Scleroderma renal crisis.

Scleroderma renal crisis (SRC) is a rare but life-threatening complication of systemic sclerosis (SSc) characterized by malignant hypertension and acute kidney injury. Historically, SRC was the leading cause of death in SSc. However, with the advent of angiotensin converting enzyme (ACE) inhibitors, mortality rates have decreased significantly. Nevertheless, one-year outcomes remain poor, with over 30% mortality and 25% of patients remaining dialysis-dependent. There is an urgent need to improve early recognition and treatment, and to identify novel treatments to improve outcomes of SRC. In this chapter, the clinical features, classification, pathophysiology, differential diagnosis, management and outcomes of SRC are presented. Specific issues relating to pregnancy, prophylactic ACE inhibition and management of essential hypertension are also discussed.

Predictors of 5-year outcomes in malignant phase hypertension: the West Birmingham Malignant Hypertension Registry.

OBJECTIVE: Malignant hypertension represents a high-risk condition and there are scarce data on current clinical patterns of this condition. The aim of the study is to identify the clinical and demographic factors associated with poor outcome. METHODS: The data collected from 1958 to May 2016 included a total of 351 patients whose 5-year survival status was known: 221 white Caucasians (63%, age 51 ±â€Š13 years, 64% male), 83 African-Caribbeans (24%, 45 ±â€Š11 years, 61% male), and 47 South Asians (13%, 42 ±â€Š11 years, 74% male). RESULTS: During the 5-year follow-up 119 (34%) patients suffered a primary outcome, defined as the composite endpoint of death or dialysis. The 5-year mortality ranged from 76% in patients diagnosed before 1967 to 7% in patients diagnosed between 1997 and 2006. The independent predictors of outcome were advanced age (vs. a reference group of < 40-year-old; P = 0.01 for age at presentation 51-60 years, P < 0.001 for age > 60 years), prior use of antihypertensive medications (P = 0.002), higher serum creatinine (P = 0.006), and proteinuria (P < 0.01). Also, white Caucasian (odds ratio12.02, 95% confidence interval 1.64-88.15, P = 0.01) and African-Caribbean (odds ratio 15.55, 95% confidence interval 2.06-117.29, P = 0.008) origins were associated with higher mortality vs. South Asians. The years of the diagnosis after 1977 were significantly associated with lower composite endpoint of death or dialysis, all P < 0.01. CONCLUSION: There has been a major improvement in 5-year survival in patients with malignant hypertension over recent decades. Abnormal renal function at presentation still predicts worse outcome. South Asian ethnicity is also associated with better outcome, although mechanisms involved are yet to be established.

Incidence, aetiology and mortality secondary to hypertensive emergencies in a large-scale referral centre in Israel (1991-2010).

Hypertensive emergency (HE) is a life-threatening condition that requires immediate blood pressure (BP) reduction. Although it has been on the decline, the incidence of HE has recently increased in a few countries. The aim of the present retrospective study was to evaluate the incidence, aetiology and 1-year mortality of HE in a large medical centre over a 20-year period (1991-2010). The electronic medical records of all patient files who were hospitalized in the Chaim Sheba Medical Center in Israel from 1991 to 2010 with a primary diagnosis (at admission or discharge) of Malignant Hypertension, Hypertensive Emergency or Accelerated Hypertension were retrieved and analysed. The study interval was divided into four periods of 5 years each. Among 306 files reviewed, only 142 patients had a true HE. Average age at presentation was 63.3±16.5 years. Men were younger than women (59±16 vs 68±16 years; P<0.001). At presentation, most patients (80.3%) had been diagnosed with essential hypertension previously and were undertreated. Average maximum mean arterial pressure (MAP) was higher in men (169±22 mm Hg) than in women (161±17 mm Hg; P=0.026). The rate of HE decreased over the course of the study, from 12.7/100 000 admissions during 1991-1995 to 6.2/100 000 admissions (2006-2010). Similarly, 1-year mortality decreased from 16.7 to 3.6%. The rate of HE has decreased and the prognosis has improved over the last two decades. Appropriate BP control of patients with essential hypertension may further decrease the risk of HE.

Mortality and cardiovascular risk in patients with a history of malignant hypertension: a case-control study.

