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1.
The abdominal manifestations of the antiphospholipid syndrome 总被引:1,自引:0,他引:1
OBJECTIVES: To study the abdominal manifestations of the antiphospholipid syndrome (APS). METHODS: We reviewed the medical literature from 1968 to 2006 using MEDLINE and the key words: APS, anticardiolipin antibodies, lupus anticoagulant, antiphospholipid (aPL) antibodies, catastrophic antiphospholipid syndrome, liver, hepatic biliary, pancreas, spleen, gastrointestinal and abdominal. RESULTS: Liver involvement is the most frequent abdominal manifestation associated with APS. Various hepatic manifestations have been reported including Budd-Chiari syndrome, hepatic-veno-occlusive disease and occlusion of small hepatic veins, nodular regenerative hyperplasia, hepatic infarction, cirrhosis, portal hypertension, autoimmune hepatitis and biliary cirrhosis. Acute intestinal infarction, intestinal angina, and intestinal bleeding have also been reported in association with aPL in addition to few sporadic cases of splenic infarction and acute pancreatitis. CONCLUSION: A high index of suspicion for any signs of abdominal involvement should be considered in patients with APS. In addition screening for aPL should be carried out in patients who present with hepatic vein occlusion and unexplained signs of intestinal angina. 相似文献
2.
Placentation, antiphospholipid syndrome and pregnancy outcome 总被引:3,自引:0,他引:3
The antiphospholipid antibodies (aPLs) are a diverse group of autoantibodies associated with a pattern of disease known as antiphospholipid syndrome (APS). Pregnancy complications secondary to placental insufficiency are key features of this disease. The mechanisms underlying the placental pathology remain unclear. In this article the process of placentation in healthy and pathological pregnancies is reviewed. The evidence for defective placentation in APS pregnancies and involvement of aPLs in this process is summarized. Finally hypotheses based on the interpretation of these studies are discussed. 相似文献
3.
T. Bohgaki M. Mukai A. Notoya M. Kondo M. Kohno U. Okushiba 《Modern rheumatology / the Japan Rheumatism Association》2001,11(2):165-167
A 24-year-old woman suffered from blurred vision and periorbital edema with remittent fever. She was diagnosed as having
systemic lupus erythematosus (SLE), complicated with myopia and retinopathy and severe chemosis. Antiphospholipid syndrome
(APS), hemophagocytic syndrome, and liver involvement were also proven. We considered that APS might cause chemosis as a result
of thrombosis-induced perfusion failure in the conjunctiva. In such cases, APS should be considered and anticoagulation therapy
associated with steroid therapy should be initiated. In systemic lupus erythematosus (SLE), chemosis, severe hepatitis, and
hemophagocytic syndrome (HPS) are rare complications. It is well known that many cases of SLE are complicated with antiphospholipid
syndrome (APS), which causes arteriovenous thrombosis. We report a case of SLE with transient myopia and severe chemosis complicated
with severe hepatitis and HPS. As this patient had antiphospholipid antibodies, these ocular complications were considered
to be related to APS.
Received: August 23, 2000 / Accepted: November 24, 2000 相似文献
4.
Our objective was to study the endocrinologic manifestations of the antiphospholipid syndrome (APS). We reviewed the medical literature from 1968 until 2005 using MEDLINE and the key words: APS, anticardiolipin antibodies, lupus anticoagulant, antiphospholipid antibodies, adrenal, thyroid, parathyroid, pituitary, diabetes, ovaries and testes. Adrenal insufficiency is the most common endocrinologic manifestation and can be the presenting symptom of APS. In patients with autoimmune thyroid disease circulating aPL have been detected. However, no clinical manifestations of APS have been described. A few cases of hypopituitarism have been reported, including a case of Sheehan's syndrome. aPL has been detected in the sera of diabetic patients, probably associated with some macroangiopathic complications. Finally only very few cases of ovarian and testicular involvement have been reported. The adrenals are the most commonly involved glands in the APS. Clinicians should keep a high index of suspicion for adrenal insufficiency in patients with APS. 相似文献
5.
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. 相似文献
6.
