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1.
Systemic lupus erythematosus Pregnancy can trigger-off or worsen the disease (60% of lupus flares). The renal damage and hypertension are important prognostic factors. Regarding the foetus, there is a 60% risk of prematurity. The risk of neonatal lupus is basically related to the presence of anti-Ro-SSA and anti-La-SSB antibodies and cannot not be foreseen. Multi-disciplinary surveillance is a necessity for any woman suffering from lupus and wishing to become pregnant. Other connective disorders Pregnancy is rare during scleroderma and has no influence on its progression except in the case of renal damage. Regarding dermatopolymyositis, pregnancy, which is rare, might trigger-off or worsen the disease. Bullous diseases Porphyria may be triggered-off or worsened during pregnancy. The various forms of pemphigus may appear or be enhanced by pregnancy. Various affections The influence of sarcoidosis on pregnancy is negligible. Complications are possible in patients exhibiting a type I or IV Ehlers-Danlos syndrome. Pregnancy can enhance the vascular complications of a pseudoxanthoma elasticum. An exacerbation is possible in the case of a type I neurofibromatosis. Progression varies in the case of psoriasis, atypical dermatitis, acne and Beh?et's disease. Genital infections are frequent during pregnancy and often represent a risk for the infant (vaginal candidosis, viral condyloma, gonococci, herpes infection). Varicella The risk of severe neonatal varicella is high when the disease appears in the mother just before or after delivery.  相似文献   

2.
The antiphospholipid syndrome is a disorder of hypercoagulability in association with circulating antiphospholipid antibodies directed against epitopes on oxidized phospholipids complexed with a glycoprotein, beta 2-glycoprotein I, or against the glycoprotein itself. Disorders associated with antiphospholipid antibodies but not the antiphospholipid syndrome, such as HIV and hepatitis C infection, appear to lack antibodies to beta 2-glycoprotein I. Patients with systemic lupus erythematosus have a high incidence of antiphospholipid antibodies with a high risk of thrombosis, often associated with anticardiolipin antibodies, beta 2-glyocoprotein I antibodies, and the presence of the lupus anticoagulant. Antiphospholipid antibodies are a significant cause of morbidity and mortality in renal patients with and without systemic lupus erythematosus. Renal manifestations include thrombotic microangiopathy and large vessel thrombosis. In patients with end-stage renal disease, antiphospholipid antibodies are prevalent and may increase in frequency with time on dialysis, possibly as a result of oxidative stress incurred during dialysis. The presence of anticardiolipin antibodies have been associated with a high incidence of hemodialysis access clotting. In renal transplant recipients, the incidence of antiphospholipid antibodies is also elevated and may be associated with a higher incidence of primary graft non-function. Although patients with systemic lupus erythematosus have similar renal allograft survival rates to the general population, survival is worse for those patients who are also antiphospholipid antibody positive. Additionally, in hepatitis C positive renal transplant recipients, the presence of anticardiolipin antibodies confers an increased risk of thrombotic complications and the development of thrombotic microangiopathy. Treatment of antiphospholipid antibody syndrome remains centered around anticoagulation with warfarin. The use of immunosuppressive agents has had no dramatic effect on antiphospholipid antibody titers and little clinical effect on thrombotic events.  相似文献   

3.
Current studies indicate that a thrombotic microangiopathy (TMA) identifies patients with systemic lupus erythematosus (SLE) who are at high risk of progressing to end-stage renal disease. We have observed two patients with SLE and one patient with a primary antiphospholipid syndrome (APS) who developed acute renal insufficiency with thrombocytopenia. Renal biopsies showed a TMA characterized by thrombi or by cellular and mucoid intimal hyperplasia of small arteries and arterioles. No arterial or arteriolar immune-complex deposits were detected by immunofluorescent or electron microscopy. Biopsies from one SLE patient and the APS patient showed no immune-complex glomerular disease. Both had serum antiphospholipid antibodies (aPL). aPL were not detected in the serum of the other SLE patient who had an active lupus nephritis. Acute renal failure and thrombocytopenia resolved in each case following treatment by plasmapheresis or prednisone and heparin. None of the patients were initially treated with cytotoxic drugs. As more knowledge is gained, the accurate identification of renal vascular lesions in SLE or related diseases could influence renal prognosis and choice of therapy. The cases reported here provide further evidence that a TMA can cause acute renal failure independent of lupus nephritis. TMA should be distinguished from other forms of renal vascular disease, particularly a noninflammatory lupus microangiopathy, which is probably mediated by subendothelial immune-complex deposits. The absence of immunoglobulin deposits in vessels involved by a TMA indicates that microvascular thrombosis is promoted by mechanisms other than those usually attributed to immune-complex disease. Phospholipid reactive antibodies may be pathogenetic in some cases.  相似文献   