The survival of patients with malignant hypertension (MHT) has considerably improved over the past decades. Data regarding the excess risk of mortality and the contribution of conventional cardiovascular risk factors are lacking. The authors retrospectively assessed cardiovascular risk factors and all-cause mortality in 120 patients with a history of MHT and compared them with 120 normotensive and 120 hypertensive age-, sex-, and ethnicity-matched controls. Total cholesterol, low-density lipoprotein cholesterol, and body mass index were lower in MHT patients compared with hypertensive controls, whereas blood pressure, high-density lipoprotein cholesterol, and smoking habit were similar. Median estimated glomerular filtration rate was lower in MHT patients compared with normotensive and hypertensive controls (both P<.01). The annual incidence of all-cause mortality per 100 patient-years was higher in MHT patients (2.6) compared with normotensive (0.2) and hypertensive (0.5) controls (both P<.01). Mortality of patients with a history of MHT remains high compared with normotensive and hypertensive controls. Patients with MHT had a more favorable cardiovascular risk profile compared with hypertensive controls but a higher prevalence of renal insufficiency.

Crise hipertensiva, pseudocrise hipertensiva e elevação sintomática da pressão arterial / Hypertensive crisis, pseudo-hypertensive crisis and symptomatic increase in blood pressure

Fundamentos: Nas elevações agudas da hipertensão arterial sistêmica (HAS), a intervenção imediata e cuidadosa é essencial para a redução da morbidade e da mortalidade decorrentes dessa complicação.Objetivos: Identificar a frequência de urgência hipertensiva (UH), emergência hipertensiva (EH), pseudocrise hipertensiva (PCH) e elevação sintomática da pressão arterial (ESPA). Comparar o conhecimento prévio da HAS, o uso prévio de anti-hipertensivos (AH), os níveis pressóricos apresentados e os desfechos hospitalares nos grupos estudados. Métodos: Estudo analítico, casos e controles, seleção consecutiva e análise retrospectiva de pacientes com elevação aguda da pressão arterial, admitidos na emergência de hospital cardiológico de atendimento privado, entre 11/2009 e 10/2010. Casos representam os pacientes com crise hipertensiva (CH): UH+EH. Controles representam os pacientes sem CH: PCH+ESPA. Resultados: Foram estudados 216 atendimentos relacionados à HAS, 113 (52,0 %) mulheres, idade entre 25-95 anos, mediana de 58 anos. EH foi diagnosticada em 18 (8,0 %) pacientes, UH em 29 (13,0 %), PCH em 8 (4,0 %) e ESPA em 161 (75,0 %). Diagnóstico e tratamento prévio de HAS não diferiram nos grupos com e sem CH. Sintomas cardiovasculares, prescrição de AH e internação foram mais frequentes naqueles com CH (p<0,05); mas apenas 7,0 % dos pacientes não receberam AH. Não houve óbitos.Conclusões: Na população estudada, CH foi identificada em 21,0 % dos casos e o tratamento AH foi aplicado em 93,0 % dos casos. O diagnóstico de HAS e tratamento AH prévio não diferiram naqueles pacientes com e sem CH.

Background: For systemic high blood pressure (SHBP) surges, immediate and careful intervention is essential for reducing morbidity and mortality rates related to this complication.Objectives: To identify the frequency of hypertensive urgency (HU), hypertensive emergency (HE), pseudo-hypertensive crisis (PHC) and symptomatic blood pressure increase (SBPI). Compare prior knowledge of SHBP, previous use of anti-hypertensive (AH), pressure levels presented and hospital outcomes in both groups. Methods: Analytical, case and control study with consecutive selection and retrospective analysis of patients with acute increase in blood pressure, admitted to the emergency cardiac care unit at a private hospital, between November 2009 and October 2010. The cases were patients with hypertensive crisis (HC): HU + HE. The controls were patients without HC: PHC + SBPI. Results: A total of 216 cases related to SHBP were studied, consisting of 113 (52 %) women between 25 and 95 years old, with a median age of 58 years. HE was diagnosed in 18 (8 %) patients, HU in 29 (13 %), PHC in 8 (4 %) and SBPI in 161 (75 %). Prior diagnosis and treatment of SHBP did not differ between the groups with and without HC. Cardiovascular symptoms, hospitalization and AH prescriptions were more frequent among those with HC (p <0.05), but only 7 % of the patients did not receive AH. There were no deaths. Conclusions: In this population studied, HC was identified in 21 % of the cases and AH treatment was administered in 93 % of cases diagnosed with SHBP. Prior AH treatment did not differ among patients with and without HC.

