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)

Anti-neutrophil antibodies of the immunoglobulin G (IgG) class have been implicated in the pathogenesis of autoimmune neutropenia, but few reports have described immunoglobulin M (IgM) anti-neutrophil antibodies. To investigate the prevalence of IgM anti-neutrophil antibodies, sera from 130 patients with possible autoimmune neutropenia were studied for IgG and IgM anti-neutrophil antibodies using an immunofluorescence flow cytometric assay. Twenty-five patients (19%) had IgG anti-neutrophil antibodies exclusively, 21 patients (16%) had both IgG and IgM anti-neutrophil antibodies, and 11 patients (8%) had IgM anti-neutrophil antibodies exclusively. Immunoglobulin M anti-neutrophil antibodies were found in adults and children with isolated chronic neutropenia and in patients with Felty's syndrome, systemic lupus erythematosus, immune thrombocytopenic purpura, and human immunodeficiency virus. Patients with neutropenia with only IgM anti-neutrophil antibodies comprised almost 20% of antibody-positive patients in this study.
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PMID:Anti-neutrophil antibodies of the immunoglobulin M class in autoimmune neutropenia. 804 48

The clinical presentation, risk factors, laboratory data, and neuroimaging and neuropathological findings in 26 patients with autopsy proved central nervous system (CNS) aspergillosis are reviewed. Eleven patients had hematological malignancies (8 underwent bone marrow transplantation), 8 patients underwent liver transplantation, and 3 patients had acquired immunodeficiency syndrome. Four had illnesses resulting in immunosuppression (systemic lupus erythematosus, infected aortic graft, neuroblastoma, and fulminant hepatic failure). The most common presenting clinical symptoms of CNS aspergillosis were fever and a strokelike syndrome. Risk factors for developing CNS aspergillosis included neutropenia, immunosuppressive therapy, low CD4 counts, and retransplantation. Spinal fluid findings were nondiagnostic. Computed tomograms and magnetic resonance scans of the head showed low-density lesions or hemorrhagic infarctions. Most aspergillosis cases occurred in the setting of widely disseminated disease commonly arising from the lung. Pathologically, multiple areas of necrosis throughout the brain were seen. Aspergillus invasion of blood vessel walls was seen microscopically. Amphotericin B with or without flucytosine was not effective treatment.
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PMID:Central nervous system aspergillosis. Analysis of 26 patients. 806 80

Kikuchi's disease is rare necrotic histiocytosis of the lymph nodes. Since the first description in Japan in 1972 by Kikuchi, several cases have been reported. The disease occurs most frequently in young women. Manifestations include enlargement of the cervical lymph nodes, sometimes with fever, and often associated with other non-specific clinical signs. Blood chemistry, including immunologic tests, are often normal but neutropenia and raised erythrocyte sedimentation rate have been reported. Diagnosis relies on the histological examination of lymph node biopsies. Generally the clinical course is favourable in 3-4 months. Secondary systemic lupus erythomatosus may develop and require regular follow-up. The aetiology of this rare disease is still unclear although certain observations would favour an immunological process. Very rare in France, we report a case of Kikuchi's disease in a pregnant woman and present a review of the literature.
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PMID:[Kikuchi's disease. Apropos of a case. Review of the literature]. 809 19

This report describes the clinicopathological features of two Chinese patients with histiocytic necrotizing lymphadenitis without granulocytic infiltration (Kikuchi-Fujimoto disease). Both cases presented painless enlarged lymph node in the posterior cervical triangle, neutropenia and lymphocytosis. One patient had detectable serum antinuclear antibody. Microscopically, the lymph node showed distinctive patch fibrinoid necrosis in the paracortical areas. The necrosis was characterized by florid nuclear dusts engulfed by histiocytes or distributed extracellularly and absence of polymorphonuclear leukocytes. Immunohistochemical studies revealed that the cells in necrotic areas were essentially histiocytes or T lymphocytes. This disorder has a good clinical course, and generally heals spontaneously with no treatment. Recognition of this disorder is, otherwise, imperative not only for the frequent concern for malignant lymphoma, but equal for consideration be given to systemic lupus erythematosus.
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PMID:Histiocytic necrotizing lymphadenitis without granulocytic infiltration (Kikuchi-Fujimoto disease): a report of two cases. 817 4

A series of 213 neutropenic patients were tested for the presence of granulocyte antibodies using the granulocyte chemiluminescence test (GCLT) and the granulocyte immunofluorescence test (GIFT). Sera containing lymphocyte (HLA) antibodies were excluded from the study. A direct GIFT was performed on granulocytes from 56 patients. Samples were obtained from patients with a range of clinical conditions including primary adult autoimmune neutropenia, autoimmune neutropenia of infancy, autoimmune neutropenia secondary to Felty's syndrome, rheumatoid arthritis, idiopathic thrombocytopenic purpura, systemic lupus erythematosus, proliferative disorders, bone marrow transplantation and patients with documented febrile or pulmonary transfusion reactions. Overall, granulocyte antibodies were detected in 52.1% of patient sera. Results for the GCLT and GIFT (IgG) were strongly correlated (p < 0.001) for both primary and secondary immune neutropenias. The results confirm the applicability of the GCLT in the granulocyte serology laboratory.
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PMID:Prospective evaluation of the chemiluminescence test for the detection of granulocyte antibodies: comparison with the granulocyte immunofluorescence test. 818 97