4.
Pregnancy and lupus nephritis.   总被引:1,自引:0,他引:1  
Family planning and pregnancy are important and usually problematic issues for a young woman with lupus nephritis. Moderate renal insufficiency and previous use of alkylating cytotoxic drugs are associated with decreased fertility. Oral contraceptives containing synthetic estrogens are contraindicated in women with active lupus nephritis, uncontrolled hypertension, history of thromboembolic diseases or high levels of antiphospholipid antibodies. Mild flares of systemic lupus erythematosus (SLE) are common during pregnancy, severe renal flares and permanent impairment of renal function are uncommon. The outlook of pregnancy for women with lupus nephritis is usually favourable if the disease (both renal and nonrenal) has been quiescent for at least 6 months before pregnancy, and if, at conception, serum creatinine is less than 140 micromol/l, proteinuria less than 3 g/24 h and blood pressure controlled. The risk of fetal loss is, however, at least 2-3 times higher than in the normal population and pre-eclampsia, prematurity and fetal growth retardation frequently complicate these pregnancies. Especially poor fetal outcome is associated with antiphospholipid antibodies. Pregnancies in women with lupus nephritis require intense fetal and maternal surveillance.  相似文献   

5.
The risk of pregnancy in patients with lupus nephritis   总被引:4,自引:0,他引:4  
Since many women with systemic lupus erythematosus (SLE) are of child-bearing age and have normal fertility, the clinician must often face many problems relating to pregnancy in patients with lupus nephritis. These include the influence of lupus nephritis on fetal outcome, obstetric complications, and the influence of pregnancy on SLE. Studies published in the 1960s underlined the increased fetal and maternal risk and recommended against pregnancy in lupus patients. The prognosis for non-pregnant SLE active patients was also poor, making it difficult to know whether pregnancy altered the prognosis of the disease. Recent prospective studies indicate that the majority of lupus mothers can sustain pregnancy without detrimental effects, providing that the pregnancy is planned during the inactive phase of the disease. Nevertheless the fetal risk, although progressively reduced during the last 40 years, continues to be higher than in pregnancies of healthy women particularly in patients with antiphospholipid antibodies.  相似文献   

6.
The antiphosphospholipid antibody syndrome (APS) describes a clinical entity with recurrent thrombosis, fetal loss, thrombocytopenia in the presence of lupus anticoagulant and/or antibodies to cardiolipin. These antibodies may be associated with connective tissue diseases such as systemic lupus erythematosus (secondary APS) or be found in isolation (primary APS). Renal syndromes increasingly being reported in association with these antibodies include thrombotic microangiopathy, renal vein thrombosis, renal infarction, renal artery stenosis and/or malignant hypertension, increased allograft vascular thrombosis, and reduced survival of renal allografts. Although much has been understood concerning the biology of these antibodies and the pathogenesis of thrombosis, the optimal therapy remains to be elucidated. This article presents a historical review of the renal involvement in the antiphospholipid syndrome and discusses therapeutic options. Further research is needed.  相似文献   

