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)

aPL-associated thrombosis (Hughes syndrome) is widely recognized as a major cause of organ damage in autoimmune diseases. Beginning with the first symposium in 1984, international aPL symposia have facilitated research on aPL antibodies, and the clinical standardization of aPL tests. It is hoped that the present symposium will continue this tradition, because much remains to be learnt about the origin and pathogenicity of aPL antibodies. In addition, new insights are needed for more effective therapies to be developed.
Lupus 1996 Oct
PMID:The syndrome of thrombosis, thrombocytopenia, and recurrent spontaneous abortions associated with antiphospholipid antibodies: Hughes syndrome. 890 57

The objective was to study antiphospholipid antibody syndrome (APS or Hughes syndrome) in two major teaching hospitals in Kuwait. patients with suspected Hughes syndrome were investigated with tests for anticardiolipin antibodies (aCL) and lupus anticoagulants (LAC) over 1 yr. Diagnosis was considered confirmed if significant levels of either or both antibodies with no obvious cause (primary), or with systemic lupus erythematosus (SLE) or SLE-like illness (including SLE serology) (secondary) were present. Twelve (37.5%; seven females, 58%) primary and 20 (62.5%; 18 females, 90%) secondary Hughes syndrome patients were seen during this period. patients were Kuwaiti, Middle-Eastern and North-African Arabs (29). Filipinos (2) and White (1). None were from the Indian subcontinent. The main presentation was thrombosis in 75% (arterial in 25% and venous in 50%), and recurrent abortions in 50% of married women. Haematological and dermatological manifestations were limited entirely to the secondary variety, seen in 25% and 19%, respectively. Clinical manifestations were severe, leading to death in one, intensive-care management in 31% and with partial or complete warfarin resistance or brittleness in 25%. Neurological/eye and cardiac manifestations were not seen, as these patients may be attending separate speciality hospitals for these diseases in Kuwait. The approximate prevalence of this syndrome was 2.66/1000 admissions in medical wards. Projected to the total referral areas of the two hospitals, an approximate figure of 52 patients/million population/year was obtained. Hughes syndrome was a common problem among Arabs, Filipinos and possibly Whites in Kuwait. Its manifestations were severe, often requiring intensive-care management, and in one case it was fatal. Patients from the Indian subcontinent were conspicuous by their absence, despite the fact that they were well represented in all other rheumatic disease groups. Ethnic and/or geographical factors could be important in this syndrome. To the best of our knowledge, this is the first report of Hughes syndrome from the Middle East.
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PMID:Hughes syndrome: a common problem in Kuwait hospitals. 894 1

Antiphospholipid Syndrome (APS) was first described by Hughes and sometimes called as Hughes syndrome. Recent studies revealed that the antigen to anticardiolipin antibody (aCL) is not cardiolipin itself but co-factor beta 2-GPI which expresses its epitope when it combines cardiolipin or gets oxidized. Lupus Anticoagulant is now possibly considered as anti-prothrombin antibody. Livedo including Snedden syndrome, pulmonary hypertension and skin ulcer became considered as the part of symptoms of this disease. In ISAPA 1998, it is reported from several laboratories that IgA aCL is also pathogenic to thrombosis as well as IgG aCL. Atherosclerosis is also accelerated by aCL. Catastrophic APS is rare but fatal, reported 3 cases in Japan and 50 cases in the world.
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PMID:[Antiphospholipid syndrome]. 1007 9

We report the case of a patient with an acute cytomegalovirus (CMV) infection who developed Hughes syndrome, manifested by a common iliac vein thrombosis. IgM anticardiolipin antibodies (aCL) appeared with the onset of the infection, followed later by IgG aCL. Five months later, both IgM and IgG aCL levels disappeared from the serum. This is the second case of Hughes syndrome associated with CMV infection to be reported in the literature.
Lupus 1999
PMID:Hughes syndrome associated with cytomegalovirus infection. 1060 54

Hughes (antiphospholipid) syndrome (APS) can mimic multiple sclerosis (MS). We analyzed the clinical, laboratory, and imaging findings of MS-like expression in a cohort of patients with APS in an attempt to identify parameters that might differentiate the 2 entities. We studied 27 patients who were referred to our unit with the diagnosis of probable or definite MS made by a neurologist. All patients were referred to our lupus clinic because of symptoms suggesting an underlying connective tissue disease, uncommon findings for MS on magnetic resonance imaging (MRI), atypical evolution of MS, or antiphospholipid antibody (aPL) positivity. aPL, antinuclear antibody (ANA), anti-dsDNA, and anti-extractable nuclear antigen (ENA) antibodies were measured by standard methods. MRI was performed in every patient and compared with MRI of 25 definite MS patients who did not have aPL. An index severity score was calculated based on the size and number of increased signal intensity areas in MRI. In the past medical history, 8 patients with primary APS and 6 with APS secondary to systemic lupus erythematosus (SLE) had had symptoms related to these conditions. Neurologic symptoms and physical examination of the patients were not different from those common in MS patients. Laboratory findings were not a useful tool to distinguish APS from MS. When MRI from APS patients was compared globally with MRI from MS patients, MS patients had significantly increased severity score in white matter (p < 0.001), cerebellum (p = 0.035), pons (p < 0.015), and when all areas were taken together (p < 0.001). Patients with APS had significantly increased scores in the putamen (p < 0.01). No differences were noticed in the degree of atrophy. When taken individually, MRI from APS patients could not be distinguished from MRI from MS patients. Most of the patients with primary APS showed a good response to oral anticoagulant treatment. In patients with secondary APS, the outcome was poorer. Hughes syndrome (APS) and MS can be difficult to distinguish. A careful medical history, a previous history of thrombosis and/or fetal loss, an abnormal localization of the lesions in MRI, and the response to anticoagulant therapy might be helpful in the differential diagnosis. We believe that testing for aPL should become routine in all patients with MS.
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PMID:Can neurologic manifestations of Hughes (antiphospholipid) syndrome be distinguished from multiple sclerosis? Analysis of 27 patients and review of the literature. 1067 Apr 10

