Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 54 year-old woman who had a 6 month history of polyarthralgias, oral ulcers, weight loss and fatigue was admitted to the Urawa Municipal Hospital. She developed high fever, dyspnea and thrombocytopenia. Chest radiograph revealed massive right pleural effusion. At this time, laboratory investigations gave the following results: hemoglobin 12.7 g/dl, WBC 7700/microliters and platelet count 9.2 x 10(4)/microliters. Antibody to DNA was negative. Antinuclear antibody was positive at a titer of 320x in a centromere pattern; Anti-RNP and anti-Sm antibodies were negative. CH50 was 18.6 u/ml. C3 was 42.9 mg/dl. C4 was 11.5 mg/dl. Circulating immune complex (Clq) was 30.5 micrograms/ml. Circulating lupus anti-coagulant and anticardiolipine antibodies were positive. Thoracocentesis was performed; the material was a straw-colored exudate with over two thousands white cell per ul and showed marked reduction of complement titiers and elevated immune complex levels. She was then diagnosis as having SLE. Two weeks after admission, progressive leukopenia and anemia succeedingly occurred and resulted in severe pancytopenia. Bone marrow biopsy demonstrated marked marrow fibrosis and increased reticulin content with no evidence of malignancy. Steroid pulse therapy for 3 days started, and subsequently she was treated with 60 mg/day of prednisolone. Three weeks after starting on steroids, the massive pleural effusion was completely disappeared and complement titiers were normalized. Circulating immune complex has not been detected any more. After 8 weeks, the peripheral blood count was normalized. The dose of prednisolone was reduced progressively. On this occasion, the biopsy showed normocullular marrow with a marked reduction in the amount of reticulin.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of systemic lupus erythematosus presenting with myelofibrosis as a cause of pancytopenia]. 797 29

A patient with systemic lupus erythematosus developed unexplained fever, nonregenerative anemia, leukopenia, and elevations in serum triglyceride and ferritin levels. Bone marrow studies established the diagnosis of macrophage activation syndrome with active hemophagocytosis. No infectious cause was found but pulmonary nocardiosis developed during the course of the disease. Intravenous gammaglobulin therapy was followed by a transient remission. Cyclophosphamide was given subsequently. In lupus patients, macrophage activation syndrome is exceedingly rare and has the same clinical, laboratory, and histologic features as those seen in patients with hemopathies, infections, or immune deficiencies. Investigations for an underlying infection are often negative, suggesting that the macrophage activation syndrome is due to lupus-related immune changes. Treatment is not standardized and relapses are common. This diagnosis should be considered in lupus patients with febrile pancytopenia.
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PMID:[Macrophage activation syndrome in lupus]. 805 32

The purpose of this study was the descriptive analysis of patients with systemic lupus erythematosus (SLE) with a particular focus on initial clinical features, evolution and outcome of disease, prevalence of clinical and serological manifestations and identification of clinicoserological associations indicative of renal and CNS involvement. The methodology applied was the following: retrospective analysis of the clinical charts of 292 unselected patients (246 female (84.2%) and 46 male (15.7%)) with SLE examined between 1982 and 1992. Multivariate analysis and hierarchical log linear models were used to examine for clinicoserological associations. Descriptive analysis was based on the prevalence of main clinicoserological features and disease outcome. The outcome was examined on the basis of the number of flares, the presence of chronic renal failure, the presence of central nervous system (CNS) involvement with subsequent disability and deaths. Flares were considered the severe alterations in disease status, requiring additional therapy to be controlled. The disease begins most frequently in the second and third decade of life with cutaneous and joint manifestations, while renal and CNS involvement developed later. The prevalence of serious renal, pulmonary and CNS involvement as well as the prevalence of RF, anti-Sm and anti-nRNP antibodies remain low. Multivariate analysis revealed the associations of renal involvement with leukopenia and serositis, of anti-Sm with leukopenia, of secondary Sjogren's syndrome with RF and of thromboembolic events with anticardiolipin antibodies. Patients with childhood onset SLE have a higher tendency for developing renal involvement than adult onset SLE patients. In addition, anti-Ro(SSA) antibodies were associated with anti-La(SSB) and RF, while anti-Sm antibodies were associated with anti-nRNP and RF. Flares occurred with a frequency of 0.07 per patient per year. Only 63.6% of flares were accompanied by positive anti-dsDNA reactivities. Reported deaths were 0.0047 per patient per year. Hierarchical log linear models indicated that the main variables of the disease were sufficient to describe our disease model and that the order of the interaction between the variables was insignificant. We conclude that the prevalence of various clinical features associated with SLE is similar, although the prevalence of CNS and pulmonary involvement as well as anti-Sm and anti-nRNP antibodies are less prominent in Greek SLE patients than that reported in the literature. The various clinicoserological associations detected do not appear to be of major significance as they are not powerful enough to subgroup the disease.
Lupus 1993 Oct
PMID:Systemic lupus erythematosus in Greece. Clinical features, evolution and outcome: a descriptive analysis of 292 patients. 830 23

