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)

Twenty-three patients with systemic lupus erythematosus (SLE) and ischemic bone necrosis are reported. All patients had received corticosteroids prior to the onset of ischemic necrosis, although one patient had received none for 13 years previously. Nineteen (83%) patients had multiple bone lesions including the femoral heads in 21 (91%) which were bilaterally involved in 15. In addition, humeral heads were affected in seven patients and the tibial plateaus, in three. The most striking feature of this group was the high incidence of Raynaud's phenomemon present in 14 (61%) of the 23 patients. Furthermore, central nervous system involvement was present in 10 (43%) patients, more prominent in those without Raynaud's (67%) than in those with vasospasm (29%). Thus, 20 of the 23 patients, or 87%, evidenced vascular abnormalities either in the form of Raynaud's phenomenon and/or systemic vasculitis. The pathogenesis of ischemic bone necrosis is discussed. In SLE, vasospasm or vasculitis, or both, augmented by corticosteroid therapy, could impede the microcirculation and result in the ischemic lesion.
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PMID:Ischemic necrosis of bone in systemic lupus erythematosus. 127 70

Two sisters had autoimmune responses to the U1RNP particle that were quantitatively similar and/or identical in molecular characteristics. No other autoantibodies were demonstrable. Both sisters immunoprecipitated only U1RNA, had a reaction of identity in gel diffusion, bound the 68-kDa band in HeLa cell extract in Western blot, and reacted almost equally to a rabbit anti-idiotypic reagent made against either sister's isolated anti-U1RNP Fab fragments. They both carried a DR4 allele, which has been associated with anti-U1RNP production in several studies. While the sisters both had Raynaud's phenomenon, their clinical pictures were otherwise dissimilar. One had a seizure disorder (Ju); the other had polymyositis and features of scleroderma (Je). In sister Ju, Raynaud's phenomenon was manifest for the first time in association with the appearance of precipitating anti-U1RNP.
Lupus 1992 Aug
PMID:Two sisters producing anti-U1RNP exhibit serological concordance and clinical discordance. 130 88

The effect of total-body cold exposure on plasma concentrations of von Willebrand factor (vWF), endothelin-1 (ET) and thrombomodulin (TM), all of which are considered to be generated from the endothelium, was studied in systemic lupus erythematosus (SLE) patients with and without Raynaud's phenomenon. The plasma levels of vWF, ET and TM in SLE patients, irrespective of the presence of Raynaud's phenomenon, were significantly higher than in normal controls even before the cold provocation test. After the cold provocation test, plasma levels of vWF and ET were significantly higher in SLE patients with Raynaud's phenomenon than in those without and in normal controls. No significant increase in TM was observed in either the SLE patients or the controls. These results suggest that SLE patients, regardless of the presence of Raynaud's phenomenon, are in a hypercoagulable state and that this state may be further intensified by cold exposure. Hence, it is concluded that we should consider antithrombotic therapy for SLE patients, especially those with Raynaud's phenomenon, to prevent unwanted activation of the coagulation system and possible endothelial damage.
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PMID:Effect of total-body cold exposure on plasma concentrations of von Willebrand factor, endothelin-1 and thrombomodulin in systemic lupus erythematosus patients with or without Raynaud's phenomenon. 133 60

