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 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Peripheral arterial occlusive disease is a frequent disease due to the classical vascular risk factors such as smoking, diabetes mellitus, dyslipidemia, and hypertension. Despite these risk factors, many thrombophilias (physiological inhibitors defects, Factor V Leiden and 20210A prothrombin gene variant, antiphospholipid antibodies, mild hyperhomocysteinemia 15-30micromol/l) can be evoked in some clinical forms of peripheral arterial occlusive disease. This paper provides a synthesis of the published data about this topic. Screening for these thrombophilias is justified in patients with venous thromboembolic disease, or signs of antiphospholipid syndrome and possibly in different situations such as premature atheroma of lower limbs, chronic ischaemia, evolutive disease despite adapted treatment and revascularisation failures without evident technical explanation. Except for the antiphospholipid syndrome, there is currently no consensus for systematic screening of thrombophilia and treatment in patients with peripheral arterial occlusive disease.
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PMID:[Thrombophilias and peripheral arterial occlusive disease]. 1855 34

Antiphospholipid syndrome is an autoimmune disease characterized pathophysiologically by the presence of antiphospholipid antibodies and > or =1 clinical manifestation, the most common being venous or arterial thrombosis. We describe the case of a 40-year-old male with unexplained severe pulmonary arterial hypertension with a seven-day history of progressive shortness of breath, hemoptysis, chest discomfort and bilateral pedal edema. Electrocardiographic, echocardiographic and imaging studies showed changes consistent with chronic thromboembolic pulmonary hypertension. Further work-up showed positive anticardiolipin antibodies and lupus anticoagulant with negative features for lupus with negative primary thrombophilic studies as well. The patient was managed adequately with oral anticoagulation with improvement of his clinical status and referred to a tertiary care center to be screened for pulmonary thromboendarterectomy. For patients meeting surgical selection criteria, pulmonary thromboendarterectomy has demonstrated positive outcomes with respect to survival, functionality and quality of life. We discuss the pathophysiology and treatment as well as novel therapies in nonsurgical candidates with chronic thromboembolic pulmonary hypertension in the setting of primary antiphospholipid syndrome.
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PMID:Chronic thromboembolic pulmonary hypertension as an uncommon presentation of primary antiphospholipid syndrome. 1859 78

There are clinical observations and data from literature showing that kidney involvement is frequently found in antiphospholipid syndrome (APS). The clinical pictures of renal involvement in APS can be different: from modest to severe hypertension, nephritic or nephrotic syndrome and chronic kidney disease at all stages. There is no clear connection between the presence of a significant level of antiphospholipid antibodies (aPL) and advancing of nephropathy especially occurring in course of connective tissue diseases. In the paper the relations between APS, the presence of aPL and nephropathy have been discussed.
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PMID:[The kidneys and antiphospholipid syndrome]. 1877 22

Antiphospholipid syndrome (APS) is an acquired thrombotic disorder. It mainly occurs with systemic disease or as a primary disorder. All organs may be involved by thrombosis, but to date the most common endocrine manifestation is chronic adrenal insufficiency. Very few cases of hypopituitarism with primary APS have been reported. We report the case of a 27-year-old woman, victim of a stroke leading to double vision and intracranial hypertension. Magnetic resonance imaging showed a macro-adenoma with hemorrhage of a suprasellar lesion. Hormone assessment showed hyper prolactinemia with positive anticardiolipin antibody. Our case is the second reported associating APS with apoplexy. We discuss the clinical, biological and radiological features observed in our case. We conclude that APS should be searched for whenever a history of adenoma with apoplexy is found associated with recurrent thrombosis.
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PMID:[Pituitary necrosis and antiphospholipid syndrome]. 1893 32

Systemic lupus erythematosus (SLE) is an autoimmune chronic disease, which involves multiple organs, particularly joints, skin, heart, lungs, nervous system and kidneys. Systemic lupus erythematosus has the predilection to women in their reproductive years and causes numerous health complications during the pregnancy and labour. Lupus flares occur during each trimester of pregnancy and also at postpartum period. Complications are usually mild and appear as the skin and joint involvement. The main challenge are flares in women with severe renal form of SLE. Such exacerbations often need the intensive treatment measures for the mother. The presence of lupus nephritis during conception may aggravate current arterial hypertension in the mother and lead to chronic renal failure progression. In the study authors report the risk factors for spontaneous abortions and premature births in women with SLE particularly in the group with the presence of antiphospholipid syndrome. The features and course of neonatal SLE was also discussed. The goals of medication of women with SLE and lupus nephritis during pregnancy and the treatment during postpartum period and lactation were described.
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PMID:[Systemic lupus erythematosus and pregnancy]. 1893 68

