Symptomatic thrombotic events among Egyptian patients with systemic lupus erythematosus: special consideration for renal vein thrombosis

OBJECTIVE: To evaluate the prevalence of symptomatic thrombotic events among Egyptian patients with systemic lupus erythematosus (SLE), and to evaluate the frequency and the risk factors associated with renal vein thrombosis in those patients. METHODS: Fifty-four patients with SLE, 51 (94.4%) females, were involved in this study. All of them were submitted for abdominal sonography, chest X-ray, echocardiography, and Doppler of renal, abdominal and lower limb veins, with examination of data on clinical and laboratory profile. Abdominal CT, brain MRI, MRI both hips, CT chest and pulmonary scintigraphy were used when needed. RESULTS: Sixteen patients (29.6%) were diagnosed with symptomatic thrombotic events. Eight patients had more than one type of thrombosis. Two patients (3.7%) were diagnosed by Doppler as having renal vein thrombosis (RVT). This was confirmed by abdominal CT. One of them presented with nephrotic syndrome, graded by renal biopsy as World Health Organization (WHO) class V, and had positive anticardiolipin antibodies (ACL). The other patient had RVT and inferior vena cava (IVC) thrombosis, nephrotic syndrome, positive ACL, and died before renal biopsy was performed. Both of them were without history of peripheral thrombotic events. One patient was diagnosed with IVC thrombosis, lupus nephritis grade II, positive ACL, and diagnosed by abdominal CT. One patient was diagnosed with portal vein thrombosis and had positive ACL. One patient with retinal vessel thrombosis and positive ACL. Four patients had deep vein thrombosis (DVT). Recurrent miscarriages were reported in 4 patients (7.4%), skin ulcerations in 3 (5.6%), avascular necrosis of the hips in 4 (7.4%), stroke in 1 (1.9%), and pulmonary hypertension in 2 patients (3.7%). CONCLUSION: Sixteen SLE patients (29.6%) were diagnosed with symptomatic thrombotic events. RVT was detected in 2 patients representing 3.7% of all patients, and 12.5% of patients with thrombosis. Both patients with RVT presented with nephrotic syndrome.     

Renal vein thrombosis and inferior vena cava thrombosis in systemic lupus erythematosus

Phlebography of the inferior vena cava with selective study of the renal veins was performed in 43 patients with systemic lupus erythematosus (SLE). Inferior vena cava thrombosis (IVCT) or renal vein thrombosis (RVT) was found in 3 of 11 patients (27%) with nephrotic syndrome, in 8 of 13 (61.5%) with previous thrombophlebitis, and in 3 of 4 (75%) with suggestive acute clinical picture. In contrast, none of the 20 control patients with SLE had IVCT or RVT. These results show that SLE patients with thrombophlebitis have a very high risk of developing IVCT or RVT; patients with nephrotic syndrome have a smaller risk. Neither IVCT nor RVT was found in SLE patients without antecedent thrombophlebitis or nephrotic syndrome.     

Acute kidney injury as the first sign of spontaneous renal vein thrombosis: report of 2 cases

Spontaneous renal vein thrombosis (RVT) is very rare in the absence of nephrotic syndrome. It is more common in newborns and infants. RVT should always be included in the differential diagnosis of flank pain and hematuria, and because RVT can induce acute renal injury. A 19-year-old man was admitted to our hospital because he complained of right flank pain and oliguria for 3 days. Another patient, a 24-year-old man, complained of a severe and sudden onset of bilateral flank pain and anuria for a day. They were both healthy before they developed the described symptoms and had different levels of decrease in renal function when they visited the hospital. Color Doppler ultrasonography revealed RVT in both the patients. The patients received therapy, including anticoagulation and thrombolysis, following their diagnoses, and they recovered in a few days.     

Fibrinolytic therapy for bilateral renal vein thrombosis

A patient with nephrotic syndrome due to membranous glomerulonephritis experienced painful bilateral renal vein thrombosis (RVT) complicated by pulmonary emboli. He was treated with systemic fibrinolysis with streptokinase for 72 hours. Rapid clinical improvement of his condition, a fall in his serum creatinine level, and a sharp rise in his platelet count were observed. Venography verified complete lysis of the thrombus within the vena cava and reperfusion of the right renal vein. The left renal vein remained chronically occluded. Thrombolytic therapy should be further investigated as initial therapy for patients with clinical evidence of RVT.     

