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)

The appearance of vasospastic features in the central nervous system (CNS) after a cold stressor test was Investigated through single photon emission computed tomography (SPECT) of regional cerebral blood flow in patients with systemic lupus erythematosus, with and without Raynaud's syndrome, and in scleroderma patients. We have shown that Raynaud's syndrome may occur in the CNS and that anticardiolipin or lupus anticoagulant positivity may favour perfusion defects.
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PMID:Changes in regional cerebral blood flow after a cold hand test in systemic lupus erythematosus patients with Raynaud's syndrome. 1074 19

A 46-year-old female was admitted to our hospital due to general fatigue, systemic edema and dyspnea with history of systemic sclerosis (SSc). The patient was diagnosed as mixed connective tissue disease (MCTD) based on Raynaud phenomenon, a high anti-RNP antibody level and clinical symptoms and laboratory findings suggesting SSc, dermatomyositis (DM) and systemic lupus erythematosus (SLE). After the admission, both alveolar hemorrhage and a rapidly progressive glomerulonephritis (RPGN) also developed and laboratory findings showed a positive remark of myeloperoxydase-antineutrophil cytoplasmic antibody (MPO-ANCA) and anti-glomerular basement membrane (GBM) antibody. She was therefore re-diagnosed as microscopic polyarteritis nodosa (microscopic PAN) combined with MCTD and treatment with high dose prednisolone and steroid pulse therapy dramatically improved general conditions and lung symptoms, but maintenance dialysis was persistent because of irreversible renal failure. However, 3 months after the admission, she died of acute exacerbation of interstitial pneumonitis that was unresponsive to steroid pulse therapy. Autopsy revealed interstitial pneumonitis with alveolar hemorrhage and crescentic glomerulonephritis (CrGN), in which immunofluorescent microscopy showed no deposition in agreement with pauciimmune type. The histological findings supported the diagnosis; primary microscopic PAN combined with MCTD, which is a quite rare case, to our knowledge. Furthermore, co-existence of MPO ANCA and anti-GBM antibody, clinical and histological findings of the case also lead us to reconsider the relevance of these antibodies to pathogenesis and/or categories of microscopic PAN and Goodpasture's syndrome.
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PMID:[A case of mixed connective tissue disease with microscopic polyarteritis nodosa associated with perinuclear-antineutrophil cytoplasmic antibody and anti-glomerular basement membrane]. 1061 88

Clinical data of 34 patients with DM, who have been treated during the years 1971 and 1998 were evaluated. 79% of the patients (27 patients) were female, 21% of them (7 patients) were male. 59% of the patients (20 pts) were between the ages of 41 and 50 years. The characteristic heliotrop rash were observed in 26 patients, Gottron's papules in 20 patients, poikiloderma in 2 patients, calcification, ulcers, Raynaud syndrome in 1 patient. 3 of the 34 patients presented with strongly itching erythematopapulosus symptoms, most prominently on the scalp. Cardiac involvement were present in 10 patients (29%), lung involvement in 8 patients (23%), gastrointestinal complaints in 11 patients, dysphagia, dysphonia in 4 patients, joint pain in 5 patients. Overlap syndrome--scleroderma-dermatomyositis, SLE-dermatomyositis--was present in 2 patients. 9 of the 34 patients (26%) suffered from malignant tumours: gastric, breast, lung, epipharynx carcinoma, malignant melanoma. 13 of the 34 patients have been treated with corticosteroids together with immunosuppressor agents, in most cases azathioprin was administered. Cyclosporin was given in 7 cases, chloroquine in 2 cases.
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PMID:[Dermatomyositis: clinical study of 34 patients]. 1069 80

We describe a 26-year-old white female with a history of Raynaud phenomenon, erythema nodosum, polyarthralgias, migraine, vertigo, seizures, transient ischemic attacks, one fetal loss, and false positive VDRL, who developed milk hypertension without overt lupus nephritis. She had positive antinuclear antibodies (ANA) and double-stranded deoxyribonucleic acid (dsDNA) antibodies. The lupus anticoagulant test (LAC) and cardiolipins antibodies (aCL) were positive. She was diagnosed as having a Systemic Lupus Erythematosus-like illness (SLE-like) with 'secondary' antiphospholipid syndrome (APS). Renal spiral computed tomography (CT) with intravenous (IV) contrast showed bilateral renal artery stenosis. Anticoagulation with acenocumarol was started. She became normotensive without antihypertensive drugs five months later. A follow-up renal spiral CT showed complete recanalization of both renal arteries, making thrombosis the more likely culprit pathology in the stenosis. After two years follow up the patient is normotensive. She remains on acenocumarol.
Lupus 2000
PMID:A reversible bilateral renal artery stenosis in association with antiphospholipid syndrome. 1071 49