Isolated tricuspid valve disease in antiphospholipid syndrome. 总被引:1,自引:0,他引:1
Cardiac valve involvement in antiphospholipid syndrome (APS) has been consistently shown. However, isolated tricuspid valve disease within this syndrome has only been recently reported. We report a patient with primary antiphospholipid syndrome who had isolated tricuspid organic valve disease, pulmonary hypertension and pulmonary thromboembolism. We suggest that the APS should be listed as one of the aetiologies of organic isolated tricuspid valve disease in the adult population. 相似文献
7.
Noureddine M Bennis A Raquim S Tahiri A Chraibi N 《Archives des maladies du coeur et des vaisseaux》2003,96(4):324-331
The antiphospholipid antibody syndrome (APS) may present with serious cardiovascular complications which should be recognised by the cardiologist. The authors report a series of 6 cases of APS diagnosed after thrombotic events and the finding of antiphospholipid antibodies. The APS was primary in 5 cases and associated with tuberculous lymphadenitis in 1 case. There was cardiac involvement in 5 patients with pericardial effusion in 3 cases, complicated by tamponade as the presenting sign of primary APS in the other 2, valvular disease in one case (moderate mitral stenosis with aortic valve disease) and pulmonary embolism in one case. Five patients developed recurrent deep vein thrombosis of the legs. One patient had a transient ischaemic cerebral attack. 相似文献
8.
The heart lesions of rheumatic fever and the heart involvement in antiphospholipid syndrome (APS), have different clinical pictures. Yet, there are several common characteristics linking both diseases: 1) central nervous system (CNS) and heart involvement; 2) molecular mimicry between the a pathogen and the origin of the disease; 3) cross reacting antibodies between the pathogen and self molecules; 4) endothelial cell activation in the 'crime-area' i.e., the valves; 5) some of the patients with RF have circulating antiphospholipid antibodies, while APS may be associated with streptococcal infection; and 6) recently, a cross-reactivity between antibodies directed to the streptococcal M-protein and its synthetic derivative in rheumatic fever (RF) and antibodies derived from APS patients targeting the beta-2-glycoprotein-I (beta2GPI) and a beta2GPI related synthetic peptide. In the current paper, we summarize the possible links between the heart involvement in RF and APS. 相似文献
9.
The term ‘catastrophic’ antiphospholipid syndrome (CAPS) is defined as an accelerated form of antiphospholipid syndrome (APS) usually resulting in multiorgan failure. These patients have in common: (i) clinical evidence of multiple organ involvement developing in a very short time period; (ii) histopathological evidence of multiple small vessel occlusions (a minority also have large vessel thrombosis); and (iii) laboratory confirmation of the presence of antiphospholipid antibodies (aPL), usually in high titres. Although less than 1% of patients with the APS develop this complication, its potentially lethal outcome emphasizes its importance in clinical medicine. 相似文献
10.
Susac's syndrome is a microangiopathic disorder of unknown pathogenesis presenting with encephalopathy, hearing loss and branch retinal artery occlusions. The term 'catastrophic' antiphospholipid syndrome (APS) is used to define a subset of the APS characterized by thrombotic microangiopathy with clinical evidence of three or more organ involvement developed in a short period of time. We describe a patient with typical features of Susac's syndrome, that appeared in less than a week, in whom aPL were detected, thus fulfilling criteria for 'probable' catastrophic APS. 相似文献
11.
In the course of the antiphospholipid syndrome (APS), the renal involvement attributable to primary APS, APS nephropathy (APSN), corresponds to vaso-occlusive lesions of the intrarenal vessels, associated side-by-side, acute thromboses with chronic arterial and arteriolar lesions, leading to zones of cortical ischemic atrophy. A second retrospective study of 114 lupus patients was undertaken to determine whether APSN can be found in the course of systemic lupus and if there are biologic or clinical predictive factors of the presence of APSN. We showed the following 相似文献
12.