Anti-pyruvate dehydrogenase (PDH) antibodies were determined in 1451 sera of patients with primary biliary cirrhosis (PBC) and several autoimmune rheumatic conditions by ELISA and immunoblotting. They were detected in sera of 93% of the patients with PBC (179 of 192 patients) in 60 of 277 (22%) patients with Sjogren's syndrome (SjS), 34 of 437 (8%) patients with scleroderma, 33 of 191 patients with SLE (17%), and 5 of 55 (10%) patients with rheumatoid arthritis (RA) but in none of the patients with polymyositis or the antiphospholipid syndrome. The ELISA studies were confirmed by immunoblots showing binding of autoimmune rheumatic sera to the same epitope (74 kd) of mitochondria that the PBC sera reacted with. The identical binding characteristics were also confirmed by protein competition assays with purified PDH. In 4 of 53 patients with SjS who were positive for anti-PDH, high titers as in PBC were detected. The anti-PDH antibodies in Sjogren's patients were associated with deranged liver function tests and extraglandular features but did not correlate with any other non-organ-specific antibody. Follow-up studies confirmed the association of the emergence of anti-PDH antibodies with defects in liver function tests. The antibodies were more prevalent in SLE and RA when they were associated with Sjogren's syndrome (30 and 18.8%, respectively). Among patients with different forms of scleroderma, anti-PDH antibodies were noted in subjects with systemic sclerosis, morphea, and Raynaud's phenomenon.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Antimitochondrial (pyruvate dehydrogenase) autoantibodies in autoimmune rheumatic diseases. 140 Sep 1

Cardiovascular autonomic function was assessed in 9 subjects with Raynaud's phenomenon. The underlying diseases were systemic lupus erythematosus (n = 5), systemic sclerosis (n = 3) and rheumatoid arthritis (n = 1). Five standard non-invasive tests, 3 of heart rate and 2 of blood pressure, were employed. Compared with age and sex matched controls (n = 25), the number of values abnormal was 24 of 45 (53%) overall and between one and 4 (median, 2) individually. Significant differences were present for 3 tests, two of heart rate and one of blood pressure. The subjects were given triiodothyronine, 60 to 80 micrograms per day, for vasospastic attacks. Autonomic function was reassessed between weeks 4 and 9 (9 subjects) and between weeks 12 and 18 (8 subjects) after introduction of triiodothyronine. Test results showed a considerable improvement. At the second reassessment, the number of values abnormal was now 5 of 40 (12.5%) overall and nil (n = 4) or one (n = 4) individually. Significant differences remained for one heart rate test only. Adverse side effects to triiodothyronine occurred in a single subject and were readily controlled. Evidence of somatic neuropathy was present electrophysiologically in all 9 subjects and clinically in 8. Triiodothyronine may have corrected autonomic dysfunction by increasing blood flow to ischaemic peripheral nerves or by acting on the autonomic system more directly. Further study of triiodothyronine in autonomic insufficiency appears merited.
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PMID:Treatment of Raynaud's phenomenon with triiodothyronine corrects co-existent autonomic dysfunction: preliminary findings. 140 89

In the past decades the general concept of the disease course and the prognosis of systemic lupus erythematosus (SLE) has changed dramatically. The improvement in prognosis of our SLE patients is often said to be related to the growing awareness of the disease. This study focussed on whether or not the clinical features at the onset of the disease, and at diagnosis, and the clinical course as well as the age at the onset of the disease had changed during the past decades. No obvious differences were observed in the age at the onset of the disease or in the age at diagnosis. Of the 22 clinical signs studied, only the prevalence of Raynaud's phenomenon at the onset of the disease had increased during the past 20 years. At diagnosis, the prevalence of oral ulcers and false positive syphilis test had decreased. Only small differences in the type but not in the number of exacerbations were observed; in the past 20 years, the prevalence of renal involvement increased from 20% to 43%. However, this was not significant. Our results did not support the theory that during the past 2 decades the disease had changed in its expression, neither did we find that the disease is presently diagnosed at an earlier age, as would be expected from the increased awareness of the disease.
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PMID:Changes in clinical features of patients with systemic lupus erythematosus followed prospectively over 2 decades. 141 Oct 85

