies]. 349 6

The clinical features and investigations of 17 patients were analysed. Thirteen of them were Chinese and the rest Indians. Their ages at presentation ranged from 8 to 63 years (mean 18.35 years). Thirteen patients (76%) were symptomatic; 8 with predominantly hepatic manifestations and 5 with neurological features. Four were asymptomatic siblings. At diagnosis, however, 10(59%) had features of liver involvement singly, 3 (18%) had neurological involvement alone and 4 (27%) had mixed presentations. Family histories were available in 15 patients; 26.9% of siblings had Wilson's Disease. Serum ceruloplasmin was low in 82% of the patients. 24-hour urinary copper was measured in 16 patients and was raised in all of them. About half the patients (41%) had evidence of concomittant renal tubular dysfunction with hypouricaemia and aminoaciduria. Three patients (18%) had joint involvement at presentation. All 17 patients were treated with Penicillamine. Complications due to therapy included pemphigus in one and toxic epidermal necrolysis and later a lupus like syndrome in another. The features of clinical improvement included fading of K-F rings, improvement of neurological signs and the normalisation of serum transaminases. One patient developed primary hepatocellular carcinoma 5 years after presentation. Delay in diagnosis was encountered in half of the patients reviewed. Being a treatable condition, Wilson's Disease, although rare, should always be thought of in patients with haemolysis, liver diseases or extrapyramidal disorders.
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PMID:Wilson's disease revisited in the tropics. 375 94

Fourteen limbal biopsy specimens from 11 dogs with chronic superficial keratitis (CSK) were examined histologically and immunohistochemically. Ten of the 14 specimens had corneal epithelial hyperplasia and/or atrophy. Eleven of the 14 specimens had thickened epithelial basement membranes. Each specimen had cellular infiltration and lamellar disruption of the stroma. An avidin-biotin immunoperoxidase complex stain was used to detect immunoglobulin (Ig) deposition. Twelve of the 14 specimens stained positive for Ig. The staining pattern was consistent and characterized by diffuse deposition of stain in the superficial conjunctival stroma near the limbus. Four of the 12 Ig-positive specimens also stained positive in the superficial corneal stroma with 1 of these 4 also staining positive along the epithelial cell basement membrane. The diffuse pattern of stain deposition and the absence of staining of specific epithelial structures indicated that CSK is not a classical autoimmune disease similar to any disease in the pemphigus group or similar to systemic lupus erythematosus. Although the results may implicate CSK as an immune-mediated disease, nonspecific factors could not be ruled out.
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PMID:Immunohistochemical staining patterns of canine eyes affected with chronic superficial keratitis. 376 2

An association between pemphigus erythematosus and systemic lupus erythematosus is well recognized. However, most reports strongly imply that the clinical manifestations of lupus erythematosus are usually minimal or only serologically significant. We describe the case of a 27-year-old black woman who experienced life-threatening systemic lupus erythematosus while recovering from pemphigus erythematosus. Unique histopathologic findings show the presence of both disorders in the same lesion.
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PMID:Pemphigus erythematosus: a unique association with systemic lupus erythematosus. 376 50


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