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Systemic lupus erythematosus presenting as panniculitis (lupus profundus).

Six patients are described in whom panniculitis was a major manifestation of systemic lupus erythematosus. These patients were seen in a combined-clinics population of 270 patients with systemic lupus erythematosus for an incidence of approximately 2%. Panniculitis was the first symptom of systemic lupus erythematosus in three of these patients indicating that systemic lupus erythematosus should be considered as an underlying cause in patients with panniculitis or Weber-Christian's disease. Analysis of these six cases and those previously reported suggests that the addition of hydroxychloroquine to the treatment regimen may be beneficial in lupus panniculitis.

Adolescent

Drug-induced lupus erythematosus with in vivo lupus erythematosus cells in pleural fluid.

Pleural involvement in drug-induced lupus erythematosus is not uncommon. Lupus erythematosus cells were found in vivo in the pleural of an elderly patient who had received procainamide (Pronestyl) hydrochloride (2 gm daily) for nine months. Patients who initially have pleural effusions while receiving drugs capable of inducing lupus erythematosus should have the fluid analyzed for lupus erythematosus cells to help clarify the cause of the effusion.

Aged

Lupus erythematosus cell preparation-antinuclear factor incongruity. A review of diagnostic tests for systemic lupus erythematosus.

The cases of 20 patients, each of whom has a positive lupus erythematosus cell preparation and a negative antinuclear factor test, are presented. The concept of a false-positive lupus erythematosus preparation is suggested. Five common mechanisms causing a false-negative antinuclear factor test are discussed and evaluated. Clinical material from the 20 patients is described and pitfalls in diagnosing systemic lupus erythematosus are reviewed.

Antibodies, Antinuclear

[Diagnostic problems associated with the coincidence of bullous dermatosis and lupus erythematosus visceralis. Demonstration of a case with Duhring's dermatitis herpetiformis in association with visceral lupus erythematosus].

This report describes a patient, whom we observed for 3 years, exhibiting a coincidence of dermatitis herpetiformis Duhring and systemic Lupus erythematosus. The difficulties involved in demarcating bullous lupus erythematosus from the more rare association of bullous pemphigoid or dermatitis herpetiformis with systemic lupus erythematosus are discussed in connection with differential, histological, direct and indirect immunofluorescence diagnostic aids.

Adult

[Morphologic changes in the chorio-allantoic membrane and organs of chick embryos inoculated with blood of patients with systemic lupus erythematosus (concerning the viral etiology of systemic lupus erythematosus)].

Morphological studies of 14 chorionallantoic membranes and organs of chick embryos which had been infected on the 7--8th day of incubation with leucocytic mass from patients with systemic lupus erythematosus were carried out. Corresponding virological and morphological control studies were performed. The changes observed were repeatedly reproduced in multiple inoculations (from the 2nd to the 16th passage). Apart from morphological changes, which may be listed among general pathological processes (impairment of the circulation, dystrophy, proliferation), changes testifying to a possible cytopathic effect disintegration of nuclei, formation of gigantic cells and multinuclear symplasts were also noted. The most pronounced changes, both in the chorionallantoic membrane and in internal organs, were observed on the 3rd--5th passage and on the 3rd--5th day of incubation following inoculation. They correlated with the clinical activity of the process in patients from whom the blood for inoculation had been taken. The data obtained justify the assumption concerning the existence in the blood of the patients with systemic lupus erythematosus a transplantable agent producing a cytopathic action, and may be considered as a new indirect corroborative evidence in favour of the concept of the viral nature of systemic lupus erythematosus.

Animals

Clq binding activity in lupus erythematosus: correlation with the "lupus band test".

A 131I C1q binding assay was employed to estimate C1q binding activity (C1q BA), presumably due to the presence of circulating immune complexes, in lupus erythematosus sera. Thirteen of 16 sera from patients with systemic lupus erythematosus (SLE) had elevated C1q BA. Only 1 of 17 sera from patients with generalized discoid LE (GDLE) and none of 5 localized DLE patient's sera had elevated C1q BA. A correlation between a positive LE band test and the level of C1q BA was apparent. A positive LE band test tended to occur in patients with higher levels of C1q BA, whereas a negative LE band test tended to occur in patients with low levels of C1q BA.

Adolescent

Uncovering potential biomarkers and metabolic pathways in systemic lupus erythematosus and lupus nephritis through integrated microbiome and metabolome analysis.

