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 objective of this study was to analyse whether primary antiphospholipid syndrome (PAPS) may precede and modify the characteristics of systemic lupus erythematosus (SLE). Out of the total 362 SLE patients in our service, 223 patients had antiphospholipid antibodies (aPL), of whom 110 met the criteria of antiphospholipid syndrome. In 26 cases (7.2%) PAPS appeared 5.5 years before the onset of lupus (PAPS+SLE Group). Their clinical findings were compared to lupus patients without (SLE only Group, n = 26) and with secondary APS (SLE+SAPS Group, n = 26). The prevalence of deep venous thrombosis, stroke/TIA, recurrent fetal loss, coronary heart disease and myocardial infarction was significantly higher in PAPS+SLE Group as compared to SLE only Group. The difference in prevalence of fetal loss (P = 0.014) between PAPS+SLE and SLE+SAPS Groups was also recorded. On comparison to PAPS+SLE Group, patients without APS (SLE only Group) were younger at onset of lupus, with more frequent flares and a higher prevalence of WHO type III/IV nephritis (P = 0.007), requiring higher doses of cyclophosphamide and corticosteroids. Lupus started in the form of PAPS in 7.2% of our SLE patients, who presented with more thrombotic and less inflammatory complications than in SLE patients without a prior or with a following secondary APS. Considering the long latency between the two diseases, PAPS may be a forerunner of lupus, but it may also coexist with SLE as an independent autoimmune disorder.
Lupus 2007
PMID:Primary antiphospholipid syndrome as the forerunner of systemic lupus erythematosus. 1757 33

The objective of this study was to analyse the prevalence and characteristics of the main clinical and immunological manifestations at the onset and during the evolution of the disease in a cohort of patients from Latin America (mainly of mestizo origin) and to compare the Latin American with the European patients. Clinical and serological characteristics of 100 APS patients from Mexico and Ecuador were collected in a protocol form that was identical to that used to study the ;Euro-Phospholipid' cohort. The cohort consisted of 93 female patients (93.0%) and seven (7.0%) male patients. There were 91 mestizos (91.0%), seven whites (7.0%) and two Amerindians (2.0%). The most common manifestations were livedo reticularis (40.0%), migraine (35.0%), inferior extremity deep vein thrombosis (32.0%), thrombocytopenia (28.0%) and hemolytic anemia (20.0%). Several clinical manifestations were more prevalent in Latin American than in European patients and they included mainly neurological (migraine, transient global amnesia, acute ischemic encephalopathy, amaurosis fugax) and cutaneous (livedo reticularis, skin ulcerations, superficial cutaneous necrosis, multiple subungual splinter hemorrhages) manifestations as well as hemolytic anemia. The APS has a wide variety of clinical and immunological manifestations at the onset and during the evolution of the disease and the ethnic origin in addition to environmental and socioeconomic factors can modify the disease expression.
Lupus 2007
PMID:Antiphospholipid syndrome in Latin American patients: clinical and immunologic characteristics and comparison with European patients. 1757 41

This article describes a 57-year-old woman with SLE and positive antiphospholipid and lupus anticoagulant antibodies (Ig type M) who developed an acute Addisonian crisis due to bilateral adrenal haemorrhage. It is important to recognise that bilateral adrenal haemorrhage can be part of the thrombo-embolic complications that are seen in both the primary and the secondary APS. Since there have been a number of similar cases, we suggest that APS with adrenal haemorrhage should be taken into consideration in SLE patients with abdominal pain, fever, nausea, hypotension and hyponatremia.
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PMID:[Acute Addisonian crisis in a patient with systemic lupus erythematosus and secondary antiphospholipid syndrome]. 1759 92