Bleeding in cirrhotic patients: a precipitating factor due to intravascular coagulation or to hepatic failure?

In 24 cirrhotic patients at different stages of hepatic failure, factor VIII:C (F VIII:C), factor VIIIR:AG (F VIIIR:AG), factor VIII AG/C ratio (F VIII AG/C), and serum fibrin-fibrinogen degradation products (FDP) were investigated. In 11 of the 24 patients, several instances of gastrointestinal bleeding due to esophageal varices rupture were documented and 5 patients died of unarrestable bleeding. In our study, we evaluated whether the cause of bleeding was the development of intravascular coagulation or the severity of hepatic failure. A statistically significant difference between F VIII:C, F VIIIR:AG/C ratio, and serum FDP was found in bleeding in comparison with non-bleeding patients. An inverse correlation between the F VIII:C plasma level and serum FDP as well as a direct correlation between F VIII AG/C ratio and serum FDP in the group of bleeding patients were also found. These data seem to suggest a hypercoagulable state which was more significant in the 5 patients who died owing to bleeding. Furthermore, only 1 of these patients had severe hepatic failure. From this study it appears that, in cirrhotic patients, bleeding is related more to the appearance of disseminated intravascular coagulation, as a consequence of both hemodynamic and endothelial changes, than to the degree of hepatic failure itself.     

Immunopathology of the nephrotic syndrome associated with renal vein thrombosis. Report of two cases and brief review of the literature

Renal tissue from two patients with the nephrotic syndrome and renal vein thrombosis was studied by immunofluorescence microscopy in addition to conventional histologic and electron microscopic technics. Granular deposits of immunoglobulins G (IgG), M (IgM) and beta₁ C/beta₁ A globulin (one case) were seen by fluorescence microscopy along the basement membranes in a pattern similar to that observed in patients with chronic membranous nephropathy and in the experimental model of chronic serum sickness. Renal vein thrombosis, associated with the nephrotic syndrome, is not clearly separated clinically or pathologically from primary glomerular disease with the nephrotic syndrome. In view of the lack of experimental evidence to show that either the glomerular lesion or the proteinuria is the result of elevated venous pressure alone, the pathogenesis of the lesion must remain in doubt.     

Renal vein thrombosis in nephrotic syndrome--a prospective study of 54 cases

Recent advance of radiographic technique makes antemortem diagnosis of renal vein thrombosis (RVT) possible. However the incidence of RVT in patients with nephrotic syndrome(NS) is still not known. This study was designed to investigate the incidence, clinical presentation and radiological features of RVT in NS patients in China. A prospective study including clinical, and pathological examination as well as renal venogram was carried out in all our hospitalized NS patients except those having contradiction for renal venogram. RVT was found in 23 of the 54 cases (42%) and there was obstruction in 34 renal vein branches. RVT was more common in left (19) than right side (15), in upper (19) than middle and lower branches (10). It is interesting that in our group not only membranous but also minimal change as well as mesangium proliferative glomerulonephritis had high incidence of RVT (47%, 45% and 36% respectively) Only 6 cases (26%) had a typical acute presentation with severe loin pain, significant increase of urinary protein, enlargement of involved kidney. Occasionally fever, deterioration of renal function and sterile urinary white cells were also present. The remaining patients (74%) had latent onset of RVT which could hardly be identified in the clinical course of the underlying NS. For the high incidence and atypical clinical course of RVT shown in our study, it is recommended that due attention should be paid of this complication during management of NS and interpretation of therapeutic response.     

Membranous Glomerulonephritis Associated with Sarcoidosis*

Summary: Two patients with sarcoidosis involving pulmonary hilar lymph nodes developed the nephrotic syndrome. Renal biopsy in both cases showed membranous glomerulonephritis. In one patient, there was an associated renal vein thrombosis.     

Venous thromboembolism in patients hospitalized with nephrotic syndrome

Purpose

Whether pulmonary embolism in patients with the nephrotic syndrome is caused by deep venous thrombosis or renal vein thrombosis is controversial. To determine which is the likely cause of pulmonary embolism in patients with the nephrotic syndrome, we investigated data from the National Hospital Discharge Survey.