7.
Making pregnancy safer for patients with lupus   总被引:7,自引:0,他引:7  
Systemic lupus erythematosus is a hormone-dependent disorder predominantly affecting young women in whom changes in hormonal activity affect the course of the disease. More specifically, pregnancy is associated in 50 to 60% of cases with a clinical flare manifesting as renal or hematological symptoms, usually during the second or third trimester but occasionally in the postpartal period. Patients should be strongly advised to start a pregnancy only if their disease has been stable for at least 6 months, and they should be informed of the few contraindications. Severe flares are uncommon (10%) and the risk of maternal death is now 2 to 3%. The risk of the fetus remains high, however. Thus, fetal loss is common, particularly in patients with a lupus anticoagulant, renal failure, or arterial hypertension. Preterm birth occurs in 25% to 50% of cases and intrauterine growth retardation in 30%. Neonatal lupus with or without congenital heart block is exceedingly rare, being seen in the 1% of SLE women who have anti-SSA (Ro) and/or SSB (La) antibodies. To ensure a favorable outcome for both the mother and the child, the pregnancy should be planned, started during a period of disease stability, monitored closely (physical examination, laboratory tests, and Doppler ultrasound), and treated as needed (with antimalarial therapy throughout the pregnancy). Even with these precautions, however, preterm birth remains common.  相似文献   

8.
The case notes of 20 obstetric patients with antiphospholipid syndrome delivering over a 4-year period were reviewed retrospectively. There were complete details for 22 singleton pregnancies. Obstetric complications in the pregnancies under review were frequent. Nine (47%) of the 19 women whose pregnancies reached the third trimester were delivered by caesarean section. During seven of the pregnancies there was significant prolongation of the phospholipid-dependent coagulation tests secondary to lupus anticoagulant and/or anticardiolipin antibodies. Four of these women received epidural blockade without sequelae. This finding, however, cannot provide valid evidence for the safety or otherwise of epidural placement in this group of patients, since the series is small and the incidence of epidural haematoma is exceedingly rare in the obstetric population. Paradoxically, these patients are hypercoagulable. The estimated blood loss at these deliveries did not exceed 600 ml. Eleven women (55%) had a previous history of thrombosis. Two women had thrombotic episodes during their pregnancies and three had them post partum despite antithrombotic measures.  相似文献   

9.
Renal replacement therapy in lupus nephritis   总被引:6,自引:0,他引:6  
The indications and the choice of renal replacement therapy for lupus patients are similar to those for other uremic patients. However, lupus patients can pose some particular problems. First, 10-28% of patients needing dialysis can have a partial renal function recovery. Therefore, the clinician has to decide whether to administer a rescue treatment, risking side-effects, or to reduce immunosuppression precluding a potential recovery. Many patients on regular dialysis show subdued biological and clinical activity. Others can show a hectic disease activity, particularly in the 1st year. In these cases, treatment is difficult, as vigorous immunosuppression can expose uremic patients to severe side-effects. The presence of circulating antiphospholipid antibodies (aPL) can favor thrombosis or stenosis of vascular access (VA). Renal transplantation is the best therapy for most lupus patients with end-stage renal failure. Many, but not all, studies have reported similar patient and graft survival rates in lupus and in non-lupus transplant recipients. The results are much better with living donor transplantation. Patients with aPL, black patients and those on long-term dialysis have a higher graft failure risk. Candidates with active lupus and/or those with significant iatrogenic morbidity should be advised to wait 6-12 months before transplantation. The recurrence risk of lupus nephritis ranged between 2% and 30% in different studies. The histological picture does not usually show severe features. Antiplatelet agents or anticoagulation can be advised for aPL patients.  相似文献   

10.
We report a case of a 5.8 cm. right renal artery aneurysm diagnosed intact 8 weeks post partum. Rupture of a renal arterial aneurysm during pregnancy is a rare but well described catastrophic event. There are no previous reports of an intact renal artery aneurysm diagnosed either ante partum or post partum. To our knowledge, this also represents the largest reported renal artery aneurysm. The aneurysm was repaired successfully with kidney salvage and closure of the fistulous connection to the renal vein.  相似文献   