Ruan Y Bridges JS Kumar K Raphael JA Acharjee S Welty FK 《Clinical rheumatology》2008,27(12):1577-1579
Antiphospholipid syndrome (APS) is a disorder characterized by recurrent venous or arterial thrombosis and/or fetal loss;
involvement of cardiac valves is also seen. A seronegative variant has been described previously. We report a case of a woman
with recurrent pregnancy loss, prior strokes, and a negative workup for known antiphospholipid antibodies. During her current
pregnancy, she presented with acute stroke and mitral valve vegetation. Her workup for antiphospholipid syndrome and other
thrombophilias remained negative even after the stroke. Her mitral valve vegetation resolved completely with aspirin, heparin,
and warfarin. We believe this to be the first report of complete resolution of valvular vegetation with antiplatelet and anticoagulant
therapy alone in a patient with seronegative antiphospholipid syndrome. Moreover, this appears to be the first report of stroke
associated with this condition. 相似文献
13.
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombotic tendency that affects most organ systems in the human body. In this report, we present a review of the endocrinologic manifestations associated with APS by evaluating the medical literature from 1968 to 2009 using MEDLINE and these keywords: APS, antiphospholipid syndrome, antiphospholipid antibodies, anticardiolipin antibodies, lupus anticoagulant, anti β-2 glycoprotein I, pituitary, adrenal, thyroid, parathyroid, ovary, testes, diabetes mellitus, and diabetes insipidus. Adrenal insufficiency was found to be the most common endocrine manifestation associated with APS. Autoimmune thyroid disease was associated with increased titers of antiphospholipid antibodies (aPL) without any APS clinical manifestations. In addition, hypopituitarism and Sheehan syndrome are increasingly being reported in association with aPL. Data regarding the prevalence and significance of aPL in diabetic patients remains uncertain. Finally, only a few cases of ovarian and testicular derangements have been reported. APS should be considered in any patient with adrenal insufficiency even in the absence of other thrombotic manifestations. It is also advisable to assess aPL in the sera of patients presenting with pituitary insufficiency. Further studies are needed to clarify the relationship between aPL and thyroid disorders and diabetes mellitus. 相似文献
14.
Pollyanna d’ávila Leite Jozélio Freire de Carvalho 《Rheumatology international》2012,32(10):3265-3268
The antiphospholipid syndrome (APS) can be primary, when it occurs alone, or secondary, when it is associated with another autoimmune disease, mainly systemic lupus erythematosus and rarely other autoimmune diseases. Cases described in literature (Medline 1966 to December 2009) associate the presence of antiphospholipid antibodies with the presence of APS and systemic sclerosis (SS). Currently, however, no cases of the SS variant sine scleroderma with APS have been described. In this study, the authors describe the case of a patient with APS characterised by thrombosis of the retinal veins, in May 2006, the presence of lupus anticoagulant and an anticardiolipin IgG antibody. In May 2007, this patient developed Raynaud’s phenomenon, a lack of oesophageal motility and nailfold capillaroscopy with a scleroderma pattern. The patient was positive for the anti-centromere antibody but lacked any evidence of cutaneous thickening or involvement. In summary, the authors describe the first case of a patient with APS associated with SS sine scleroderma. 相似文献
15.
Starakis I Mazokopakis E Siagris D Liossis S Karatza C Antonopoulos A 《Rheumatology international》2007,27(8):781-783
We are reporting a case of catastrophic antiphospholipid syndrome (CAPS) in an adult female manifested with severe hypertension
followed by adrenal haemorrhagic infarction and adult respiratory distress syndrome. Adrenal involvement appears to be exceedingly
high in CAPS and adult respiratory distress syndrome (ARDS) is the prevailing pulmonary manifestation in this condition, compared
with these impediments in patients with simple antiphospholipid syndrome (APS). Even though the above manifestations are well
recognized, their combination as an initial presentation of CAPS is uncommon. 相似文献
16.
A growing body of evidence suggests that aPL are not only serological markers of the antiphospholipid syndrome (APS), but
may also directly contribute to the development of thrombosis and other manifestations, including the APS vasculopathy. The
latter has been documented in leptmeninges, lung, skin, myocardium, peripheral arteries, and kidney. Renal lesions, a common
feature of primary antiphospholipid syndrome (PAPS), include occlusion of principal renal arteries or their main branches,
TMA, cortical ischemia, and renal vein thrombosis. Within the cardiac manifestations associated with aPL, valvular involvement
is the most common. Histologic findings in valve specimens are consistent with a noninflammatory lesion characterized by intravalvular
capillary thrombosis, laminar or verrucous superficial thrombosis, vascular proliferation, fibrosis, and calcification. Even
though there is general consensus that endothelial damage triggers the chain of events that results in valve thickening, fusion,
rigidity, and ultimately functional abnormalities, we believe that more experimental work remains to be done on the initial
valve insult in APS. 相似文献
17.