Antineutrophil antibodies may be found in the sera of patients with chronic neutropenia as well as in the sera of a variety of patients with neutropenia and associated autoimmune or infectious disorders. We evaluated an immunofluorescent flow cytometric technique for the measurement of antineutrophil antibodies in serum. Sera from patients with suspected immune neutropenia were studied and compared with a group of sera from normal healthy individuals, as well as with sera from patients with rheumatoid arthritis and systemic lupus erythematosus. Of 159 patients with suspected immune neutropenia and a variety of associated clinical disorders, 59 (37%) were found to have evidence for enhanced binding of IgG to normal target neutrophils, interpreted as positive for antineutrophil antibodies. Whereas 0/37 non-neutropenic patients with typical RA had positive results, 51/244 (21%) of sera from nonneutropenic patients with SLE or other collagen vascular disorders showed enhanced IgG binding to neutrophils. Living neutrophils were used to study the effects of cellular activation, and increased antibody binding was observed with certain sera that contained IgG directed against activation-dependent antigens. We found that, under controlled conditions, flow cytometry can be reliably used to detect antineutrophil autoantibodies, with unfixed, living neutrophils as antigenic targets.
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PMID:Detection of antineutrophil autoantibodies by flow cytometry: use of unfixed neutrophils as antigenic targets. 827 57

Although myelofibrosis has been described in systemic lupus erythematosus (SLE), this coexistence must be rare since there are few reports showing this combination. The possible relationship between hematologic abnormalities and SLE remains unresolved. The authors describe a 39-year-old woman with persistent neutropenia and SLE in whom myelofibrosis was found. Unlike previously reported cases, myelofibrosis did not resolve with steroid therapy. In this report, the clinical course of the patient is compared with others described in the literature.
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PMID:Neutropenia associated with myelofibrosis in systemic lupus erythematosus. 817 40

Agranulocytosis is a well recognized but uncommon complication of procainamide (PA) therapy, whereas a lupus-like syndrome occurs in approximately 20% of patients treated chronically with PA. In order to gain insight into the immunopathogenic relationships among these conditions, we compared the humoral immune abnormalities in these patient groups as well as in asymptomatic PA-treated patients. A relatively uniform profile of IgM but not IgG autoantibody reactivity with a set of chromatin-related antigens was observed in eight elderly men who developed agranulocytosis after treatment with PA. In contrast PA-induced lupus patients had predominant reactivity with [(H2A-H2B)-DNA] in both IgM and IgG classes. Five of eight patients with agranulocytosis had elevated levels of neutrophil-reactive IgG which appeared to be due to immune complexes based on Fc gamma receptor blocking studies. However, 12 of 15 patients with PA-induced lupus, none of whom had neutropenia, had similar levels of neutrophil-reactive IgG, suggesting that this reactivity was not causally related to agranulocytosis. Agranulocytosis developed after less than 3 months treatment with PA in six of eight patients. This time course was similar to that seen in 77 PA-induced agranulocytosis patients reported in the literature plus 127 patients reported to the U.S. Food and Drug Administration in whom 90% developed agranulocytosis within 3 months of starting PA. In contrast, the mode duration of treatment with PA before lupus-like symptoms develop is 10-12 months. These findings, together with the different profiles of autoantibodies and clinical presentations, suggest that agranulocytosis arises from a different mechanism than that underlying PA-induced lupus.
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PMID:Procainamide-induced agranulocytosis differs serologically and clinically from procainamide-induced lupus. 862 53

Our study describes the presence of antineutrophil cytoplasmic antibodies (ANCA) in a group of different pathologies comprising 101 patients. Rheumatoid arthritis, systemic lupus erithematosus, idiopatic neutropenia, acute post-streptoccocal glomerulonephritis, minimal change nephrotic syndrome, Downs syndrome, adult periodontitis, tumoral calcinosis, monoartheritis and lipodystrophy were investigated for ANCA, through indirect immunofluorescence and an indirect solid-phase immunoassay (ELISA). Our results show the pattern of distribution of ANCA in the diseases investigated, and allowed us to make the first description of ANCA in diseases such as Downs syndrome, acute post-streptococcal glomerulonephritis and adult periodontitis. The high percentage of reactivity for ANCA detected in adult periodontitis, raise important questions about the possibility of reporting inaccurate percentages of positivity for some diseases, due to the presence of a concurrent disease such as adult periodontitis.
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PMID:[Antineutrophil cytoplasmic antibodies (ANCA): study of their presence in diseases not associated with arteritis]. 871 20

Nine patients with SLE-associated neutropenia and infections received 48 Mio U G-CSF per day s.c. for 2-17 days as an adjunct to antibiotic treatment. Granulopoiesis was normal or hyperplastic in all cases. The mean granulocyte count increased within 2 days from 1.4 per nl to 11.4 per nl. Side-effects were exacerbating CNS symptoms in two patients and a case of leukocytoclastic vasculitis. G-CSF induced constantly a rapid and distinct increase of neutrophil granulocytes in lupus-associated neutropenia patients with normo- or hyperplastic granulopoiesis.
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PMID:[G-CSF in patients with lupus-associated neutropenia and infections]. 876 47


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