It has been reported that immunity to the 65 kDa heat shock protein of Mycobacterium tuberculosis (MT-hsp65) not only accompanies rheumatoid arthritis (RA), but may also be characteristic of chronic inflammation. We now report serum antibodies to MT-hsp65 in 47% of systemic sclerosis (SSc), 38% of primary Raynaud's phenomenon (PRP) and 5% of systemic lupus erythematosus (SLE). Antibody levels were higher in patients with active or progressive SSc and correlated with the degree of skin fibrosis. Thus, immunity to MT-hsp65 appears in SSc and is not limited to RA. However, it does show some degree of specificity beyond chronic inflammation: PRP patients have a higher reactivity than do SLE patients.
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PMID:Antibodies to mycobacterial 65 kDa heat shock protein in systemic sclerosis (scleroderma). 141 88

A patient with systemic lupus erythematosus who presented with subcutaneous nodules over the flexor aspect of the fingers in association with arthritis and Raynaud's phenomenon is described. Histopathological examination of the nodules showed appearances consistent with rheumatoid nodules. Further investigations revealed leucopenia and circulating anti-nuclear antibody but negative rheumatoid factor. Immunofluorescence studies of normal non-light exposed skin showed the presence of IgM deposits at the dermo-epidermal junction consistent with systemic lupus erythematosus. The nodules almost disappeared following treatment with hydroxychloroquine. Rheumatoid-like nodules have been reported in systemic lupus erythematosus, although rarely. However, the distribution of the nodules and the patient's clinical course differ from the few cases previously reported in the literature.
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PMID:Systemic lupus erythematosus presenting with 'rheumatoid nodules'. 142 63

In this report the association of autoimmunity and autoimmune syndromes with lymphoproliferative disorders (LPD) is described in 15 patients. Non-Hodgkin's lymphoma (NHL) developed in 10 patients, Hodgkin's disease (HD) in 3 and chronic lymphocytic leukemia (CLL) in two. In most instances clinical and laboratory phenomena preceded the development/diagnosis of these disorders. Manifestations ranged from the presence of autoantibodies in the serum to the presence of both ill defined or incomplete autoimmune syndromes including cold urticaria, Raynaud's phenomenon, cold agglutinin disease, thyroiditis, nephrotic syndrome and vasculitis to typical systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and even one of scleroderma. It is suggested that in some patients (in)complete clinical manifestations of autoimmunity may precede the development of lymphoid neoplasias. The link between autoimmunity and lymphoproliferative disorders is briefly discussed.
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PMID:Autoimmunity and auto-immune syndromes associated with and preceding the development of lymphoproliferative disorders. 143 18

A 38 year old woman with systemic lupus erythematosus (SLE) was admitted because of epigastralgia and fever. The diagnosis of SLE was made 22 years ago based on Raynaud's phenomenon, butterfly rash, hair loss, photosensitivity and positive antinuclear antibody. She had episodes of consciousness disturbance, transient visual disturbance of the left eye, and a necrosis of the left big toe. She underwent artificial arthroplasty of bilateral femoral heads 11 years ago, when multiple aseptic necroses of thirteen bones were found, and when anti-cardiolipin (CL) antibody was found to be positive. An echogram of abdomen suggested an obstruction of superior mesenteric artery (SMA) when she was admitted. Selective angiography revealed a complete obstruction of SMA and splenic artery, and incomplete obstruction of celiac artery. Conservative treatment with urokinase infusion and prednisolone 50 mg/day was not effective, and small intestine and right colon were resected on the 23rd hospital day. The pathological examination showed thrombosis of SMA. There was no evidence of arteritis or atherosclerosis. Anti-CL antibody and lupus anticoagulant were positive on admission, but the level of both anti-DNA antibody and complement was normal. Therefore, it was suggested that the thrombosis was related with anti-phospholipid antibody. The characteristic clinical feature were multiple aseptic bone necroses and thromboses of several arteries. We discussed the relationship of thrombosis and the etiology of multiple bone necrosis in this case with anti-phospholipid antibody.
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PMID:[A systemic lupus erythematosus patient with multiple aseptic bone necroses, thrombosis of superior mesenteric artery and anti-phospholipid antibody]. 144 87


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