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)

POEMS syndrome [plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal (M) protein, skin changes] although mimicking the connective tissue diseases in several ways has not been reported in a patient who also meets criteria for the diagnosis of systemic lupus erythematosus (SLE). We describe a patient with POEMS syndrome and SLE and review the similarities and differences between POEMS syndrome and other connective tissue disorders.
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PMID:POEMS syndrome in systemic lupus erythematosus. 161 12

A healthy 20 yr old woman presented for evaluation following a cervical smear which showed viral effects typical of human papilloma virus. Colposcopy showed changes of cervicitis with the main finding on histologic examination of biopsy material being an acute and chronic cervicitis associated with typical features of cytomegalovirus (CMV) infection. Viral identification was confirmed by immunoperoxidase staining, in situ hybridization and electron microscopy. The patient was lost to follow up for 18 mths. Following this, a repeat colposcopy again showed inflammation, with cervicitis, mild dysplasia and CMV inclusions on biopsy. Full immunological work-up, including human immunodeficiency virus (HIV) study, was performed and was normal. Only 11 other cases of endocervical biopsies with histological evidence of CMV inclusions were found in the literature, although the reported rate of detection of genital CMV in women on culture is 4-12%. In the 9 cases where information was available, endocervical inflammation was present. One patient was on immunosuppressive medication for systemic lupus erythematosus and another was found to have Acquired Immune Deficiency Syndrome (17% of total). These cases demonstrate that although histologic examination is an insensitive marker for CMV within the cervix, its presence may signify immunodeficiency and so immunological assessment of a patient with this finding is advisable.
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PMID:The clinicopathological significance of cytomegalovirus inclusions demonstrated by endocervical biopsy. 166 15

Cerebral ischemia is very rare in children and young adults. There can be a multitude of causes; in many cases etiology remains undetermined. We report here on 7 cases, 11 to 25 years of age. Pathogenetic factors (lupus erythematodes, endocarditis, fibromuscular dysplasia) and risk factors (cigarette smoking, oral contraceptives) were found in 5 patients whereas in 2 cases the etiology was not determinable. Three patients were treated with low weight dextrans, two patients received prostaglandin E1, and in 2 cases regional thrombolysis was performed. Three female patients died, two with occlusions of the rostral part of the basilar artery and one with an occlusion of the carotid artery and lupus erythematodes as the primary disease. Long-term observations of the surviving patients showed good recovery from neurological deficits. Prognosis quoad sanationem seems better in this age group than in elderly patients.
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PMID:Cerebral ischemia in children and young adults. 245 51

According to the past reports, neuropsychiatric manifestations have been seen in 10-75% of patients with systemic lupus erythematosus and are second only to renal involvement as a cause of death. The clinical feature is multiple. And cerebrovascular diseases due to systemic lupus erythematosus are detected in 3-16% of the neuropsychiatric manifestations. Occlusion of the intracranial major arteries is less frequently found in other cerebrovascular diseases. And central nervous system involvement usually occurs at some intermediate or terminal stage of systemic lupus erythematosus, so is rarely regarded as one of the initial symptoms. We studied the case of a patient with systemic lupus erythematosus with occlusion of the right middle cerebral artery indicated by angitis and 'string of beads' appearance of the right internal carotid artery indicated by fibromuscular dysplasia. The patient was a 38 year old female and began to feel weakness in the left hand and developed mild-left hemiparesis due to infarction of right temporo-parieto-occipital lesion which was revealed by CT scan. Carotid angiograph showed irregularity at the right middle cerebral artery and 'string of beads' appearance of the right internal carotid artery. Gradually neurological manifestations improved, but a facial 'butterfly' rash, palmar erythema and polyarthritis were detected.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of systemic lupus erythematosus with neurological manifestations as initial symptoms]. 267 62

Two T-lymphocyte subsets develop in the thymus which differ in the expression of glycoproteins on their cell surface. About 60% of the circulating T cells express the glycoprotein T4, while about 30% have the glycoprotein T8. T4 and T8 cells can be determined in the peripheral blood or various organs with monoclonal antibodies. T4 and T8 cells differ in their antigen recognition, have different functions, and can cause various pathohistological changes. T4 cells recognize the antigen in association with the HLA-D/DR/DP determinants. Upon antigenic stimulation they liberate various factors and initiate and amplify an immune response (T4 = helper/inducer T-cells). They can also be cytotoxic and are mediating effector functions via macrophage activation. T8 cells recognize the antigen in association with HLA-A/B/C determinants. They exert their cytotoxic or suppressive effector functions mainly in viral infections. The T4 or T8 cell-mediated pathohistological changes are discussed in the light of the well studied T-cell infiltrations in lepra lepromatosa or lepra tuberculosa. The T4/T8 cell dyscrasia in the peripheral blood, described in a variety of infectious, autoimmune or immunodeficiency diseases, may be due to enhanced proliferation, selective sequestration, reduced production or the elimination of a subset. T-cell subset analysis in joints, bronchial lavages and tissues has clarified the pathomechanism in a variety of autoimmune diseases, although the etiology remains obscure. For example, in rheumatoid arthritis, multiple sclerosis, and sarcoidosis, a T4 cell-mediated reaction with macrophage activation can be found. T4/T8 cell analysis may also be of value in dissecting heterogenous diseases, e.g. systemic lupus erythematosus. Of value is also the additional demonstration of membrane components reflecting T-cell activation (IL-2 receptor or DR-antigen expression) which serves to identify the activated T-cell subset in peripheral blood. Finally, T4/T8 cell analysis can be helpful in deciding treatment, as the T-cell subsets have a different sensitivity to immunosuppressive drugs.
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PMID:[Analysis of T-cell subpopulations. Pathophysiological concept and significance for clinical medicine]. 315 84