OBJECTIVE: This study aims to explore the relationship between gut microbiota and fecal metabolomic profiles in patients with systemic lupus erythematosus (SLE), with and without lupus nephritis (LN), in order to identify potentially relevant biomarkers and better understand their association with disease progression. METHODS: Fecal samples from 15 healthy controls (HC) and 36 SLE patients (18 SLE-nonLN and 18 SLE-LN) were analyzed using 16S rRNA gene sequencing and untargeted metabolomics. Differential microbial taxa and metabolites were identified using Linear Discriminant Analysis Effect Size (LEfSe) and Orthogonal Partial Least Squares Discriminant Analysis (OPLS-DA). Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway and Receiver Operating Characteristic (ROC) curve analyses were used to assess the potential clinical relevance of selected metabolites. RESULTS: Beta diversity analysis demonstrated distinct microbial clustering between groups (p&#x2009;<&#x2009;0.05). SLE-LN samples showed an increased relative abundance of Proteobacteria and decreased Firmicutes compared to SLE-nonLN. Metabolomic profiling identified multiple differentially abundant metabolites, with notable enrichment in primary bile acid biosynthesis pathways (e.g., Glycocholic acid, AUC&#x2009;=&#x2009;0.951). In the SLE-nonLN group, increased Glycoursodeoxycholic acid levels (AUC&#x2009;=&#x2009;0.922) were observed in pathways related to taurine and hypotaurine metabolism. Correlation analysis indicated a negative association between Escherichia-Shigella and bile acid levels (p&#x2009;<&#x2009;0.01). CONCLUSION: This integrative analysis suggests that patients with SLE and LN harbor distinct gut microbiota and metabolomic profiles. The identified microbial taxa and metabolites may have potential as non-invasive biomarkers and could contribute to a better understanding of SLE pathogenesis and progression.

Humans

Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset.

We have characterized the clinical and laboratory features of 27 patients who had in common a recurring, superficial, nonscarring type of cutaneous lupus erythematosus (LE) that occurred in a characteristic distribution (subacute cutaneous lupus erythematosus [SCLE]). This clinically distinct form of cutaneous LE has not previously been analyzed as a separate entity and thus, its clinical importance has not been fully appreciated. We found that these patients frequently had a mild systemic illness marked by musculoskeletal complaints and serologic abnormalities. Forty-eight percent had systemic LE by American Rheumatism Association criteria; however, none had serious CNS or renal disease. Thus, those with SCLE are a subset of patients with LE who generally have an illness intermediate in severity between discoid LE and severe systemic LE.

Adult

Lupus cardiomyopathy: cardiac mechanics, hemodynamics, and coronary blood flow in uncomplicated systemic lupus erythematosus.

Right and left heart pressures, left ventricular volumes, indices of contractility, myocardial wall stiffness, and coronary blood flow were determined in five young women with systemic lupus erythematosus (SLE) during diagnostic right and left heart catheterization. Examinations revealed (1) increases of right and left ventricular enddiastolic pressures; (2) decreases of cardiac output, stroke volume, ejection fraction, contractility indices, diastolic left ventricular volume inflow; (3) decreases of pharmacologically induced coronary vasodilation in SLE. The results demonstrate impaired pump function, reduced contractility, increased myocardial wall stiffness, and decreased coronary vascular reserve in SLE. It is concluded that lupus cardiomyopathy associated with an impairment of left ventricular function may be apparent in young women with SLE who have no clinical signs of cardiac dysfunction.

Adult

Pulmonary manifestations of systemic lupus erythematosus: review of twelve cases of acute lupus pneumonitis.

Acute lupus pneumonitis was the presenting manifestation of systemic lupus erythematosus in six of 12 cases in this series. The clinical picture was characterized by severe dyspnea, tachypnea, fever and arterial hypoxemia. Radiographic findings included an acinar filling pattern which was invariably found in the lower lobes and was bilateral in 10 of the cases. Studies failed to reveal evidence of infection as a cause of the acute pulmonary infiltrates. All patients were treated with oxygen and corticosteroids; seven received azathioprine. Six patients survived and are clinically well 14 months to four years following their acute illness. Three of these patients have residual interstitial infiltrates with persistent pulmonary function test abnormalities indicating progression to chronic interstitial pneumonitis. Histologic sections of the lungs available from four patients revealed hyaline membranes and interstitial edema (four cases), acute alveolitis (two cases), arteriolar thrombosis (one case) and a prominent lymphocytic interstitial pneumonitis with organizing bronchiolitis (one case).