11.
AIM: To determine the prevalence of antiphospholipid syndrome nephropathy (APSN) in Thai systemic lupus erythematosus (SLE) patients who underwent renal biopsy and to compare the relationship of renal histopathology and other significant clinical parameters between SLE patients with and without APSN. METHODS: A retrospective analysis was undertaken in systemic lupus erythematosus patients (n = 150, 44 <15 years old, 106 0e;15 years old) who underwent renal biopsy. The specimens were evaluated for histological features of APSN and other significant clinical parameters. The result of antiphospholipid antibodies, clinical course, and renal function from chart review were analysed. RESULTS: The prevalence of APSN in systemic lupus erythematosus patients who underwent renal biopsies was 34% (16% in <15-year-old group, 41.5% in > or =15-year-old group). APSN was associated with more severe hypertension (P = 0.002 for systolic and P = 0.004 for diastolic blood pressure), acute renal failure (P = 0.003), persistent heavy proteinuria (P < 0.001 for 4+ proteinuria), severe lupus nephritis (class III and IV, P = 0.014, high activity and chronicity indices, P < 0.001) and a tendency to progress to end-stage renal disease. CONCLUSION: Systemic lupus erythematosus patients who underwent renal biopsies in our institute showed a prevalence of APSN comparable to those in western countries. The presence of APSN was significantly higher in the adult than in the paediatric population. Its association with poor prognostic indicators suggests poor renal outcome. Clinicians should be aware of this condition in order to give proper care to systemic lupus erythematosus patients.  相似文献   

12.
Antiphospholipid syndrome nephropathy in systemic lupus erythematosus.   总被引:7,自引:0,他引:7  
In the course of the antiphospholipid syndrome (APS), the existence of vaso-occlusive lesions capable of affecting numerous organs is now well established. The renal involvement attributable to primary APS, APS nephropathy (APSN), corresponds to vaso-occlusive lesions of the intrarenal vessels, associating side-by-side, acute thromboses with chronic arterial and arteriolar lesions, leading to zones of cortical ischemic atrophy. A retrospective study of 114 lupus patients undergoing renal biopsy was undertaken to determine the following: (1) if APSN can be found in the course of systemic lupus erythematosus (SLE); (2) if certain clinical and biologic factors can permit the prediction of the presence of APSN; and (3) if APSN is a superadded renal morbidity factor in lupus patients. This study shows the following: (1) APSN occurs in SLE (32% of patients with renal biopsies) in addition to, and independently of, lupus nephritis; (2) APSN is statistically associated with lupus anticoagulant but not with anticardiolipin antibodies; (3) APSN is associated with extrarenal APS, mainly arterial thromboses and obstetrical fetal loss, but not with the venous thromboses of APS; (4) APSN is an independent risk factor, over and above lupus nephritis, that contributes to an elevated prevalence of hypertension, elevated serum creatinine, and increased interstitial fibrosis. Thus, it seems likely that, because of its associations with hypertension, elevated serum creatinine, and increased interstitial fibrosis, APSN may worsen the prognosis in these patients. APSN may also have therapeutic significance in that its recognition should permit a better balance between immunosuppressor and antithrombotic and/or vasoprotective therapy. Finally, this study suggests that APSN should be considered as an element to be included in the classification criteria of APS.  相似文献   

13.
Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome. We present the case of a 12-year-old boy who presented with a devastating illness with multiple thrombotic episodes and rapidly progressive renal failure.  相似文献   

14.
ABSTRACT Unlike the Libman-Sacks vegetations of acute systemic lupus erythematosus (SLE), which are usually asymptomatic, valve involvement in chronic SLE and primary antiphospholipid antibody syndrome (APLAS) is similar to that of chronic rheumatic disease. Typical findings include valve thickening and nodularity, poor coaptation, and regurgitation. Elevated levels of antiphospholipid antibodies have been associated with the development of these valvular abnormalities in some but not all reported cases, and there are undoubtedly other etiologic cofactors. When cardiac valvular replacement is required, special attention must be given to preoperative reduction of elevated antibody levels, prevention of intraoperative thromboembolism, and prompt and aggressive postoperative anticoagulation.  相似文献   