Clinical and pathological evaluation of hypertensive emergency-related nephropathy.

OBJECTIVE: The prognosis of patients with hypertensive emergencies has recently improved dramatically owing to the development of effective antihypertensive therapy. We examined the histological and clinical features of patients with hypertensive emergency-related nephropathy. METHODS: Twelve patients (11 men and one woman) were diagnosed as having hypertensive emergencies with acute renal failure according to the Joint National Committee-7 classification of blood pressure for adults and underwent renal biopsies at our hospital between 1995 and 2008. These patients were enrolled in this retrospective study. RESULTS: The age of the subjects was 40.1±9.8 years. At presentation, the mean systolic/diastolic blood pressure was 232±32/146±12 mmHg and none of the patients were being treated with antihypertensive drugs, although 10 patients had histories of hypertension. The mean serum creatinine level was 6.1±4.7 mg/dL. All 12 patients showed left ventricular hypertrophy on echocardiography. On light microscopy of the renal biopsy specimens, all 12 patients showed onion skin patterns of the arterioles; however, no fibrinoid necrosis of the small arteries was found. Electron microscopy revealed electron-lucent widening of the subendothelial zone of the glomerular capillary walls in seven patients. One of the 12 patients did not respond to medical therapy and required regular dialysis. The other 11 patients responded to treatment. CONCLUSION: An onion skin pattern of the arterioles is the most frequent histological finding in patients with hypertensive emergency-related nephropathy. Long-standing hypertension might contribute to this arteriolar change, since left ventricular hypertrophy was also seen in these patients. With strict control of hypertension using antihypertensive medications, the prognosis of patients with hypertensive emergency-related nephropathy can be improved.

Long-term renal outcome in patients with malignant hypertension: a retrospective cohort study.

BACKGROUND: Malignant hypertension is frequently complicated by renal insufficiency. Although the survival of this hypertensive emergency has improved, recent data on renal outcome and its predictors are lacking. We assessed renal outcome and its predictors in patients with malignant hypertension. METHODS: Retrospective analysis of patients admitted with malignant hypertension in Amsterdam, the Netherlands between August 1992-January 2010. Follow-up data on vital status, renal function and blood pressure (BP) were obtained from the outpatient department and from general practitioners. The primary composite endpoint was end-stage renal disease (ESRD) defined as the start of kidney replacement therapy (KRT) or ≥ 50% decline of estimated glomerular filtration rate (eGFR). The secondary endpoint was all cause mortality. RESULTS: A total of 120 patients admitted with malignant hypertension were included. After a median follow-up period of 67 months (IQR 28 to 108 months) the primary endpoint was reached by 37 (31%) patients, whereas 18 patients (15%) reached the secondary endpoint. Twenty-nine (24%) patients started KRT and 8 patients (7%) had an eGFR decline ≥ 50%. After the acute phase (> 3 months after admission), initial serum creatinine and follow-up BP were the main predictors of future ESRD with hazard ratios of 6.1 (95% CI, 2.2-17) for patients with initial serum creatinine ≥ 175 µmol /L and 4.3 (95% CI, 1.4-14) for patients with uncontrolled hypertension. CONCLUSIONS: Progressive renal function decline leading to ESRD remains a major threat to patients with malignant hypertension. BP control during follow-up was an important modifiable predictor of renal outcome.

Cranial decompression for the treatment of malignant intracranial hypertension after ischemic cerebral infarction: decompressive craniectomy and hinge craniotomy.

OBJECT: Recent randomized trials have demonstrated a positive role (improved survival) in patients treated with cranial decompression for malignant cerebral infarction. However, many variables regarding operative decompression in this setting remain to be determined. Hinge craniotomy is an alternative to decompressive craniectomy, but its role in space-occupying cerebral infarctions has not been delineated. The objective of this study was to compare the authors' experiences with these 2 procedures in the management of space-occupying cerebral infarctions to determine the efficacy of each. METHODS: The authors conducted a retrospective review of 28 cases involving patients who underwent cranial decompression (hinge craniotomy in 9 cases, decompressive craniectomy in 19) for treatment of malignant intracranial hypertension after ischemic cerebral infarction. RESULTS: No significant differences were identified in baseline demographics, neurological examination, or Rotterdam score between the hinge craniotomy and decompressive craniectomy groups. Both treatments resulted in adequate control of intracranial pressure (ICP). The need for reoperation for persistent intracranial hypertension and duration of mechanical ventilation and intensive care unit stay were similar. Hospital survival was significantly higher in the decompressive craniectomy group (89% vs 56%), whereas long-term functional outcome was better in the hinge craniotomy group. Cranial defect size was comparable in the 2 groups. Postoperative imaging revealed a higher rate of subarachnoid hemorrhage, contusion/hematoma progression, and subdural effusions/hygromas after decompressive craniectomy. The requirement for cranial revision in survivors was higher for patients undergoing decompressive craniectomy (100%) than those undergoing hinge craniotomy (20%). CONCLUSIONS: Hinge craniotomy appears to be at least as good as decompressive craniectomy in providing postoperative ICP control at a similar therapeutic index. Although the in-hospital mortality was higher in patients treated with hinge craniotomy, that procedure resulted in superior long-term functional outcomes and may help limit postoperative complications.