The vasculature plays a crucial role in inflammation, angiogenesis, and atherosclerosis associated with the pathogenesis of inflammatory rheumatic diseases, hence the term 'vascular rheumatology'. The endothelium lining the blood vessels becomes activated during the inflammatory process, resulting in the production of several mediators, the expression of endothelial adhesion molecules, and increased vascular permeability (leakage). All of this enables the extravasation of inflammatory cells into the interstitial matrix. The endothelial adhesion and transendothelial migration of leukocytes is a well-regulated sequence of events that involves many adhesion molecules and chemokines. Primarily selectins, integrins, and members of the immunoglobulin family of adhesion receptors are involved in leukocyte 'tethering', 'rolling', activation, and transmigration. There is a perpetuation of angiogenesis, the formation of new capillaries from pre-existing vessels, as well as that of vasculogenesis, the generation of new blood vessels in arthritis and connective tissue diseases. Several soluble and cell-bound angiogenic mediators produced mainly by monocytes/macrophages and endothelial cells stimulate neovascularization. On the other hand, endogenous angiogenesis inhibitors and exogenously administered angiostatic compounds may downregulate the process of capillary formation. Rheumatoid arthritis as well as systemic lupus erythematosus, scleroderma, the antiphospholipid syndrome, and systemic vasculitides have been associated with accelerated atherosclerosis and high cardiovascular risk leading to increased mortality. Apart from traditional risk factors such as smoking, obesity, hypertension, dyslipidemia, and diabetes, inflammatory risk factors, including C-reactive protein, homocysteine, folate deficiency, lipoprotein (a), anti-phospholipid antibodies, antibodies to oxidized low-density lipoprotein, and heat shock proteins, are all involved in atherosclerosis underlying inflammatory rheumatic diseases. Targeting of adhesion molecules, chemokines, and angiogenesis by administering nonspecific immunosuppressive drugs as well as monoclonal antibodies or small molecular compounds inhibiting the action of a single mediator may control inflammation and prevent tissue destruction. Vasoprotective agents may help to prevent premature atherosclerosis and cardiovascular disease.
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PMID:Vascular involvement in rheumatic diseases: 'vascular rheumatology'. 1894 76

Pre-eclampsia is a pregnancy-associated disease of the second part of the pregnancy, occurring mainly after 20th weeks gestation. The prevalence of hypertension in pregnancy is between 5 to 11% and affects mainly women under 20 years of age. An inadequate invasion of trophoblasts with consequential placental ischemia as a result of insufficiently dilated uterine spiral arteries is thought to be an initial cause in the pathogenesis of pre-eclampsia. The clinical symptoms of pre-eclampsia, such as loss of intravascular volume and edema, are caused by generalized endothelial dysfunction. These symptoms are potentiated by hypertension and reduced colloid osmotic pressure in the plama. The organs being affected by pre-eclampsia are those of the vascular-, hepatic-, renal-, cerebral- and coagulatory systems. The prognosis is much more severe when pre-eclampsia develops very early in the pregnancy. The symptoms include elevated blood pressure (over 140 mmHg systolic, 90 mmHg diastolic) combined with proteinuria. Frequent symptoms are hyperreflexia and edema. The etiology of pre-eclampsia has not been clearly defined. Risk factors/triggers for the development of pre-eclampsia can include chronic hypertension, advanced maternal age at first pregnancy (over 35 y), nephropathy, thrombophilia (heterozygous factor V Leiden mutation, antiphospholipid syndrome, heterozygous prothrombin mutation and homozygous MTHFR), multiple gestation and prior pregnancy with preeclampsia. The incidence of preeclampsia is higher in nulliparous than multiparous women. In many countries pre-eclampsia is still most frequent cause of maternal perinatal mortality. HELLP-Syndrome (haemolysis-elevated liver enzyme- low platelets) is a severe progressive course of this disease. Eclampsia, characterized by generalized tonic-clonic convulsion, is the most dangerous complication of pre-eclampsia, and may develop before or after delivery. This form of pre-eclampsia is associated with higher maternal and fetal mortality. Constant maternal hypertension potentially alter vascular integrity of the placenta with further consequences in fetal blood supply leading to growth restriction or zero growth and subsequently resulting in low birth weight or fetal death. The sooner the disease is detected and confirmed, the better the maternal and fetal prognoses are. This is the reason why it is major importance, together with the employment of preventive measures, to identify patients with risk factors with pre-eclampsia though an adequate screening method, thereby detecting the disease earlier and ensuring better pregnancy outcomes for both mother and child.
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PMID:[Pre-eclampsia screening in first and second trimester]. 1897 29