A 25-year-old patient with lupus erythematosus was admitted with myositis and erythema of the skin under chloroquine therapy. After improvement of clinical symptoms with cyclophosphamide and prednisolone he was again progredient with myositis. The changing of therapy to methotrexate showed a hepatotoxic side effect with elevated liver enzymes. Under subsequent therapy with azathioprine and prednisolone he developed leukopenia and sepsis. Because of persistent erythema of the skin under therapy with different immunosuppressives we performed a therapy with high-dose intravenous immunoglobulins. After application of immunoglobulins we observed an improvement of the erythema after 10 days, which was persistent after dose reduction for about 4 months.
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PMID:[Immunoglobulin therapy for systemic lupus erythematosus]. 834 81

Patients with systemic lupus erythematosus (SLE) are susceptible to infections, notably salmonellosis. We report 37 cases of salmonella infection in 24 patients with SLE. These cases were detected in a group of 770 patients with SLE. All the patients were women, with a mean age of 25.6 years. At the onset of salmonella infection, 81% were taking prednisolone and 27% were taking cytotoxic drugs. Renal involvement was present in 75% of patients, which was approximately the same as in other SLE patients. Bacteremia, arthritis, osteomyelitis and rare manifestations of salmonellosis, including pulmonary and urinary tract involvement, were encountered. Diagnosis was based on isolation of the microorganism, mostly from blood cultures. Salmonella species other than typhi and paratyphi were often responsible. Widal agglutination test was positive in less than half the cases, and leukopenia was not seen frequently. Recurrence of infection in 29% of the patients and the high mortality rate (28.5%), despite the conventional period of appropriate antimicrobial treatment, show a poor prognosis of salmonellosis in SLE patients. This special picture of salmonellosis argues for a much longer period of treatment in these patients.
Lupus 1993 Feb
PMID:Salmonella infection in systemic lupus erythematosus. 848 61

An 18-year-old female with 7 years' history of epilepsy was admitted for developing malar rash. She had been treated with hydantoin for 7 years. Laboratory examinations revealed leukopenia and high titer of anti-dsDNA antibodies. Renal biopsy also showed diffuse segmental mesangial proliferative glomerulonephritis. A diagnosis of systemic lupus erythematosus (SLE) was made, and she received 40 mg of prednisolone daily. At follow up 4 months later since her first visit, she developed choreiform movements involving the right upper and lower limbs, despite no signs of increase in her disease activity. Neither biological false positive testing for syphilis nor the lupus anticoagulant (LAC) was detected. MRI demonstrated no signal abnormalities in the brain. Administration of haloperidol was started and the choreiform movements were decreased. Anticonvulsants are associated with drug-induced lupus. On the other hand, seizure is known to be one of the first manifestations of SLE. In drug-induced lupus, positive testing for anti-dsDNA, anti-Sm antibodies, hypocomplementemia and renal involvement are not a frequent as in SLE. In this case, laboratory findings showed high titer of anti-dsDNA antibodies, positive testing for antihistone, anti-SSA, anti-Ki antibodies, and hypocomplementemia. And mesangial proliferative glomerulonephritis was detected. So we diagnosed her as SLE and suggested that epileptic seizure developed 7 years ago had been the first manifestations of SLE. Neurologic complications of SLE are common, but chorea has been rarely reported. Since it is known that LAC is associated with thrombosis, it has been suggested that small infarctions in the basal ganglia may play a part in the pathogenesis of chorea in SLE. In this case, the LAC was negative and MRI showed no detectable abnormalities. As a result another mechanism may be attributed to chorea in this case.
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PMID:[A case of systemic lupus erythematosus diagnosed 7 years after epileptic seizure and developed chorea during prednisolone treatment]. 877 91

A 56-year-old Japanese woman had a 8-year history of systemic lupus erythematosus (SLE) with recurrent flares. When she was 48 years old, she was diagnosed as having SLE on the basis of fever, polyarthritis, oral ulcers, leukopenia, and positive anti-DNA antibody. Three years later she developed pericarditis and pleuritis, that were improved with treatment with 30 mg of prednisone a day. With tapering of prednisone dose to 9 mg a day in January 1993, she was admitted due to shortness of breath on exertion. Chest radiograph revealed bilateral elevated diaphragms and sluggish movement with clear lung field. Pulmonary function tests showed restrictive defect with a vital capacity 38% of predicted value. A diagnosis of "shrinking lung syndrome" was made. Simultaneously, blood test revealed leukopenia, elevated red cell sedimentation rate, and elevated anti-DNA antibody titer. Therefore, we suspected this pulmonary involvement was related to lupus flare-up. Treatment with 20 mg of prednisone a day resulted in resolution of the patient's dyspnea and in improvement of her vital capacity. Corticosteroid therapy for acute "shrinking lung syndrome" in active SLE can improve symptoms and pulmonary function.
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PMID:[A case of the "shrinking lung syndrome" in SLE--improvement with corticosteroid therapy]. 881 May 47