We studied the prevalence and the effect of coagulation factor V Leiden mutation on the occurrence of thrombotic episodes in 120 Hungarian patients having systemic lupus erythematosus (SLE) with or without antiphospholipid antibody. The frequency of the factor V Leiden mutation in Hungarian SLE patients was 13%, which is comparable with those found previously in a healthy Caucasian population. The incidence of venous thrombosis among factor V Leiden carriers has been found to be higher (odds ratio [OR] 1.7) than it is in patients without Leiden mutation (38% vs 29%). In addition, the frequency of venous thrombosis in the heterozygous SLE patients (OR 8.4 [confidence interval (CI) 0.8-83.9] P = 0.06) is dependent on the coexistence of other risk factors, such as antiphospholipid antibody. Moreover, among heterozygous factor V SLE patients, the Leiden mutation could explain the tendency to have significantly higher prevalence of fetal losses (OR 3.9 [CI 1.2-12.0] P = 0.02) and higher prevalence of cerebrovascular lesions, cardiac valvular abnormalities, and Raynaud's syndrome than that found in individuals without factor V Leiden mutation of those having antiphospholipid antibody. Systemic lupus erythematosus patients with combined defects suffer more severely from thrombosis than those with a single risk factor do, suggesting that thrombophilia is a multifactorial disorder in SLE, also. Although, the factor V Leiden mutation does not seem to be a significant risk factor for venous thrombosis in SLE, these data demonstrate that Leiden mutation can be regarded as an additive thrombogenic factor providing higher predisposition to several vasoocclusive disorders in SLE.
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PMID:The Leiden mutation of coagulation factor V in Hungarian SLE patients. 1072 48

Using a highly sensitive Radioimmunoassay (RIA), the kinetics of synthesis of anti-fibrillin (Fbn-1) autoantibodies were studied in 17 patients with mixed connective tissue disease (MCTD) and two with CREST syndrome calcinosis, Raynaud's oesophageal dismotility, sclerodectyly and teleangiectasis who were found to be positive for this autoimmune response. IgG autoantibodies specific for recombinant Fbn-1 (rFbn-1) (aa 369-425) were found in all patients excepting one with MCTD, multiple sclerosis, and dermatomyositis. IgM were found in fewer cases. Several kinetics patterns of anti-Fbn-1 autoantibodies were observed: a) long lasting persistence of IgG and IgM autoantibodies up to 14 years; b) fluctuation of antibodies during various periods up to 16 years; c) disappearance of antibody response after several years, and d) patients producing IgG but not IgM autoantibodies. No differences in the synthesis of autoantibodies were observed between MCTD patients with a stable disease, and those developing during the course features of systemic sclerosis (SSc), Sjogren's syndrome, or rheumatoid-like arthritis. In one patient displaying a lupus-like syndrome for 3 years, the appearance of anti-Fbn-1 autoantibodies coincided with the occurrence of MCTD and scleroderma. While the detection of anti-Fbn-1 autoantibodies may be clinically useful in differential diagnosis or eventual prognosis of patients with connective tissue diseases, their role in the pathogenesis of scleroderma syndromes requires further investigation.
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PMID:Kinetics of anti-fibrillin-1 autoantibodies in MCTD and CREST syndrome. 1075 89

Malignant neoplasms are associated with a wide variety of paraneoplastic rheumatological syndromes. Among these, hypertrophic osteoarthropathy, carcinoma polyarthritis, dermatomyositis/polymyositis, and paraneoplastic vasculitis are the most frequently recognized. Other less known associations are based upon a smaller number of reported patients, and include fasciitis, panniculitis, erythema nodosum, Raynaud's syndrome, digital gangrene, erythromelalgia and lupus-like syndromes. Musculoskeletal manifestations of malignancy may coincide, follow or antedate the diagnosis of cancer, or herald its recurrence. The clinical course generally parallels that of the primary tumour, and treatment of the underlying malignancy often results in regression of the rheumatic disorder. Awareness that cancer can cause certain non-metastatic symptoms is important for early diagnosis and treatment of an occult neoplasm. Rheumatic manifestations suggesting a hidden cancer include: rapid onset of an unusual inflammatory arthritis clubbing or diffuse bone pains in a patient 50 years of age or older, chronic unexplained vasculitis, refractory fasciitis, Raynaud's syndrome unresponsive to vasodilator therapy, rapidly progressive digital gangrene or Lambert-Eaton myasthenic syndrome. Management consists of control of the underlying cancer and symptomatic treatment of the rheumatic syndrome with non-steroidal anti-inflammatory drugs or corticosteroids.
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PMID:Paraneoplastic rheumatic syndromes. 1098 84