Perinatal thrombosis in infants born to mothers with antiphospholipid antibodies (aPL) is a rare event, but with risk of death or severe sequelae. We analysed 16 infants with such perinatal thrombosis reported in the literature in the last 20 years. Thromboses were arterial (13/16), mostly strokes (8/16). Hydrops fetalis with left renal vein thrombosis was associated to a lupus anticoagulant (LA) present only in the child. Risk factors additional to aPL: either prenatal (preeclampsia and/or intra-uterine growth retardation) or perinatal (asphyxia, sepsis, arterial or venous catheter and congenital thrombophilia) were present (one to four of them) in nine out of the 14 evaluable babies. aPL were the only risk factor found in five full term babies who suffered from stroke in four cases and from renal thrombosis in another. Eleven of these infants with aPL in their serum presented a neonatal APS with the same antibody (LA or aCL IgG) found in neonates and their mothers, while the other infants had thrombosis with aPL only in their mother's blood. aCL IgM was only found in one neonate who suffered from sepsis. Thrombosis treatments were diverse. This analysis suggests that women with aPL should be investigated for other thrombophilic risk factors and that aPL should be detected systematically at birth in the offspring of mothers with APS.
Lupus 2007
PMID:Infant perinatal thrombosis and antiphospholipid antibodies: a review. 1771

APS is recognized increasingly as a leading cause of vascular thrombosis in the pediatric population. With the obvious exception of pregnancy morbidity, most of the clinical features that may occur in adults with APS have been described also in children. Because the coincident prothrombotic factors that are common in adults have little or no impact in children, pediatric patients with APS constitute a suitable sample to investigate the relationship of aPL with the associated clinical manifestations, such as thrombocytopenia, hemolytic anemia, chorea, and livedo reticularis, and the specificities of aPL that are more linked to thrombosis. On the other hand, because of the high frequency of infectious processes in early life, children may have a greater prevalence of nonpathogenic and transient aPL. For these reasons, the diagnostic and therapeutic approach to APS in childhood may be different from that for adults. Because of the rarity of aPL-related thrombosis in children, the natural history and optimal management can be defined only through large, multicenter, controlled studies. A internet-based registry for pediatric patients with APS (Ped-APS Register) has been recently established as part of the activities of the Euro-aPL Forum and the Lupus Working Group of the Pediatric Rheumatology European Society. This registry is aimed to obtain information on APS in childhood, particularly regarding association of aPL with clinical manifestations, specificity of aPL, impact of treatment and long-term outcome (http://www.med.ub.es/MIMMUN/FORUM/PEDIATRIC.HTM).
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PMID:Antiphospholipid syndrome in pediatrics. 1582 Mar 76

Immune thrombocytopenic purpura (ITP) can be classified as primary (known also as idiopathic thrombocytopenic purpura) or as secondary to an underlying condition such as a malignant or nonmalignant disorder. Commonly occurring conditions associated with secondary ITP include lymphoproliferative disorders (chronic lymphocytic leukemia [CLL], Hodgkin's disease and non-Hodgkin's lymphomas), autoimmune collagen vascular diseases (systemic lupus erythematosus [SLE], thyroid disease, antiphospholipid syndrome [APS]), and chronic infections (human immunodeficiency virus [HIV], Helicobacter pylori, hepatitis C virus [HCV]). The mechanism of platelet destruction in thrombocytopenias associated with lymphoproliferative disorders and collagen vascular diseases is identical to the autoimmune mechanism seen in primary ITP. Drug-induced thrombocytopenias are uncommon and generally resolve quickly upon drug discontinuation, but are often attributed to other causes. Platelet destruction in infection-associated ITP occurs via various mechanisms including accelerated platelet clearance due to immune complex disease as seen in HIV infection or cross-reactivity of anti-platelet glycoprotein antibodies and viral antigens in HIV, HCV, and H pylori infections (antigenic mimicry). In patients with HCV-related cirrhotic liver disease, splenic sequestration secondary to portal hypertension and decreased production of thrombopoietin may further contribute to development of thrombocytopenia. The current treatment paradigm for secondary ITP varies according to the underlying condition. Standard treatments for primary ITP (corticosteroids, IVIG, anti-D, splenectomy) are often successful in secondary ITP. In cases of ITP with H pylori and HCV infection, treatment should focus on the underlying disorder.
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PMID:Other immune thrombocytopenias. 1809 69

The APS was described 25 years ago as a triad of manifestations (GRV Hughes). During the last 3 decades the disease became more systemic than systemic lupus erythematosus (SLE). The paper entails many of the old clinical findings as well as novel ones which are still not well documented in large series. The authors also refer to the second hit theory of infectious origin of APS. How to treat and indications for self monitoring the INR are detailed. The question of whether specific IVIG (directed against anti cardiolipin) or anti CD 20 be incorporated into the therapeutic armamentarium employed in APS will be answered in the near future.
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PMID:Hughes Syndrome (the antiphospholipid syndrome): ten clinical lessons. 1819 Aug 89

