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)

Of a total of 800 patients with systemic lupus erythematosus, 37 were found to have developed avascular necrosis of bone (4.6%). Routine radiological screening of all joints was not performed and these 37 represented symptomatic patients only. Twenty-seven of these were positive for antiphospholipid antibodies (aPL) (73%). Both aPL positive and aPL negative patients had received large doses of oral steroids. The frequency of vasculitis and livedo reticularis, as well as arterial thrombosis was higher in the aPL positive group, but this did not reach statistical significance, perhaps because of the small numbers of patients involved. Although the difference in the frequency of thrombocytopenia was more marked in the aPL positive group (p < 0.08), once again, this was not statistically significant at the 5% level. Since the prevalence of aPL positivity in the general lupus population is about 30-40%, the presence of the aPL may be associated with an increased tendency to develop avascular necrosis. This is supported by recent reports of avascular necrosis occurring in patients with primary antiphospholipid syndrome in the absence of previous steroid administration.
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PMID:Avascular necrosis of bone and antiphospholipid antibodies in systemic lupus erythematosus. 847 66

In an earlier study of a cohort of 667 patients with systemic lupus erythematosus (SLE) followed for 7.5 months with mean antiphospholipid (aPL) testing 2.5 times, we found a 10% prevalence of definite antiphospholipid syndrome (APS) (2 clinical manifestations and high aPL levels). To determine if more followup and aPL testing increased such prevalence we restudied the cohort after a mean followup of 3.1 years and a mean aPL testing of 5.6 times and found a 15% prevalence of definite APS. Another 21% of patients with SLE had probable APS with either high titers of aPL but only one clinical manifestation or low titers with 2 clinical manifestations. The prevalence of high titer positivity of aPL (IgG and/or IgM isotype) reached 41%. One aPL related feature that increased significantly was livedo reticularis possibly from increased awareness. Factors that influenced significantly the mobility upwards in APS category were more pregnancies and further aPL testing. Conversely, immunosuppressive treatment decreased higher aPL levels. The large number of patients with long disease duration in our cohort showed that the maximum prevalence of definite APS is reached after 15-18 years and is 23%. This might be the highest prevalence of definite APS within SLE.
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PMID:Influence of disease duration, continued followup and further antiphospholipid testing on the frequency and classification category of antiphospholipid syndrome in a cohort of patients with systemic lupus erythematosus. 796 94

This report describes a case of livedo reticularis associated with increased titres of anticardiolipin antibodies (aCL) in a patient with systemic lupus erythematosus. A 38-year-old woman presented with fever, malaise, arthritis and livedo reticularis in a severe form. Antibodies to native DNA and an increased level of aCL were found. A significant positive correlation exists between livedo reticularis and elevated serum antiphospholipid activity in patients with systemic lupus erythematosus. aCL are shown to play a possible pathogenetic role in thrombotic events. This suggests that thrombosis is the underlying cause of livedo in these patients. A biopsy performed in a patient at the site where livedo was most marked showed no evidence of thrombi. It is postulated that the mechanism of livedo in lupus patients with aCL consists of both thrombosis and dysfunction in the regulation of the tone of the peripheral vascular bed.
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PMID:Livedo reticularis in a patient with systemic lupus erythematosus and anticardiolipin antibodies. 848 95

Although several clinical and morphological changes observed in overt systemic lupus erythematosus have been associated with the presence of antiphospholipid antibodies (aPL), the relation between these antibodies and lupus nephropathy (LN) is not clear. Twenty-three patients with biopsyproved LN were retrospectively studied (average age 28.5 +/- 12.3 years, all women) in order to investigate the relationship between the presence of aPL and clinical and immunobiological data. The average follow-up period was 55 +/- 42 months. The presence of aPL (IgG and IgM) was detected at least once in all patients by ELISA and/or lupus anticoagulant (kaolin time). Seven patients (30.4%) were aPL+, and the remainder aPL-. We did not find differences related to age, period of follow-up, blood pressure and livedo reticularis. However the prevalence of thrombosis, strokes and hemolysis was slightly higher in the aPL+ patients. The levels of antinuclear antibodies or anti-DNA antibodies, immunoglobulins and complement serum levels (C3, C4) were also similar in both groups. In the aPL+ group, proteinuria was significantly higher than in aPL- cases (2.21 +/- 1.5 and 0.91 +/- 1.07 g/24 h, respectively; p = 0.029). The renal histological pattern in both series was similar. However, microthrombosis in the glomerular capillary lumens was more frequent in the aPL+ group. The evolution of renal function was less favorable in aPL+ patients when compared with aPL- patients. We conclude that the presence of aPL in patients with LN is associated with several characteristics of renal impairment which may contribute to its evolution.
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PMID:The role of antiphospholipid antibodies in lupus nephropathy. 853 46