Sanna G Bertolaccini ML Cuadrado MJ Khamashta MA Hughes GR 《Rheumatology (Oxford, England)》2003,42(2):200-213
The antiphospholipid (Hughes) syndrome (APS) is characterized by arterial and/or venous thrombosis and pregnancy morbidity in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disorder or secondary to a connective tissue disease, most frequently systemic lupus erythematosus. Central nervous system (CNS) involvement is one of the most prominent clinical manifestations of APS, and includes arterial and venous thrombotic events, psychiatric features and a variety of other non-thrombotic neurological syndromes. In this review we focus on the common and some of the less common CNS manifestations that have been reported in association with antiphospholipid antibodies. 相似文献
18.
Systemic lupus erythematosus and antiphospholipid syndrome in children and adolescents 总被引:3,自引:0,他引:3
Systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) can be associated with significant morbidity in children and adolescents. Renal involvement in SLE appears to be more severe and more frequent in the pediatric age group, with the major predictors for poor outcome being the severity of histopathologic lesions, severity of renal impairment at diagnosis, and hypertension. In addition to currently recognized cardiovascular and pulmonary involvement, accelerated atherosclerosis is of increasing concern in young individuals with SLE, because of both disease effects and medication usage. Neuropsychiatric SLE seen in childhood ranges from subtle cognitive dysfunction to severe central nervous system involvement; however, there is controversy over the value of different diagnostic studies. APS in children may be associated with SLE, idiopathic, or associated with viral infections. Systemic anticoagulation is recommended for patients with thrombotic events, but long-term management has not been well studied in children. 相似文献
19.
Anam Akhlaq Taimur A. Ali Saulat H. Fatimi 《Journal of the Saudi Heart Association》2016,28(2):124-126
Libman–Sacks endocarditis, first discovered in 1924, is a cardiac manifestation of systemic lupus erythematosus (SLE). Valvular involvement has been associated with SLE and antiphospholipid syndrome (APS). Mitral valve, especially its posterior leaflet, is most commonly involved.We report a case of a 34 year old woman with antiphospholipid antibody syndrome and SLE, who presented with mitral valve regurgitation. The patient underwent a prosthetic mitral valve replacement, with no followup complications.We suggest mechanical valve replacement employment in the management of mitral regurgitation in Libman–Sacks endocarditis, in view of the recent medical literature and our own case report. 相似文献
20.
Krause I Lev S Fraser A Blank M Lorber M Stojanovich L Rovensky J Chapman J Shoenfeld Y 《Annals of the rheumatic diseases》2005,64(10):1490-1493
BACKGROUND: Heart valves lesions and central nervous system involvement are among the most common manifestations of the antiphospholipid syndrome (APS). OBJECTIVE: To evaluate possible interrelations between these manifestations in a large group of APS patients. METHODS: 284 APS patients were evaluated retrospectively, 159 of whom had primary APS. Cardiac-CNS associations were determined for the entire study population, and for subgroups of patients with primary APS or APS associated with systemic lupus erythematosus (SLE). RESULTS: Significant associations where found between cardiac vegetations and epilepsy (p < 0.02), and between cardiac valve thickening or dysfunction and migraine (p = 0.002). Borderline association was found between valvar vegetations and migraine (p = 0.09). A significant association was also found between all valvar lesions and stroke or transient ischaemic attacks. Subanalyses showed that patients with primary APS had significant associations between cardiac valve pathology and all CNS manifestations, while patients with APS associated with SLE had no such associations. CONCLUSIONS: The study suggests potential differences in biological behaviour between primary APS and APS associated with SLE. The presence of cardiac valve pathology may be a risk factor for several types of CNS involvement in PAPS. 相似文献