Methods

The number of patients discharged from nonfederal short-stay hospitals in the United States with a diagnostic code of nephrotic syndrome, deep venous thrombosis, renal vein thrombosis, and pulmonary embolism was obtained using ICD-9-M (International Classification of Diseases, Ninth Revision, Clinical Modification) codes.

Results

From 1979 to 2005, 925,000 patients were discharged from hospitals with the nephrotic syndrome and 898,253,000 patients did not have the nephrotic syndrome. With the nephrotic syndrome, 5000 (0.5%) had pulmonary embolism, 14,000 (1.5%) had deep venous thrombosis, and fewer than 5000 had renal vein thrombosis. The relative risk of pulmonary embolism comparing patients with the nephrotic syndrome to those who did not have it was 1.39, and the relative risk of deep venous thrombosis was 1.72. Among patients aged 18-39 years, the relative risk of deep venous thrombosis was 6.81. From 1991-2005, after venous ultrasound was generally available, the relative risk of deep venous thrombosis (all ages) was 1.77.

Conclusion

The nephrotic syndrome is a risk factor for venous thromboembolism. This is strikingly apparent in young adults. Renal vein thrombosis was uncommon. Therefore, pulmonary embolism, if it occurs, is likely to be due to deep venous thrombosis and not renal vein thrombosis.     

Membranous Glomerulonephritis Associated with Sarcoidosis*

Summary: Two patients with sarcoidosis involving pulmonary hilar lymph nodes developed the nephrotic syndrome. Renal biopsy in both cases showed membranous glomerulonephritis. In one patient, there was an associated renal vein thrombosis.     

The coagulogram in patients with nephrotic syndrome

The coagulogram done in half of the 100 cases of nephrotic patients admitted into our hospital from 1986 to 1988 was studied. It is shown that the patients of either the renal vein thrombosis (RVT) positive or negative group were in hypercoagulability state. 46 patients in this series had RVT proved by renography. The mechanism of RVT and the clinical significance of the changes of these hemostatic data are discussed.     

Thrombosis of temporal artery and renal vein in Kimura disease related nephrotic syndrome

Kimura disease (KD) is an angiolymphoid proliferative disorder of unknown etiology, occurs mainly in Asian patients, presenting with subcutaneous slowly growing masses, with a predilection for preauricular and submandibular regions. The clinical course of the disease is thought to be benign. Concomitant peripheral blood eosinophilia and elevated serum immunoglobulin E levels are often observed. Main systemic manifestation of the KD is renal involvement. Renal abnormalities, notably proteinuria and nephrotic syndrome have been found to be associated with KD. We report a 42-year-old man with KD and a steroid-sensitive membraneous nephrotic syndrome with bilaterally temporal artery and renal vein thrombosis. This is the first reported case of KD associated nephrotic syndrome complicated with wide arterial and venous thrombosis from Anatolia.     

Nephrotic syndrome, renal vein thrombosis and renal failure. Report of case with recovery of renal function, loss of proteinuria and dissolution of thrombus after anticoagulant therapy

A case of nephrotic syndrome, renal vein thrombosis and advanced renal failure in a 52 year old woman with adult onset diabetes mellitus is reported. The diagnosis of renal vein thrombosis was established by selective renal venography. Following 15 months of anticoagulant therapy creatinine clearance increased to the level noted prior to renal vein thrombosis, and proteinuria decreased to less than 500 mg/day. Repeat renal venography demonstrated clearing of the renal vein thrombosis. Renal biopsy performed at this time revealed changes compatible with arterial nephrosclerosis on light microscopy, but there were no findings typical of diabetic intercapillary glomerulosclerosis. No immune deposits were seen on immunofluorescent microscopy. Electron microscopy revealed fusion of the foot processes, but there were no electron dense deposits in or on the basement membrane to suggest membranous glomerulonephritis. Analysis of the sequence of events suggests that the renal vein thrombosis in this patient was probably a complication of the nephrotic syndrome, the etiology of which is not clear although the biopsy findings are compatible with lipoid nephrosis.     