Long-term renal survival in malignant hypertension.

BACKGROUND: Some studies have shown an improvement in the prognosis of patients with essential malignant hypertension (MHT), but data about long-term outcome and prognostic factors of these patients are scarce. METHODS: We performed a single-centre retrospective analysis of 197 patients with MHT, diagnosed in the period 1974-2007. RESULTS: Incidence of MHT remained stable along the different periods of the study. Renal damage at presentation was common (63% of patients) but renal function improved or remained stable after diagnosis in a majority of patients. The probability of renal survival was 84 and 72% after 5 and 10 years, respectively. Diagnosis during the first period (1974-85) of the study, previous chronic renal impairment, baseline renal function and proteinuria, presence of microhaematuria, systolic and diastolic blood pressure and proteinuria during follow-up were associated with an unfavourable outcome. By multivariate analysis, mean proteinuria during follow-up remained as the only significant risk factor (OR, 2.72; 95% CI, 1.59-4.64). Renal survival for patients with mean proteinuria <0.5 g/24 h was 100 and 95% after 5 and 10 years, respectively. The number of patients who improved or stabilized their renal function significantly increased in the second and third periods of the study (1987-2007). CONCLUSIONS: Renal survival in MHT has improved in recent years. Mean proteinuria during follow-up is a fundamental prognostic factor for renal survival.

Improving survival of malignant hypertension patients over 40 years.

BACKGROUND: To examine changing demography and survival of patients with malignant phase hypertension (MHT) over 40 years. METHODS: Patients from our MHT registry whose survival status on 31 December 2006 was known were included, with analyses conducted based on decade of MHT diagnosis. RESULTS: Four-hundred and forty-six patients with MHT (overall mean (s.d.) age 48.2 (12.9), years; 65.5% male; 64.7% white-European; 20.4% African Caribbean, and 14.8% South-Asian) were included. No significant demographic differences at diagnosis were evident over the 40 years, with the exception of a significant increase (P = 0.001) in the proportion of MHT among ethnic minorities (South-Asian and Afro-Caribbeans). There were no significant differences in mean systolic blood pressure (SBP) at presentation but baseline diastolic BP (DBP) was significantly lower after 1976 (P < 0.0001). The total number of person-years of observation was 5,725.5 years, with a median (interquartile range (IQR)) length of follow-up of 103.8 (31.3-251.2) months. Overall 203 patients (55.6%) died, 125 (32.0%) within 5 years of diagnosis. There was a significant improvement in 5-year survival from 32.0% prior to 1977 to 91.0% for patients diagnosed between 1997 and 2006. SBP and DBP improved significantly during follow-up (P < 0.0001). Multivariate analyses revealed that age, decade of MHT diagnosis, baseline creatinine, and follow-up SBP were independent predictors of survival (all P < 0.0001). CONCLUSIONS: Demography and number of new cases of MHT have not changed dramatically over the past 40 years. Five-year post-MHT survival has improved significantly, possibly related to lower BP targets, tighter BP control, and availability of new classes of antihypertensive drugs.

Cerebral hemodynamics during treatment with sodium nitroprusside versus labetalol in malignant hypertension.

