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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The improvement of the quality control in the detection of fluorescent antinuclear antibodies (FANA) have decreased the differences among institutions. However, the positive lower or upper reference limit and the criteria for the determination vary among laboratories. By examining 725 sera from children and 227 sera from healthy adults, we proposed the titers of 160-320 for children and 80-160 for adults as the positive lower limits. The higher positive incidence in the children's sera might be due to some abnormality in the ANA production or the higher sensitivity of the reagents used. The clinical significances of the anti-U1RNP antibody and anticentromere antibody (ACA) in ANA have been described. The anti-U1RNP antibody, present in only MCTD and related disorders, might play some role in the development of Raynaud's phenomenon and symptoms in a case of neonatal lupus erythematosus. ACA was detected in sera not only of CREST syndrome patients but also of PBC patients. The functions and molecular structures of nuclear antigens have recently been extensively investigated. However, the pathogenetic significance of most of the ANA and the mechanism of their production have not been clarified yet.
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PMID:[Detection of antinuclear antibodies and their significance as disease markers]. 802 74

Six pediatric patients with Raynaud's phenomenon are reported. Three patients had clinical and immunological evidence for a connective tissue disease; one had primary Raynaud's phenomenon associated with antinuclear antibodies and two had Raynaud's phenomenon secondary to exposure to lead and to a vibrating instrument, respectively. The diagnostic and therapeutic implications of these observations will be discussed.
Lupus 1993 Jun
PMID:Clinical diversity of Raynaud's phenomenon in childhood: report of six cases. 836 9

Raynaud's syndrome manifests as a progressive color change of the fingers in response to cold, vibration or stress; the digits first turn white, then blue and finally red. The condition is called Raynaud's disease when it is a benign, primary condition. When it is secondary to another disease, such as lupus, scleroderma or atherosclerosis, it is termed Raynaud's phenomenon. Laboratory tests, i.e., complete blood count, chemistry screen, antinuclear antibody, lupus erythematous test and rheumatoid factor, should be used to seek underlying diseases before the symptoms are manifest. Other tests should be selected as indicated by the history and physical. There are many adjustments in lifestyle and working conditions that the patient can use to minimize the symptoms of Raynaud's syndrome. The primary care provider has an important role in teaching patients to protect their hands from the effects of cold, stress, nicotine and vibration. Adaptive devices and protective clothing minimize the symptoms of Raynaud's syndrome.
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PMID:The diagnostic puzzle and management challenge of Raynaud's syndrome. 845 39

The American Rheumatism Association (ACR) preliminary and revised criteria for classification of systemic lupus erythematosus (SLE) were evaluated for sensitivity and specificity in a population of 100 patients with SLE and 100 patients with other rheumatic diseases. Bayes' theorem was applied for evaluation of ARA criteria for the classification of SLE and a scoring system was developed which allows simple determination of the probability of SLE. The evaluation revealed considerable differences in values of the ARA criteria. The serologic tests and discoid lupus appeared to be the most distinctive criteria, while Raynaud's phenomenon, oral and nasal ulcers and arthritis were of little value. Comparison of SLE and control patients presenting the same number of criteria revealed that patients with SLE exhibit more distinctive criteria. This finding emphasizes the need for a quantitative evaluation of classification criteria.
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PMID:Evaluation of criteria contributions for the classification of systemic lupus erythematosus. 848 Jan 39

We have evaluated the autoantibody profiles in the sera of 117 patients with systemic lupus erythematosus (SLE) and compared and contrasted the clinical and laboratory features of the disease of patients segregated according to an autoantibody profile. Using this approach we are able to demonstrate that autoantibody profiles identified subsets of patients with SLE. Patients with a negative autoantibody profile had fewer clinical and laboratory features of their disease when compared to the other subsets of patients. In contrast, patients with profile A (anti-nDNA and/or anti-Sm antibodies) had a statistically significant increase in malar rash, renal and hematologic involvement and hypocomplementemia when compared to patients with a negative profile. Patients with profile B (anti-nRNP antibodies) had a clinical pattern of disease different from that of patients with profile A and had a statistically significant increase in Raynaud's phenomenon when compared to patients with a negative profile. Patients with profile C (anti-SSA and/or anti-SSB antibodies) had a statistically significant increase in lupus-related rashes and photosensitivity. None of the lupus patients reviewed in this study has profile D (antibodies to centromere and/or Scl-70), this profile being seen largely in patients with scleroderma or one of its variants. Both patients with profile E (anti-histone antibodies) had drug-induced lupus. We conclude that the use of autoantibody profiles defines subsets of patients with lupus that may have clinical, therapeutic and prognostic implications.
Lupus 1993 Feb
PMID:The clinical significance of autoantibody profiles in patients with systemic lupus erythematosus. 848 55

A 15-year-old girl had severe Raynaud's phenomenon and arthralgias. A high ANA-IF titer was found and undifferentiated connective tissue disease was diagnosed. After 7 years of moderately flaring disease the patient deteriorated and presented with congestive heart failure, pleuropericardial effusion, hemolytic uremic syndrome, proteinuria and moderate hypertension. Autoantibodies against DNA, Sm-protein, and very high titers against U1RNP were detected. Therapy with high steroid doses, a cyclophosphamide pulse and 4 weeks of plasmaphresis with plasma exchange improved the heart, but not the renal condition. Symptomatic pancreatitis became the dominant problem of a progressively consuming process that resulted in the death of the patient. Post-mortem examination revealed widespread vasculopathy with intima proliferation and only minimal fibrosis involving the kidneys, heart and other main organs, including the pancreas. Taken together, the clinical picture was of an overlap between scleroderma and systemic lupus erythemathosus; the serologic and histopathologic findings suggest a diagnosis of a severe form of mixed connective tissue disease (MCTD).
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PMID:Widespread vasculopathy with hemolytic uremic syndrome, perimyocarditis and cystic pancreatitis in a young woman with mixed connective tissue disease. Case report and review of the literature. 851 21