Lupus nephritis: 211 cases

PURPOSE: Renal involvement is one of the most severe and frequent manifestations of systemic lupus erythematosus. Prognosis factors are variable in the different studies. We analyze in 211 patients clinical, biological and histologic characteristics of lupus nephritis and the different prognosis factors. METHODS: It's a retrospective study in 211 with lupus nephritis followed-up between 1975 and 2003. RESULTS: There were 195 women and 16 men aged meanly of 28,8 years. At first presentation, we noted hypertension in 32,3% of cases, nephrotic syndrome in 47,7% of cases and renal failure in 51,6% of cases. histologic examination of kidney revealed class III in 59 cases, class IV in 97 cases and class V in 33 cases. Two hundred and five patients were treated by corticosteriods associated with immunosupressive agents in 95 cases. After a mean follow-up of 103 months (2-289 months), we obtained remission in 55,3% deterioration of renal function in 34,8% with end stage renal failure in 14,7% and relapses occurred in 51% of cases. Thirty-three patients died. Age <24 years, hypertension, nephrotic syndrome and initial renal failure were statistically associated with deterioration of renal function. CONCLUSION: Lupus nephritis is severe in our patients with predominance of proliferative forms. Age <24 years, hypertension, nephrotic syndrome and initial renal failure were statistically associated with deterioration of renal function.     

Renal vein thrombosis and the nephrotic syndrome. Report of two cases with successful treatment of one

A nephrotic syndrome and bilateral renal vein thrombosis developed in two women. One suffered low back pain, the other oliguric renal failure. Both suffered pulmonary emboli and had exhibited edema, proteinuria and hypertension during pregnancies several years before. Results of thrombectomy and anticoagulant therapy were successful in one patient, although she suffered serious hemorrhage at the biopsy site during anticoagulant therapy. The other patient died of pulmonary embolism. Renal interstitial changes usually associated with renal vein thrombosis were found in both, but neither showed membranous nephropathy (membranous glomerulonephritis). This study suggests that (1) although membranous nephropathy is frequently associated with renal vein thrombosis, it is probably not the result of that condition nor invariably associated with it. (2) Prognosis in renal vein thrombosis with nephrotic syndrome may be partially dependent upon underlying renal disease. (3) Renal biopsy is useful for diagnosis and assaying prognosis. (4) Treatment should consist of thrombectomy and anticoagulants. However, serious hemorrhage may occur in patients recently subjected to biopsy.     

慢性肾小球性疾病止血与血栓的临床研究

本文对肾病综合征、慢性肾小球肾炎和慢性肾功能衰竭患者共193例,分别进行了血小板功能,凝血、抗凝血系统,纤溶系统的测定和血栓弹力图检查,结果发现三组疾病都有高凝状态,以肾病综合征最显著。本文结果为慢性肾小球疾病采用抗凝治疗提供了理论根据。     

Renal Vein Thrombosis in a Newborn With Abnormal Factor VIII Level: Clinical Case Report

Renal vein thrombosis (RVT) in neonates is a rare condition of low mortality but significant morbidity due to renal impairment.We report the case of a male term newborn with left RVT and elevated serum factor VIII (FVIII).The main symptoms of the patient and the important clinical findings: prompt diagnosis of RVT was possible because the classic clinical presentation of macroscopic hematuria, thrombocytopenia, and palpable flank mass were present in this newborn infant.The main diagnoses: finally, the reason of RVT was established when the infant was 3 months of age: the increased level of FVIII was confirmed. We discuss the diagnosis, therapy, and outcome of the patient and compare with the literature.Therapeutics interventions: however, despite anticoagulant therapy the left kidney developed areas of scarring and then atrophy.Conclusions and outcomes: Prothrombotic defects should be considered in all patients with perinatal RVT. Elevated factor VIII as a reason of RVT in neonatal period is particularly rare. Given a poor renal outcome in children associated with elevated levels of factor VIII, consideration could be given to more aggressive antithrombotic therapy in such cases.     

Long-term prognosis of idiopathic membranous glomerulonephritis. Study of 116 untreated patients.