In patients with malignant hypertension, immediate blood pressure reduction is indicated to prevent further organ damage. Because cerebral autoregulatory capacity is impaired in these patients, a pharmacologically induced decline of blood pressure reduces cerebral blood flow with the danger of cerebral hypoperfusion. We compared the reduction in transcranial Doppler-determined middle cerebral artery blood velocity during blood pressure lowering with sodium nitroprusside with that of labetalol. Therefore, in 15 patients, fulfilling World Health Organization criteria for malignant hypertension, beat-to-beat mean arterial pressure, systemic vascular resistance (Modelflow), mean middle cerebral artery blood velocity, and cerebrovascular resistance index (mean blood pressure:mean middle cerebral artery blood flow velocity ratio), were monitored during treatment with sodium nitroprusside (n=8) or labetalol (n=7). The reduction in mean arterial blood pressure with sodium nitroprusside (-28+/-3%; mean+/-SEM) and labetalol (-28+/-4%) was comparable. With labetalol, both systemic and cerebral vascular resistance decreased proportionally (-13+/-10% and -17+/-5%), whereas with sodium nitroprusside, the decline in systemic vascular resistance was larger than that in cerebral vascular resistance (-53+/-4% and -7+/-4%). The rate of reduction in middle cerebral artery blood velocity was smaller with labetalol than with sodium nitroprusside (0.45+/-0.05% versus 0.78+/-0.04% cm.s(-1).%mm Hg(-1); P<0.05). In conclusion, sodium nitroprusside reduced systemic vascular resistance rather than cerebral vascular resistance with a larger rate of reduction in middle cerebral artery blood velocity, suggesting a preferential blood flow to the low resistance systemic vascular bed rather than the cerebral vascular bed.

Hypertensive crisis: hypertensive emergencies and urgencies.

Hypertensive crisis is a serious condition that is associated with end-organ damage or may result in end-organ damage if left untreated. Causes of acute rises in blood pressure include medications,noncompliance, and poorly controlled chronic hypertension. Treatment of a hypertensive crisis should be tailored to each individual based on the extent of end-organ injury and comorbid conditions. Prompt and rapid reduction of blood pressure under continuous surveillance is essential in patients who have acute end-organ damage.

Malignant hypertension in patients with idiopathic IgA nephropathy.

BACKGROUND: Idiopathic IgA nephropathy is one of the main causes of secondary malignant hypertension, especially in Chinese population. But little information has been available about malignant hypertension secondary to IgA nephropathy (IgANMHT). The purpose of this study is to evaluate the clinico-pathological features and outcomes of IgANMHT patients. METHODS: A case control retrospective study was carried out in 45 cases of IgANMHT and 26 cases of primary malignant hypertension (PMHT) diagnosed by renal biopsy. Their clinical features and pathological findings were investigated. Their average follow-up time was 37.4 months. Univariate analysis and multivariate Cox regression analysis were performed to select variables to predict renal survival. RESULTS: In the study, 1.2% of all the IgA nephropathy patients presented malignant hypertension. The amounts of urine protein excretion and red blood cells in IgANMHT patients were significantly higher, while the levels of serum creatinine were significantly lower than those in PMHT patients. The glomerular injury in IgANMHT patients was more severe than that in PMHT patients. The two characteristic vascular lesions of primary malignant hypertension, proliferative endoarteritis and fibrinoid necrosis were also found in IgANMHT patients but with less severity. Renal survival of IgANMHT patients was significantly higher than that of PMHT patients (p = 0.0043). However, log-rank test showed no significant difference in the renal survival between IgANMHT and PMHT patients with similar SCr levels at admission. Multivariate Cox regression analysis revealed that a high amount of urine protein excretion(> or =1.5 g/24 h), mesangial proliferation and elevated serum creatinine (> or =2 mg/dl) were statistically independent risk factors for renal prognosis (RR = 1.90, 2.72, 2.84, respectively). Conversely, strict blood pressure control had a favorable effect on renal prognosis. CONCLUSION: The clinico-pathological features and outcomes of IgANMHT patients were different from those of PMHT patients. The renal survival of IgANMHT patients was poor, which was determined by many factors. Early control of proteinuria, early monitoring and strictly controlling blood pressure may contribute to the renal survival.

Long-term adrenomedullin infusion improves survival in malignant hypertensive rats.