Difficulties to establish general characteristics of patients with Raynaud's phenomenon, especially frequency, rates and predisposing factors of the evolution of primary to secondary cases probably originate from substantial variation of evaluated cohorts. We conducted a prospective study using standardised diagnostic procedures in order to look for the specificity of patients referred to the vascular centre; moreover, we assayed anticardiolipin antibodies in these patients using double ELISA and compared its frequency to sex and age matched a control group of 50 healthy individuals. 124 patients (20 men), mean age at onset 35.5 yr, range 9-69 yr, had confirmed diagnosis of Raynaud's phenomenon. Ninety nine patients were found to have secondary phenomenon, 72% of them had trophic changes of fingers and/or toes. Anticardiolipin antibodies assay was positive in seven patients and four healthy donors. Vascular diseases constituted about 20%, and connective tissue diseases 50% of secondary cases, but SLE (17 cases) not a scleroderma (11 cases) was the most frequent clinical entity in the latter group. There were only two patients with Buerger's disease and one with atherosclerosis as an underlying disease for vasospastic disorder. We concluded in the vascular medicine centre that there were a lot of patients with ischemic necrosis or other type of trophic changes, and very little primary, benign Raynaud's disease cases; surprisingly, peripheral arterial occlusive disease was very seldom responsible for vasospastic episodes. Primary or secondary antiphospholipid syndrome is not associated with Raynaud's phenomenon.
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PMID:Clinical spectrum of Raynaud's phenomenon in patients referred to vascular clinic. 1099

Systemic lupus erythematodes is a disease commonly associated with peripheric circulation disturbances. The goal of the study was the evaluation of the role of the following nuclear antibodies: anti-Ro (SS-A), anti-La (SS-B) and anti-Sm in the endothelium damage process in the SLE patients with symptoms of microcirculation disturbances. The concentration of plasma thrombomodulin was used as a marker of the endothelium destructive changes intensity. Twenty-four patients with SLE (22 women and 2 man) aged of 18-57 (the average age 40.50 +/- 9.72 years) in which occurred: the Raynauds symptom (16 patients), fingers cyanosis (5 patients) and fingers and/or toes necrosis (3 patients) were investigated. Antinuclear antibodies and thrombomodulin level was estimated with the ELISA method. In all patients, the concentration of anticardiolipin antibodies in serum was determined (ELISA-method, cardiolipin was used as an antigenes, Sigma USA). Higher titers of ANA antibodies in 83%, anti-SM antibodies in 30%, anti-Ro in 42% and anti-La in 42%, examined patients and higher titer of anti-Ro and anti-La antibodies together in 29% of examined patients were stated. Statistical significant correlation between the increased level of thrombomodulin and anti-La antibodies concentrations in serum of patients with the disease lasting over 6 years was found out. The significant statistical correlation of thrombomodulin concentration increasing and anti-La antibodies in serum was proved. The dynamism of limbs circulation disorders depends on the disease duration.
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PMID:[Endothelial response for the presence of chosen antinuclear antibodies, anti-Ro (SS-A) and anti-La (SS-B) and anti-Sm in vasculitis against the background of existing lupus erythematosus]. 1107 29

Previously a strong positive correlation was found between antibodies to C1q (C1qAb) and antibodies against human heat shock protein (hsp60) and mycobacterial hsp65 in HIV infected patients. Here the levels of these antibodies were measured in the sera of patients with different autoimmune diseases (122 systemic lupus erythematosus (SLE), 55 systemic sclerosis, 33 undifferentiated connective tissue disease (UCTD), 27 primary Raynaud syndrome, 21 rheumatoid arthritis (RA), 14 polymyositis/dermatomyositis (PM/DM), and 192 healthy blood donors. The prevalence of IgG C1qAb was found to be high (P<0.0001 as compared to the healthy controls) only in the SLE group. The levels of the anti-hsp60 (P=0.0094) and anti-hsp65 (P=0.0108) antibodies were high only in the UCTD patients. No correlation was found between the C1qAb and anti-hsp antibodies in any group except a significant (P=0.011) positive correlation between C1qAb and hsp65 antibodies in the patients with UCTD. These findings indicate that the autoantibodies against C1q are heterogeneous: in different diseases different types of C1qAb may dominate.
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PMID:Levels of antibodies against C1q and 60 kDa family of heat shock proteins in the sera of patients with various autoimmune diseases. 1113 33


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