One hundred sixteen patients, mainly adults, with idiopathic membranous glomerulonephritis were studied to evaluate their clinical course and long-term prognosis.The onset was marked by a nephrotic syndrome in 88 patients (75.8 per cent) and by a proteinuria without the nephrotic syndrome in 28 patients (24.2 per cent). Clinical remission occurred in 23.4 per cent of cases, clinical improvement in 14.6 per cent, renal insufficiency in 19 per cent (with end-stage renal failure in 9.5 per cent) and the condition remained unchanged in 43 per cent. The actuarial survival curve shows that 76 per cent of the patients were alive at 10 years. Twenty-five per cent of the patients had hypertension during the course of the membranous glomerulonephritis. Seven women had nine pregnancies after the diagnosis of membranous glomerulonephritis was made, with successful deliveries in seven. Renal vein thrombosis was present in five of 16 patients examined for it.Glomerular lesions were classified into three groups, according to the classification of Bariety et al. At the first biopsy, 22 patients were classified as having type I lesions, 79 as having type II lesions and 15 as having type III lesions. The mean interval between discovery of the disease and renal biopsy according to the type of glomerular lesions, is shorter in patients with type I lesions than in those with type II and III lesions. Clinical improvement and clinical remission are more frequent in those with type I lesions than in those with type II and type III lesions. End-stage renal failure was never encountered in those with type I lesions and was present in 13 per cent of those with type II lesions and 7 per cent of those with type III lesions. The different histologic types can correspond to a progression of lesions with time but not to a progressive severity of the disease. In two cases there was complete resolution of the lesions and recovery of the normal appearance of the capillary wall.     

粘附分子P选择素在老年肾病综合征患者肾组织中的表达和周围血流式细胞分析

目的：探讨粘附分子Ｐ选择素（ＣＤ６２Ｐ）在老年肾病综合征（ＮＳ）中的变化。方法：采用流式细胞术和直接免疫荧光标记单克隆抗体对老年ＮＳ患者周围血和肾活检组织进行了ＣＤ６２Ｐ表达的研究。结果：①２６例老年ＮＳ患者周围血ＣＤ６２Ｐ表达量较正常组显著增高（Ｐ＜０．０１）；②肾组织中显示ＣＤ６２Ｐ表达２２例（８４．６２％），表达部位常见于肾小球系膜区、血膜袢，其次为肾小管、间质小血管及球囊壁。③ＣＤ６２Ｐ在周围血表达水平与肾组织表达程度呈显著正相关（Ｐ＜０．０１），血、肾组织的表达与患者尿蛋白定量呈正相关（Ｐ＜０．０５）。结论：ＣＤ６２Ｐ在老年ＮＳ患者肾组织和周围血中表达增强，提示其在ＮＳ发病机制中具有重要作用     

Proteinuria in renal artery occlusion is related to active renin concentration and contralateral kidney size.

OBJECTIVES: Angiotensin II, in addition to having vasopressor effects, induces proteinuria in experimental models. Proteinuria has been reported, sometimes in the nephrotic range, in patients with chronic complete renal artery occlusion. We aimed to identify the factors associated with proteinuria in such cases. DESIGN AND MAIN OUTCOME MEASURE: Complete renal artery occlusion was detected by intra-arterial angiography in 96 patients referred for hypertension. We analysed patient characteristics at presentation to identify the factors associated with proteinuria. SETTING: A referral hypertension unit. RESULTS: Median protein excretion was 0.25 g/day (range 0-11). Nine patients had nephrotic syndrome (proteinuria >/= 3.5 g/day per 1.73 m2). Patients in the upper tertile for proteinuria differed from those with lower proteinuria in terms of total cholesterol levels (P < 0.01), the proportion of diabetics (P < 0.01) and supine active renin concentration (P = 0.02). They tended to have higher systolic blood pressure levels (P = 0.07), a lower frequency of contralateral renal artery stenosis (P = 0.09) and a longer contralateral kidney (P = 0.09). In multivariate logistic regression, the factors independently linked to proteinuria in the upper tertile were active renin concentration (P = 0.05) and contralateral kidney length (P = 0.02). Proteinuria significantly decreased in nephrotic patients (P < 0.01) treated with revascularization or nephrectomy and/or angiotensin converting enzyme inhibition. CONCLUSIONS: Proteinuria in renal artery occlusion is positively related to active renin concentration, which reflects plasma angiotensin II concentration. Therapy aimed at lowering angiotensin II levels decreased proteinuria in nephrotic patients. The positive relationship between proteinuria and contralateral kidney length may reflect compensatory hypertrophy in response to nephron function loss.