An 82-year-old female developed hemolytic uremic syndrome (HUS) after a prodromal illness of bloody diarrhea. No specific enteric pathogen was isolated. A renal biopsy performed 5 days after the onset of azotemia revealed typical thrombotic microangiopathy. By electron microscopy, massive annular-tubular deposits admixed with fibrillar fibrin were demonstrated in glomerular capillaries. Immunofluorescent staining of the intracapillary material was positive for IgG, IgM, C3, C1q and fibrin-related antigens. No evidence of plasma cell dyscrasia, cryoglobulinemia or systemic lupus erythematosus was found, and the patient recovered renal function uneventfully in 2 months. Organized immune deposits appear to have played a role in the pathogenesis of HUS in this patient.
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PMID:Glomerular annular-tubular immune deposits in adult hemolytic uremic syndrome. 326 13

During an evaluation for nephrotic syndrome, a 20-year-old woman was found by ultrasonographic examination to have large kidneys with multiple renal cysts suggestive of polycystic kidney disease. A subsequent renal biopsy revealed membranous glomerulopathy due to systemic lupus erythematosus, as well as the unexpected finding of glomerulocystic kidney disease (GCKD), an uncommon disorder previously reported to occur primarily in infants and children. No evidence of renal dysplasia was present and no cysts were found in any abdominal or pelvic organs. Other than one bifid renal pelvis, no significant congenital anomalies or structural chromosomal abnormalities were present. Ultrasonographic evaluation of the patient's family revealed similar-appearing cortical cysts in several members, all of whom had no clinical evidence of renal dysfunction. The pattern of involvement was compatible with autosomal dominant inheritance. Follow-up ultrasonograms of the patient and affected family members 1 year after the initial study showed enlargement of the cysts with development of additional cysts in two individuals and no change in the other family members. Although renal failure was present and progressed in our patient, renal function remained normal in all affected family members 1 year after detection of the renal cysts. This patient and her family provide additional insight into the inheritance and natural history of GCKD and demonstrate that this condition should be considered in the evaluation of multicystic renal disease in adults. In contrast to several previously reported cases, it appears that GCKD may be associated with normal renal function for many years.
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PMID:Familial adult glomerulocystic kidney disease. 382 63

Neutropenia is a life-threatening sequel of hematological disorders and a dominant factor limiting the dosage of cytotoxic chemotherapy. The role of the neutrophil is of such importance in defence against microbial invasion that measures that modify the behaviour of residual hemopoietic tissue to promote a modest increase in neutrophils, can confer considerable benefit by reducing the frequency and severity of infection. Such a change can be mediated in bone marrow depression by diversion of more progeny of immature precursors into the neutrophil series, or by enhancement of the stimulatory drive operating on neutrophil production. The former effect can be achieved by hypertransfusion of red cells to reduce the demand on the limited precursor population for cells of the erythroid series. The latter effect can be achieved by administration of lithium carbonate. Neutropenia caused by autoimmune injury to the neutrophil series can also be successfully modified by measures which suppress the underlying immune dyscrasia or the function of the reticulo-endothelial system. Corticosteroid administration and splenectomy can be helpful in certain specific types of neutropenia. Administration of cyclophosphamide and azathioprine has both mutagenic and marrow suppressive potential, but can induce remissions in severe chronic isolated neutropenia and in systemic lupus erythematosis.
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PMID:New concepts in management of neutropenia. 385 79

Out of 2,474 bone marrow biopsies we have observed 330 cases (13.3%) with presence of lymphoid nodules (LN). LN were frequent in old age (24.6% over 80 years), in females (17%) and in some diseases, such as rheumatoid arthritis and systemic lupus erythematosus (73.7% of the cases), partial aplasia (34%), hypersplenism (30.4%), hemopoietic dysplasia (25%), chronic renal failure (20.4%), polycythemia vera (20.2%), idiopathic thrombocytopenic purpura (18.8%), acute leukemia (17.7%). Nodular lymphoid hyperplasia of the bone marrow was found especially in systemic autoimmune diseases (26.3%), hypersplenism (9.8%), preleukemia (7.3%) and acute leukemia (4.2%). The presence of excessive medullary LN could indicate a bone marrow microenvironment damage, possibly of autoimmune origin.
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PMID:Lymphoid nodules and nodular lymphoid hyperplasia in bone marrow biopsies. 393 2

Nine patients (2.2%) in a group of 415 who were followed in a longitudinal prospective study of systemic lupus erythematosus (SLE) were found to have various monoclonal (M) proteins in their blood (IgG [6 patients], IgA [2 patients], IgM [1 patient]). No other findings compatible with plasmacytic dyscrasia were found. Bence Jones proteinuria was absent. Bone marrow aspirates and skeletal radiographs did not reveal any associated features of malignancy. Four of the 9 patients were under the age of 50. From the point of view of the M components, 3 groups emerged: transient (2 patients), persistently stable (6 patients), and increasing serum concentrations (1 patient). Using current measures of disease status, no correlation was apparent between the presence, type, and concentration of the M protein and the clinical and laboratory variables of lupus activity. Thus, M proteins were found in 2% of our SLE patients, but their relationship to the polyclonal B cell activation seen in this disorder, or perhaps to therapeutic modalities used in its treatment, remains to be elucidated.
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PMID:Systemic lupus erythematosus with paraproteinemia. 661 Apr 25


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