Acute Disease

Recurrent venous thrombosis with a "lupus" coagulation inhibitor in the absence of systemic lupus.

A young man presenting with recurrent deep venous thrombosis was found to have a lupus type coagulation inhibitor. He showed neither clinical nor serological evidence of systemic lupus. The value of the Russell viper venom coagulation time in the detection of the inhibitor is demonstrated. Anticoagulant therapy has not caused any bleeding complication despite the presence of the inhibitor.

Adult

Problems in diagnosis and treatment of lupus psychosis. Report of a patient with systemic lupus erythematosus, Hashimoto's thyroiditis, and Sjögren's syndrome.

A 34-year-old woman with an organic psychosis was presumed to be suffering from corticosteroid psychosis because of prednisone treatment for urticaria. Lupus was found to be the cause of both urticaria and psychosis. The lupus psychosis was reversed when sufficiently high doses of steroids were given, in combination with immunosuppressant agents.

Adult

Lupus erythematosus cells in pleural effusion: the initial manifestation of procainamide-induced lupus erythematosus.

A 63-year-old man developed an asymptomatic pleural effusion following the administration of 500 gm of procainamide hydrochloride over a six-month period. The diagnosis was initially suggested by the finding of lupus erythematosus cells in the pleural fluid. Lupus erythematosus cells and antinuclear antibodies appeared in the blood two months later and remained for a period of six months. The diagnosis was corroborated by the presence of antibodies to denatured DNA, but not to native DNA.

Antibodies, Antinuclear

[A special form of lupus disease. Deep cutaneous lupus. Apropos of 2 cases].

Deep cutaneous lupus is a clinical form of lupus disease for which a very old description has recently been brought up to date. Two new cases are reported here and a review of the literature makes it possible to analyse the clinical, biochemical, histological, and immunological features of this panniculitis. Different from the Weber-Christian syndrome, it is characterized by repeated eruptions of nodules and/or subcutaneous plaques, and histologically by vasculitis, lymphocyte infiltration, and sometimes te presence of immunoglobulins on the basal dermal membrane and around the vessels.

Adipose Tissue

Discoid lupus erythematosus as part of a larger disease spectrum. Correlation of clinical features with laboratory findings in lupus erythematosus.

This study compares the immunologic features of a homogeneous group of patients with discoid lupus erythematosus (DLE) strictly limited to the skin (group 1) with those of patients with active discoid skin lesions plus visceral involvement (group 2) and with those of lupus erythematosus (LE) patients with proliferative glomerulonephritis (group 3). Positive antinuclear antibody (ANA) was found in 4% of group 1, 93% of group 2, and 100% of group 3. Low total hemolytic complement (CH50) was found in 4% of group 1, 47% of group 2, and 100% of group 3. Antibodies to native DNA (nDNA) were not found in group 1, were rarely found in group 2, and were present in nearly all patients in group 3. No group 1 patient had subepidermal immunoglobulin deposits in normal skin, 20% of group 2 had this finding, and 100% of group 3 had this finding. The ability to develop chronic discoid skin lesions appears to be associated with a reduced incidence of immunologic parameters of disease activity. The data suggest that patients with active discoid skin lesions rarely have severe renal disease.

Antibodies, Antinuclear

Diagnosis of lupus nephritis by skin immunofluorescence, in the absence of extrarenal manifestations of systemic lupus erythematosus.

Six patients with glomerulonephritis were found to have granular deposits of complement and/or immunoglobulins at the dermalepidermal junction of normal skin. No patient had extrarenal clinical manifestations of systemic lupus erythematosus (SLE). The only serologic test suggestive of SLE was a positive antinuclear antibody (ANA) reaction; results of complement and antinative deoxyribonucleic acid (DNA)-antibody tests were repeatedly normal. The patients with glomerulonephritis had a favorable initial response to therapy with prednisone with or without azathioprine. These patients may represent a variant of SLE in which the diagnosis can only be established by a direct immunofluorescence test of normal skin. Alternatively, they may constitute a separate new clinical entity. Because of the favorable response to therapy, we suggest that skin immunofluorescence be performed in patients who present with unexplained glomerulonephritis and a positive ANA.

Adolescent