The patient is a newborn girl, born at term by cesarian section, weighing 3,550 g, and measuring 50 cm in length. According to her mother, she had been presenting erythematous spots involving the scalp, face, and trunk since birth that had increased in size with time. The mother denied any other changes and reported normal growth and development. Physical examination at 2 months of age revealed an infant in good general condition, hydrated, with no fever and no abnormalities detected on careful physical examination. The dermatologic examination revealed numerous circumscribed erythematous-brownish flat maculae with sharp borders and irregular contours, with no follicular hyperkeratosis, but with telangiectasias and areas of atrophy. They were lenticular and nummular, especially on the face (peri-orbital heliotropic lesions), and appeared as plaques on the trunk, with a tendency to confluence. The lesions also involved the scalp, neck, and extremities (Fig. 1). Histopathologic examination revealed an atrophic epidermis with focal areas of hydropic degeneration of the basal layer. A discrete perivascular and periannexal lymphohistiocytic infiltrate was observed in the superficial dermis, with melaninophages, frequent extravasation of red blood cells, angiectasia, and edema (Fig. 2). Serologic tests were reactive for antinuclear factor (ANF) (titer 1:100) of the speckled pattern, the presence of anti-Ro antibodies, and absence of anti-RNA and anti-Sm antibodies (Table 1). Blood counts and electrocardiogram were normal (Table 1). HLA typing showed positivity for DR-3 (Table 2). At 5 months of age the patient already showed a marked improvement of the skin lesions with only some areas of discrete pigmentation, a few atrophic areas, and rare telangiectasis (Fig. 3). The serologic tests (ANF, anti-Ro) had become nonreactive and the anti-RNA and anti-Sm tests continued to be negative. Examination of the mother revealed an asymptomatic 25-year-old woman reporting no manifestations suggestive of lupus. General and special physical examination revealed no abnormalities. Dermatologic examination showed no active or residual lesions of discoid or systemic lupus erythematosus. The pregnancy had been uneventful. Histopathologic examination of the girl's skin revealed the epidermis without obvious changes, minimal edema in the dermis, and a discrete perivascular inflammatory mononuclear cell infiltrate. Direct immunofluorescence of normal skin not exposed to the sun was negative. Blood counts revealed mild anemia and a tendency to leukopenia and thrombocytopenia (Table 1). Serologic tests showed reactive ANF (titers 1:400 and 1:800, speckled pattern), the presence of anti-Ro antibodies, and the absense of anti-RNA and anti-Sm antibodies (Table 1). HLA-typing revealed positivity for DR-3 (Table 2).
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PMID:Neonatal lupus erythematosus. 883 29

It has previously been shown that immunization with pathogenic anti-DNA idiotypes (Ids; e.g. 16/6 Id) leads to the induction of experimental system lupus erythematosus (SLF) in naive mice. The disease is characterized by serological (e.g. anti-double-strand DNA), clinical (elevation of erythrocyte sedimentation rate, leukopenia and proteinuria) and histological (immune complex deposition in kidneys) parameters. To determine whether the 16/6 Id carrying anti-DNA antibodies has unique pathogenic ability, in the current study we have employed diverse sources of anti-DNA antibodies to induce experimental SLE. An IgM anti-DNA antibody lacking the 16/6 Id was able to induce the production of the serological markers of experimental SLE, but not the clinico-histological findings. Furthermore, an IgA anti-DNA (16/6 Id derived from the serum of a patient with celiac disease was very effective in inducing the whole presentation of experimental SLE. Other anti-DNA antibodies failed to induce the autoimmune condition. Combined with our previous experience, the current study points to the diverse potential of various anti-DNA antibodies to induce SLE. The 16/6 Id is only one of a list of the potent pathogenic anti-DNA Ids. These facts may explain in part the diversity of clinical presentations of SLE, including asymptomatic subjects who carry high serum titers of anti-DNA antibodies.
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PMID:The diverse pathogenic potential of anti-DNA antibodies from various sources to induce experimental systemic lupus erythematosus. 885 93

Osteonecrosis (ON) is a well-known complication in patients with systemic lupus erythematosus (SLE) often associated with steroid therapy. In a cohort of 280 SLE patients followed over the last 10 years, seven patients developed symptomatic ON, one of them after septic arthritis of the hip. Two other patients developed ON several years after discontinuing steroids. One patient developed ON of both humeral and femoral heads within a few months after the diagnosis of SLE. When we compared the cumulative steroid doses taken by our patients with those described in other reports (43,700 mg and 45,300 mg, respectively), our patients received less steroids (38,834 mg). We found no increased frequency of Raynaud's phenomenon, leukopenia, anti-phospholipid antibodies, or a flare of SLE activity in our patients with ON, factors which have been reported to be associated with ON. Various pathogenic mechanisms, which could lead to ON in SLE patients are discussed.
Lupus 1996 Aug
PMID:Osteonecrosis in systemic lupus erythematosus, steroid-induced or a lupus-dependent manifestation? 886 6


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