Previous studies have demonstrated that adrenomedullin has inhibitory effects on the proliferation and DNA synthesis of mesangial cells and vascular smooth muscle cells in vitro and that plasma adrenomedullin levels are markedly elevated in malignant hypertension. This study was designed to examine whether chronic adrenomedullin infusion has renoprotective effects in malignant hypertensive rats. We studied the following 3 groups: control Wistar Kyoto rats, deoxycorticosterone acetate-salt spontaneously hypertensive rats, and adrenomedullin-treated deoxycorticosterone acetate-salt spontaneously hypertensive rats. Chronic adrenomedullin infusion using an osmotic minipump was started simultaneously with deoxycorticosterone acetate-salt treatment. After 3 weeks of the treatment, malignant hypertensive rats were characterized by higher blood pressure, kidney weight, urinary protein excretion, glomerular injury score, plasma renin concentration, aldosterone level, endogenous rat plasma adrenomedullin level, renal cortical tissue angiotensin II level, angiotensin-converting enzyme mRNA level, and transforming growth factor-beta1 mRNA level in the renal cortex, and by lower creatinine clearance, compared with the control rats. Chronic adrenomedullin infusion significantly improved these parameters (kidney weight -6.5%, urinary protein excretion -63.8%, glomerular injury score -38.3%, plasma renin concentration -52.4%, aldosterone -23.2%, rat adrenomedullin -28.6%, renal angiotensin II -28.1%, renal angiotensin-converting enzyme mRNA -38.3%, renal transforming growth factor-beta1 mRNA -56.2%, and creatinine clearance +20.5%) without significant reduction of mean arterial pressure (-4%). Kaplan-Meier survival analysis showed that adrenomedullin infusion significantly prolonged survival time. These results suggest that subdepressor dose of chronic adrenomedullin infusion has renoprotective effects in this malignant hypertension model, at least in part, via inhibition of the circulating and intrarenal renin-angiotensin system.

Trends in the pathophysiological characteristics of malignant hypertension.

The aim of the present study is to investigate the pathophysiological characteristics of a number of recent cases of malignant hypertension (MHT) and to compare them to the characteristics of earlier cases. Patients with MHT (age 25-76, mean 44+/-2 years) who were admitted to our hospital from 1984-1999 were retrospectively studied. All of the patients had either grade III or IV retinopathy and diastolic blood pressure levels higher than 120 mmHg. The observations in this study were compared to previously reported findings regarding 59 MHT patients who were admitted from 1971-1983. Of the 37 recent MHT patients, 20 had essential hypertension (EHT) as the underlying disease, 13 had chronic glomerulonephritis (CGN), and the remaining 4 presented with other diseases including pyelonephritis and renovascular hypertension. A positive family history of hypertension was more prevalent in the EHT patients than in other patients, and persistent proteinuria, microhematuria, and anemia were more prevalent in the CGN patients. These characteristics were similar between the recent and previous cases. Within 4 weeks after admission, hemodialysis was initiated in 3 of the 13 patients (23%) with CGN and 2 of the 20 (10%) patients with EHT. The prevalence of renal death at 1 year after admission was 30%, which was lower than the prevalence in the previous cases (42%). Grade IV retinopathy was seen in 45% of the patients admitted from 1984-1999, significantly less than in the patients admitted from 1971-1983 (66%, p<0.05). In addition, left ventricular hypertrophy was less frequently observed on electrocardiogram in the recent cases (67%) than in the previous cases (88%, p<0.05). Our results suggest that the recent cases of MHT demonstrate less severe organ damage.

Serum urate is associated with baseline renal dysfunction but not survival or deterioration in renal function in malignant phase hypertension.

BACKGROUND: There has been speculation whether serum uric acid levels are an independent prognostic factor in patients with hypertension. OBJECTIVE: To investigate the clinical associations and prognostic value of serum urate in patients with malignant phase hypertension (MHT), by comparing clinical features in patients with serum urate levels above and below the median levels for this population, and secondly, by performing a survival analysis in these patients. PATIENTS AND METHODS: Review of the data on 153 patients (98 males; mean age 50.3 years, SD 13.5) with MHT on the west Birmingham MHT register. Median uric acid levels in this population was 0.41 mmol/l (6.9 mg/dl), with an interquartile range of 0.34-0.50 mmol/l (5.7-8.4 mg/dl). Clinical characteristics of patients with a serum urate <0.41mmol/1 (group 1) were compared to those with levels above the median (0.41 mmol/l, group 2). RESULTS: Mean duration of follow-up was similar in both groups. The mean diastolic blood pressure at presentation and both mean systolic and diastolic blood pressures at follow-up were significantly higher in group 2 (that is, those with high serum urate levels) (unpaired t test, P= 0.039). There was also more renal dysfunction in group 2 patients with MHT, with higher mean serum urea and creatinine levels, both at presentation and at follow-up (unpaired t test, P< 0.01). The commonest causes of death were myocardial infarction (n = 7), heart failure (n = 4), stroke (n = 10) and renal failure (n = 5). There was no difference in mean survival duration between groups 1 and 2 (Kaplan-Meier, 64.6 versus 66.8 months; log-rank test, P= 0.519). Serum urate levels also did not predict the rise in serum creatinine levels (log-rank test, P= 0.84) or urea (P= 0.4033) amongst these patients. Using a multivariate Cox proportional hazards analysis, the only independent predictors of outcomes (death or the need for dialysis) were age (P = 0.007) and serum creatinine levels at presentation (P = 0.0046). CONCLUSION: Our analysis of a large series of patients with MHT shows that those with high urate levels had higher diastolic blood pressures and greater renal impairment at baseline. At follow-up, patients with median serum urate >0.41 mmol/l showed a greater deterioration in renal function and higher blood pressures, but no significant difference in survival. Serum urate levels also do not appear to be predictive of the deterioration in renal function or overall survival in patients with MHT.