The clinical activity of systemic lupus erythematosus (SLE) may be influenced by ethnic and environmental factors. The Consensus group on Activity Criteria for SLE attempted in a multicenter study to determine well-accepted criteria for disease activity. Of the 704 randomly assigned patients, 41 (5.8%) were from Israel. A detailed history including epidemiological, clinical and laboratory data was recorded. Significant differences were found between Israeli and European patients in the occurrence of Raynaud's phenomenon [12 (30%) vs. 358 (51%), respectively (P < 0.05)] and skin vasculitis [6 (15%) vs. 216 (34%), respectively (P < 0.05)]. In addition to those clinical differences, significant differences were also found in the occurrence of VDRL, low complement levels and lupus anticoagulant. Additional differences were found in some laboratory data, indicating differences in the sensitivity of the various laboratories. We conclude that the differences between the Israeli and European groups in the clinical data can be attributed mainly to environmental factors (weather, viruses), and in the laboratory data to ethnic differences (e.g., HLA) and to the different diagnostic methods used.
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PMID:Comparison of clinical and laboratory parameters for systemic lupus erythematosus activity in Israelis versus Europeans. 863 44

Fibrinolysis triggered by t-PA bound to fibrin is one of the main antithrombotic mechanisms. Defects in the fibrinolytic system-decreased tissue-type plasminogen activator (t-PA) activity and elevated levels of plasminogen activator inhibitor (PAI-1), in patients with SLE have been associated with an increased tendency to thrombosis. In the present study, 43 patients with SLE fulfilling the ACR criteria for the disease, were studied for the presence of autoantibodies to fibrin-bound t-PA, i.e. the physiological active form of this plasminogen activator. A solution of 200 IU/ml of t-PA was incubated with solid-phase fibrin prepared as previously described (Anal Biochem 1986; 153; 201-210). Sera diluted 1:50 were incubated with fibrin-bound t-PA, the plates were then washed, and bound immunoglobulins were detected using a polyvalent peroxidase-labeled goat anti-human Ig. Plates coated with fibrin alone were used as controls. Sera were considered positive when A490/630 obtained with normal human sera in two independent test was greater than the mean plus 2 SD. Eleven of 43 (26%) SLE sera demonstrated antibody reactivity against fibrin-bound t-PA. Within the anti-t-PA positive group there was a higher proportion of SLE patients with severe Raynaud's phenomenon and thrombotic events when compared to the anti-t-PA negative group: 36% vs 6% and 18% vs 6% respectively. These results suggest that autoantibodies to fibrin-bound t-PA could play a role in the pathogenesis of vascular disease in some SLE patients.
Lupus 1996 Aug
PMID:Antibodies to fibrin-bound tissue-type plasminogen activator in systemic lupus erythematosus are associated with Raynaud's phenomenon and thrombosis. 886 98

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

We have assessed the cutaneous signs in 73 patients with systemic lupus erythematosus (SLE), seen during a 5-year period in an English hospital. Most previous information about the cutaneous manifestations of SLE has been obtained from studies performed in the U.S.A. We classified lesions as specific cutaneous and mucosal LE (acute, subacute and chronic) or non-specific LE-related, e.g. photosensitivity, urticaria, erythema, Raynaud's phenomenon or vasculitis. Acute cutaneous LE lesions included a butterfly rash with erythematous macules, telangiectasia or papulosquamous lesions, seen in 37 patients (51%) and facial oedema seen in four patients (5%). Five patients (7%) had psoriasiform subacute cutaneous LE. Chronic cutaneous LE was common: 18 patients (25%) had chronic discoid lesions (DLE) and, in 12 (15%), these had preceded systemic disease. One patient had facial lupus profundus. Ten patients (14%) had scarring alopecia secondary to DLE. Fifteen patients (20.5%) had chronic chilblain lupus. Twenty-three patients (31.5%) had a history of mouth ulceration. Of these, 11 (15%) gave a history of ulcers at the onset of their disease. Three (4%) had erythema and superficial ulceration of the palate, not typical of aphthous ulcers, and three (4%) had chronic buccal plaques. Cheilitis due to DLE was seen in three (4%), episcleritis in three (4%), five (7%) had nasal disease, six (8%) bullous skin eruptions, one 'the bullous eruption of SLE', four bullae associated with cutaneous vasculitis, and one bullae associated with ultraviolet radiation. Forty-six (63%) observed photosensitivity. A non-scarring alopecia occurred in 29 (40%). Vascular phenomena were common: three patients (4%) had chronic palmar erythema, Raynaud's phenomenon occurred in 44 patients (60%), chronic urticaria, worsened by sun exposure, was noted by 32 (44%) (in whom the lesions often lasted more than 36 h), eight (11%) had cutaneous vasculitis and three (4%) livedo reticularis. Skin changes play a prominent part in SLE and may provide helpful diagnostic information. In this British population, chilblains and urticaria were particularly common. Lesions of subacute cutaneous LE were relatively unusual in this group of patients with SLE.
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PMID:Cutaneous manifestations of systemic lupus erythematosus. 894 25


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