15.
The objective of this study was to look for the occurrence of catastrophic antiphospholipid syndromes (APS) and to try to detect discriminating factors for predicting a worse prognosis, related to Lupus anticoagulant (LA) and antiphospholipid antibodies (aPL), in systemic lupus erythematosus (SLE) with main renal involvement. Regression, recursive partition and logistic regression analyses were applied to our 80 SLE patients prospectively followed up since 1980. Immunologic and other laboratory parameters including beta 2-glycoprotein 1 dependence, resistance to activated protein C caused by a substitution on the coagulation factor V gene, induction of monocyte procoagulant activity. Regression studies demonstrated an overall worse prognosis in term of both thrombosis and death for the group of LA/aPL positive patients (33/80). However, recursive partition analysis was able to isolate a small high risk-subgroup (8/33) characterized by persistent LA/aPL antibodies positive result, widespread signs of noninflammatory vasculopathy (skin, brain, kidney) and renal pathology mimicking that of thrombotic microangiopathy or arteriolosclerosis, also in the absence of classic SLE-nephritis. Only in this subset, three catastrophic APS were recorded, while, in traditional SLE nephritis, even persistent LA/aPL positive results (sometimes after one previous thrombosis) did not seem to imply a particularly severe prognosis. All serologic criteria employed are unable to identify high-risk patients. We conclude that catastrophic APS is a rare event in renal SLE. Before more predictive serologic markers become available, a simple algorithm, dealing with clinical data and renal histologic patterns, may help physicians to identify putatively high risk-LA/aPL antibodies in SLE patients with main renal involvement. This ominous subset does not belong to the group of classic SLE-nephritis.  相似文献   

16.
Antiphospholipid antibodies are a relatively heterogeneous mix of immunoglobulins with binding specificities for negatively charged or neutral phospholipids. Currently, the most commonly detected antiphospholipid antibodies include the anticardiolipin antibody, the lupus anticoagulant, and an antibody implicated in false-positive VDRL testing. Recently, a clinical syndrome of vaso-occlusive disorders associated with antiphospholipid antibodies has been identified and may result from immune-mediated disruption of endothelial function. This clinical syndrome encompasses arterial and venous thrombosis, recurrent fetal loss, neurologic dysfunction (eg, migraine, chorea, and encephalopathy), systemic and pulmonary arterial hypertension, and endocardial disease. Although most commonly associated with systemic lupus erythematosus, the antiphospholipid antibody syndrome also has been identified in patients with vaso-occlusive disease without systemic lupus erythematosus. Recently, identification of antiphospholipid antibodies has been facilitated by the development of a more sensitive assay for anticardiolipin antibody. In this article, case histories of three patients with arterial thrombosis and associated anticardiolipin antibodies, including the first associated case of terminal aortic thrombosis, are reviewed and the subject of the antiphospholipid antibody syndrome is discussed.  相似文献   

17.
The vascular endothelium is a common target of inflammatory joint disease. Autoimmune diseases including rheumatoid arthritis, systemic lupus erythematosus, and Sj?gren's syndrome can be responsible for a spectrum of vascular disorders that encompasses vasculitis, thrombosis and/or atheroma associated with the antiphospholipid syndrome, and vascular damage caused by cryoglobulin deposition. These mechanisms can coexist, particularly in lupus patients. Joint disease is sometimes the presenting manifestation in primary vasculitis. Autoantibodies are detectable in most patients with vascular involvement and inflammatory joint disease. They are not merely markers for vascular involvement: in vitro and in vivo data suggest that some autoantibodies may contribute to the genesis of endothelial lesions, together with other factors. For instance, evidence of pathogenic effects has been found for antineutrophil cytoplasmic antibody (ANCA), most notably with antimyeloperoxidase or antiproteinase-3 specificity, in small-vessel vasculitides (Wegener's granulomatosis, Churg-Strauss syndrome, and microscopic polyangiitis); for immune complexes, particularly those containing cryoglobulins, in vasculitides secondary to CTDs; and for circulating anticoagulant and anticardiolipin antibodies, above all anti-beta2-glycoprotein I, in antiphospholipid syndrome. Antibodies to annexin V, modified lipoproteins, and endothelial cells may be of interest; their clinical relevance is unclear, however, and no standardized assays are available, so thatthese antibodies are not looked for in everyday practice. When deciding which antibody tests should be performed in a given patient, the circumstances surrounding the onset of the vasculopathy should be borne in mind. In patients with previous CTD, the tests are selected based on the diagnosis. In contrast, in a patient with no previous diagnosis, a vasculopathy can be either primary or secondary to undiagnosed CTD or to antiphospholipid syndrome: consequently, a broader array of tests is needed in this situation.  相似文献   