Outcome of patients with malignant hypertension and end-stage renal failure treated by long-term hemodialysis.

Malignant hypertension is associated with high mortality and morbidity usually caused by cardiovascular events. The course and prognosis of malignant hypertension patients treated with renal replacement therapy has not been thoroughly investigated. In the present work, we compared the clinical evolution and survival of 24 end-stage renal failure malignant hypertension patients with that of a group of individually matched renal failure patients admitted to the same dialysis center during a period of 21 years. Survival rates at 1, 5 and 8 years were 87, 82 and 50% for malignant hypertension patients and 87, 75 and 65% for controls, respectively (p = 0.766, NS). Nonfatal cardiovascular complications occurred in 2 individuals of each group. The most important cause of death in both groups was cardiovascular. The frequency of fatal cardiovascular events was similar in the two groups: 64% of deaths for malignant hypertension and 71% for controls (NS). In conclusion, previous malignant hypertension did not increase the risk of patients in long-term hemodialysis in our series.

[Scleroderma renal crisis. 7 cases and review of the literature]. / La crise rénale sclérodermique. Sept observations et revue de la littérature.

We report a series of seven patients who had scleroderma renal crisis. Their primary clinical and laboratory features along with the details of their management were compared with those of similar cases from the literature. The seven patients died within one to four months of the diagnosis with a pattern of acute renal failure, left ventricular failure and malignant hypertension. Histopathologic examination was performed in four of the patients, in two of whom it revealed thickening of the wall of the interlobular arteries related to the scleroderma, and in the other two patients nonspecific lesions of malignant hypertension. This histopathologic particularity led us to propose, on the basis of multiple renal biopsies performed in patients with scleroderma, a lesion chronology of the kidney in patients with scleroderma. Nevertheless, the triggering factors and pathophysiologic mechanisms of scleroderma renal crisis remain unclear and its prognosis is severe. Early treatment with angiotensin-converting enzyme inhibitors and other vasodilatators administered intravenously can prevent death and dialysis.

Malignant hypertension in young women is related to previous hypertension in pregnancy, not oral contraception.

Previous studies have suggested that one-third of women of childbearing age who develop malignant phase hypertension (MHT) are likely to be taking oral contraceptives (OC). We surveyed 104 women with a history of MHT. None of the 65 aged > 45 years were taking OC or other sex hormones. Thirty-nine (mean age 34.9 years, SD 8.0) were aged 15-44 years at presentation: 22 Caucasian, 10 Black/Afro-Caribbean and seven Indo-Asian. Of these 39, 22 had a history of hypertension in pregnancy (group 1), and 17 did not (group 2). Three of group 1 also had a history of OC-induced hypertension. None were pregnant, but one was taking an OC at presentation with MHT. Blood pressures at presentation and follow-up, and mean serum urea and creatinine at presentation were similar between groups, as was median survival (96 vs. 47 months, Lee-Desu statistic 0.75, p = 0.38). There was a trend towards poorer renal function at follow-up in group 1 patients, with higher mean serum urea and creatinine levels. The causes of death were renal failure (5), stroke (4) and heart disease (2). The OC was not a common cause of MHT-amongst our sample of women of childbearing age, but a past history of hypertension in pregnancy was important. Such patients also had a longer duration of hypertension and poorer renal function at follow-up.