The antiphospholipid syndrome is characterized by recurrent fetal loss, venous and/or arterial thrombosis, and thrombocytopenia associated with elevated titers of lupus anticoagulant and anticardiolipin antibodies. Although thrombosis is the characteristic vascular involvement in APS, the development of vascular aneurysms in patients with APS has been reported. We describe four patients with established APS who developed abdominal aortic aneurysm, and review the literature on previous published cases of arterial aneurysms developing in patients with APS. In addition, we discuss the possible pathophysiological association between APS and the development of this vascular abnormality.
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PMID:Coexistence of the antiphospholipid syndrome and abdominal aortic aneurysm. 1830 May 73

Fertility in patients with SLE and other systemic autoimmune disease is usually unaltered. However, fertility may be impaired by anovulation during episodes of active disease or chronic renal failure, administration of NSAIDs, high dose of corticosteroids and cyclophosphamide. Early pregnancy loss occurs in SLE patients with aPLs. An association of autoimmune disease with infertility has been suggested, but the studies are not conclusive. Ovulation stimulation as a fertility treatment could theoretically induce SLE. However, two recent studies did not find previous use of fertility drugs and in vitro fertilization to be more frequent in the history of SLE patients when compared with controls. Patients with SLE or primary APS, who are undergoing infertility treatment, could be at risk of flare or thrombosis. In the past 10 yrs, many reports have been published regarding the risk of lupus exacerbation associated with controlled ovarian hyperstimulation; not all found excess risk. At the moment we do not have any prospective study in this field. A trend towards a worse prognosis in cases of SLE patients undergoing assisted reproductive techniques (ARTs) for pregnancy rate, live-birth rate and maternal complications can be seen. If hormonal ovarian stimulation is useful, well-advised management would administer a low effective gonadotropin dose in a patient whose disease has been silent preferably for at least 6 months. Further data are needed to establish safety and efficacy of ART in SLE patients.
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PMID:Treating infertility in autoimmune patients. 1850 85

The involvement of the central nervous system (CNS) is one of the major causes of morbidity and mortality in systemic lupus erythematosus (SLE) patients and the less understood aspect of the disease. Its recognition and treatment continue to represent a major diagnostic and therapeutic challenge. Due to the lack of controlled randomized trials, current therapeutic approach is still empirical and based on clinical experience. The therapeutic choice depends on accurate diagnosis, identification of underlying pathogenic mechanism, severity of the presenting neuropsychiatric symptoms, and on prompt identification and management of contributing causes of CNS disease. Mild neuropsychiatric manifestations may need symptomatic treatment only. In more severe CNS disease it is important to distinguish between thrombotic and non-thrombotic mechanisms. Focal CNS manifestations, particularly TIA and stroke, are associated with the presence of antiphospholipid antibodies (aPL). Anticoagulation is warranted in patients with thrombotic disease, particularly in those with the antiphospholipid (Hughes) syndrome (APS). Other CNS manifestations, such as demyelinating syndrome, transverse myelitis, chorea, seizures, migraine and/or cognitive dysfunction, when associated with persistent positivity for aPL, may also benefit from anticoagulation in selected patients. Severe diffuse CNS manifestations, such as acute confusional state, generalised seizures, mood disorders and psychosis, generally require corticosteroids in the first instance. Pulse intravenous cyclophosphamide therapy may help when more severe manifestations are refractory to corticosteroids and other immunosuppressive agents, generally when response is not seen in 3-5 days. Plasmapheresis may also be added in severe cases of symptoms refractory to conventional treatment. Intravenous immunoglobulins, mycophenolate mofetil, rituximab, intratecal methotrexate and dexametasone deserve further studies to confirm their usefulness in the treatment of neuropsychiatric SLE. This article reviews the clinical approach to therapy in patients with SLE and neuropsychiatric involvement.
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PMID:Neuropsychiatric involvement in systemic lupus erythematosus: current therapeutic approach. 1853 50


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