18.
This study compares pregnancy outcomes in systemic lupus erythematosus (SLE) patients post renal transplant with recipients with other primary diagnoses, utilizing data from the National Transplantation Pregnancy Registry, Philadelphia, PA. Recipients were referred from transplant centers nationwide. A retrospective analysis was performed using data from questionnaires, hospital records and telephone interviews. Outcomes of pregnancies post renal transplant secondary to lupus nephritis (SLE: n = 38; 60 pregnancies) were compared with the pregnancy outcomes of renal recipients with other diagnoses (non-SLE: n = 247; 374 pregnancies). Drug-treated hypertension during pregnancy was less common in the SLE group than in the non-SLE group (45.0% vs. 62.5%, p = 0.015). There were fewer cesarean sections in the SLE group (30.2 vs. 53.2%, p = 0.008). There was no primary or gestational diabetes in the SLE group. There were no other statistical differences in maternal conditions or pregnancy outcomes between the SLE and non-SLE groups, or in the incidence of post pregnancy graft loss. Female recipients transplanted for renal failure secondary to lupus nephritis can successfully maintain pregnancy. Outcomes are comparable to renal recipients with other diagnoses. Newborns in both groups were often premature and had low birthweight. Overall childhood health was reported to be good; there were no apparent predominant structural malformations among the children.  相似文献   

19.
BACKGROUND:: Cerebral cavernous malformations are brain vascular malformations associated with intracranial hemorrhage. It is unclear whether pregnancy is a risk factor for hemorrhage, yet there is speculation that it may be. OBJECTIVE:: To compare the risk of clinically significant hemorrhage during pregnancy and nonpregnancy. METHODS:: A total of 186 patients from the University of Toronto Vascular Malformations Study Group were enrolled. The obstetrical history of each patient was collected and matched to their neurological history from the records of the study group. All hemorrhagic events occurring during childbearing years were associated with either a defined pregnancy risk period or nonpregnancy period. Patients were also asked to recall advice that they received from health care professionals regarding risk of hemorrhage in pregnancy. RESULTS:: Among our patient population there were 349 pregnancies (283 live births) and 49 hemorrhages during childbearing years, 3 of which were during pregnancy but none during delivery or within 6 weeks post partum. The hemorrhage rate for pregnant women was 1.15% (95% confidence interval: 0.23-3.35) per person-year and 1.01% (95% confidence interval: 0.75-1.36) per person-year for nonpregnant women. Relative risk of pregnancy was 1.13 (95% confidence interval: 0.34-3.75) (P = .84). Neurosurgeons and obstetricians were the source of most hemorrhage risk advice. The majority of neurosurgeons suggested that the risk was unchanged, but the obstetricians were divided. Four patients never conceived, and 2 others began contraception because of the advice that they received. CONCLUSION:: The risk of intracranial hemorrhage from cerebral cavernous malformations is likely not changed during pregnancy, delivery, or post partum. ABBREVIATIONS:: CCM, cerebral cavernous malformationCI, confidence interval.  相似文献   

20.
We experienced a case of lupus nephritis with antiphospholipid antibody syndrome. A renal biopsy specimen from this patient showed various renal histological changes, but the results of urinalysis were almost normal. The patient was a 56-year-old woman diagnosed as having systemic lupus erythematosus with antiphospholipid antibody syndrome in 1983. In 1998, she had diarrhea and blood gas analysis showed metabolic acidosis. Therefore, she was admitted to our hospital and underwent renal function examination. The glomerular filtration rate was reduced(GFR: 40/ml/min), but urinalysis was almost normal. To examine her renal dysfunction, we performed open renal biopsy. Her renal tissues showed global glomerular sclerosis, mesangial cell proliferation and infiltration of cells in the tubulointerstitial area(WHO II). Furthermore, some arterioles showed organized thrombus formation and recanalization due to the antiphospholipid antibody syndrome. Renal biopsy of patients with lupus nephritis is useful not only for precise diagnosis, but also for the selection of appropriate treatment.  相似文献   

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