Clinical analysis was performed on anti-cardiolipin x beta2 glycoprotein 1 (ACL x beta2 GP1) antibody positive patients with collagen vascular diseases. Nine patients out of 89 showed positive aCL x beta2 GP1 antibody which was a relatively lower percentage compared to that of othoffanti-phospholipid antibodies, such as anti-cardiolipin antibody (23 out of 58), lupus anti-coagulant (15 out of 51) or biological false positive (BFP) test for syphilis (10 out of 50). However, 8 patients out of 9 with positive aCL x beta2 GP1 antibody showed thrombotic lesions, a relatively higher frequency compared to that seen in aCL x beta2 GP1 negative patients. Among these cutaneous manifestations, livedo reticularis and palmar nodules with histopathological evidence of thrombosis were the most characteristic features. All but one patient with palmar nodules showed positive aCL x beta2 GP1 antibody, anti-cardiolipin antibody and lupus anticoagulant.
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PMID:Clinical analysis of anti-cardiolipin.beta 2 glycoprotein 1 antibody positive patients in anti-phospholipid syndrome. 866 20

'Antiphospholipid' (aPL) antibodies are of clinical importance because of their strong association with vascular thrombosis, recurrent pregnancy loss, thrombocytopenia and other clinical manifestations like livedo reticularis, chorea and cardiac valvular disease. While aPL antibodies have traditionally been thought to be directed against negatively-charged (anionic) phospholipids current evidence suggests that these autoantibodies recognise protein-phospholipid complexes or the proteins themselves. A number of candidate proteins have been investigated with the two most extensively researched being beta 2-glycoprotein I and prothrombin.
Lupus 1996 Apr
PMID:Immunology of antiphospholipid antibodies and their interaction with plasma proteins. 874 30

Cutis marmorata telangiectatica congenita (CMTC) is a cutaneous vascular abnormality characterized by a persistent cutis marmorata pattern, spider nevuslike telangiectasia, and superficial ulceration. An infant girl was noted at birth to have characteristic features of CMTC as well as anti-Ro/SSA antibodies and a positive autonuclear antibody (ANA) test. Serologic studies carried out on her mother were positive for Ro/SSA antibodies as well as ANA (titer 1:2560). Therefore the diagnosis of neonatal lupus was made. We propose that livedo patterns mimicking CMTC might sometimes represent the residual phase of neonatal lupus active during intrauterine life.
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PMID:Cutis marmorata telangiectatica congenita or neonatal lupus? 880 25

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

Parvovirus B19 can cause a wide range of cutaneous or extracutaneous diseases. There are three types of exanthemas, erythematous maculopapulous or vesicopapulous exanthema including erythema infectiosum, the less common maculoerythematous exanthemas and purpural exanthemas including vascular, thrombocytopenic or non-vascular or non-thrombopenic forms such as le socks and gloves syndrome. Reports of other manifestations have been more sporadic: generalized livedo, polymorphous rash, lupus-like rash and Kawasaki syndromes. The presence of unusual presentations and the risk of fetomaternal transmission are two reasons for better understanding this virus.
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PMID:[Cutaneous manifestations of parvovirus B19 infection]. 894 10

Since ethnic differences in the disease expression of systemic lupus erythematosus (SLE) have been recognized, we compared the clinical and serological features in two different ethnic groups of patients with SLE. The study population consisted of 222 African-American and 300 Latin American (Colombian) SLE patients. Clinical, demographic, and laboratory data were obtained by chart review using a standard data collection form. African-American patients more commonly manifested discoid skin lesions, pulmonary fibrosis, and pleuritis, and less commonly manifested photosensitivity, livedo reticularis, and vascular thrombosis than did Latin Americans. In addition, there was a higher frequency of anti-Sm, anti-RNP, and anti-Ro positivity among African-American patients compared with Latin Americans. These results are additional evidence for the presence of ethnic differences in the clinical expression of SLE.
Lupus 1997
PMID:Ethnic differences in the clinical expression of systemic lupus erythematosus: a comparative study between African-Americans and Latin Americans. 911 21


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