The objective of this study is to report a case of primary antiphospholipid syndrome (APS) presenting as complete renal artery occlusion, and to review the literature on the subject. We describe the clinical presentation, course and outcome of one patient who presented with resistant hypertension later found to be due to thrombosis and complete occlusion of the left renal artery. We review the medical literature registered in the Medline PubMed database from 1966 to 2004 using keywords: antiphospholipid, Hughes syndrome, kidney, renal, renal artery thrombosis. We describe one patient and analyzed ten well-documented cases of renal artery thrombosis due to APS. Most of the patients were women, at a mean age of 32 years. All but one case had primary APS. The presenting symptom was hypertension in ten cases. Most patients had both lupus anticoagulant and anticardiolipin antibodies. Arterial occlusion was left sided in 55%, right sided in 27% and bilateral in 18%. Renal artery thrombosis is an uncommon presentation of APS. This entity should be considered in the differential diagnosis of severe hypertension.
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PMID:Antiphospholipid syndrome presenting as unilateral renal artery occlusion: case report and literature review. 1903 8

Although the kidney represents a major target organ in antiphospholipid syndrome (APS), renal involvement in APS was poorly recognized until recently. The most well-recognized renal manifestations of APS are the renal artery thrombosis/stenosis, renal infarction, hypertension, renal vein thrombosis, end-stage renal disease, increased allograft vascular thrombosis, some types of glomerular disease, and a small-vessel vaso-occlusive nephropathy, recently defined as APS nephropathy. APS nephropathy was first described in primary APS patients, characterized by acute thrombotic lesions in glomeruli and/or arterioles (thrombotic microangiopathy) and chronic vascular lesions such as fibrous intimal hyperplasia of arterioles and interlobular arteries, organized thrombi with or without recanalization, and fibrous arterial and arteriolar occlusions or focal cortical atrophy. APS nephropathy was also detected in further studies including patients with systemic lupus erythematosus (SLE)-related APS and SLE/non-APS patients with positive antiphospholipid antibodies, independently of lupus nephritis. The same histologic lesions, especially thrombotic mictroangiopathy, were also observed in patients with catastrophic APS. The most frequent clinical and laboratory characteristics of APS nephropathy in all the above groups of patients are hypertension (often severe), proteinuria (ranging from mild to nephrotic range), hematuria, and acute or chronic renal insufficiency.
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PMID:Renal involvement in the antiphospholipid syndrome (APS)-APS nephropathy. 1904 14

Rheumatologists are sometimes asked to see patients with severe manifestations of vascular syndromes termed "pseudovasculitis," which often closely mimic autoimmune vasculitis. These syndromes include cholesterol embolism, fibromuscular dysplasia, antiphospholipid syndrome, ergotism, neurofibromatosis, and primary amyloidosis. In patients with suspected vasculitis, sometimes dramatic clinical findings may require urgent decision-making before all testing has been concluded. These circumstances make it particularly important for the clinician to consider pseudovasculitis in the differential diagnosis. Our review describes the common presentations of pseudovasculitis-which may include fever, weight loss, weakness, hypertension, and vascular occlusions-and emphasizes on their diagnostic features. A careful history and physical examination, laboratory and radiographic studies, as well as a high level of suspicion will help the clinician to consider pseudovasculitis in which treatment with immunosuppressive agents such as high dose corticosteroids or cytotoxic agents would be contraindicated and possibly disastrous.
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PMID:"Vasculitis look-alike" clinical syndromes. 1907 66


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