Anti-beta2-Glycoprotein I (beta2GPI) autoantibodies are the prominent laboratory feature of Hughes syndrome. By prolonging some coagulation tests in the presence of exogenous phospholipids (PL), they behave as classical Lupus Anticoagulants (LA). We investigated the effect of 3 affinity-purified anti-beta2GPI IgG preparations from patients with Hughes syndrome on fibrin polymerization and fibrinolysis of normal plasma, measured by comparing the optical densities of assay mixtures in the presence of the autoantibodies or normal IgG. The presence of anti-beta2GPI IgG in diluted Russell Viper Venom Time (dRVVT) assays, carried out using a PL dilution of 1:8 or 1:64, resulted in a delay in the onset of polymerization by 30-40 and 60-70s, respectively. Fibrin polymerization was complete after 250s for both anti-beta2GPI IgG and normal IgG. The inhibitory effect of the anti-beta2GPI antibodies was not observed in the presence of excess PL, as expected for LA. Anti-beta2GPI IgG increased the plateau level of polymerization when dRVVT was performed in the presence of 1.5 nM recombinant tissue plasminogen activator, but did not impair the fibrinolytic process, which was almost complete after 250 min. The autoantibodies did not delay the onset of fibrin polymerization in tests carried out using recombinant tissue factor. On the contrary, the autoantibodies enhanced polymerization in prothrombin time assays, and accelerated it in tissue thromboplastin inhibition tests, with no effect on fibrinolysis. These data provide evidence that anti-beta2GPI LA may act as either anticoagulants or procoagulants in different in vitro coagulation tests.
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PMID:Effect of anti-beta2glycoprotein I Lupus Anticoagulants on fibrin polymerization and fibrinolysis. 1095 74

Despite an active international effort to improve diagnosis and treatment of the antiphospholipid syndrome (Hughes syndrome), there remain problems of lack of standardization and lack of prospective and multivariate epidemiologic analysis which restrict the diagnostic and predictive ability of commercially available tests. Nevertheless, current published series provide some data from which strategic approaches can be used to maximize the efficiency and usefulness of available tests. For further updates on new research and developments of interest to physicians and patients with this syndrome, the following web sites may prove helpful: www.slrapls.org, www.hematology.org, www.acforum.org, www.americanheart.org, www.rarediseases.org, www.aarda.org, and www.lupus.org.
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PMID:Which antiphospholipid antibody tests are most useful? 1153 58

The antiphospholipid or Hughes syndrome is the association between antiphospholipid antibodies (aPL), venous and arterial thromboses and pregnancy morbidity. Antiphospholipid syndrome (APS) commonly coexists with autoimmune diseases usually systemic lupus erythematosus (SLE), when it is known as secondary APS. When present in isolation it is known as primary APS (PAPS). Although the kidney may be affected in APS, its involvement is perhaps not as well described as that of other organs. Thrombotic microangiopathy (TMA) affecting the kidney has been reported as a manifestation in both primary and secondary APS. This report describes hypertension related to underlying renal TMA as a presenting symptom of APS.
Lupus 2002
PMID:Hypertension as the presenting feature of the antiphospholipid syndrome. 1204 90

A hallmark of systemic lupus erythematosus (SLE) and related autoimmune diseases such as the antiphospholipid syndrome (APL or Hughes syndrome) is an apparent breakdown in tolerance, the process by which the body distinguishes self from nonself in order to maintain a versatile immune defense while protecting itself from self-annihilation. To some extent, loss of tolerance is a desirable feature of host immunity, and is known to occur in healthy individuals. Optimal tolerance then is probably not an all or nothing phenomenon. Autoimmunity should be seen as a breakdown in homeostasis rather than a completely aberrant kind of immunity. This leads to special considerations in the assessment of potentially toleragenic therapies, in which an attempt is made to re-educate the immune system. LJP 1082 is designed as a polyvalent antigenic structure aimed at crosslinking specific surface immunoglobulin and tolerizing B cells to beta2-glycoprotein I. Issues of antigenic selection and multiplex forces influencing tolerance and immunity may have impact on its optimal development and use in patients.
Lupus 2004
PMID:LJP 1082: a toleragen for Hughes syndrome. 1523 Feb 88

The antiphospholipid (Hughes) syndrome (APS), is characterized by arterial and/or venous thrombosis and pregnancy morbidity in association with antiphospholipid antibodies (aPL). Since its classical description 21 years ago, the clinical spectrum of Hughes syndrome has embraced the realms of obstetrics, nephrology, cardiology, neurology, gastroenterology and now, possibly orthopaedics. This is not surprising, given that this disease can affect virtually any organ system and blood vessel of any size and nature. Just as venous thrombosis may affect limbs and internal organs, arterial thrombosis has been shown to affect organs such as the brain, eye, heart, kidney, liver and may also involve the skeleton. In this review, the skeletal aspects of Hughes syndrome, postulated pathogenesis and possible implications of anticoagulation will be discussed. Finally, the approach to APS patients undergoing orthopaedic surgery shall also be outlined.
Lupus 2005
PMID:Orthopaedic manifestations of the antiphospholipid (Hughes) syndrome. 1593 32


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