Generation of a Core Set of Items to Develop Classification Criteria for Scleroderma Renal Crisis Using Consensus Methodology.

OBJECTIVE: To generate a core set of items to develop classification criteria for scleroderma renal crisis (SRC) using consensus methodology. METHODS: An international, multidisciplinary panel of experts was invited to participate in a 3-round Delphi exercise developed using a survey based on items identified by a scoping review. In round 1, participants were asked to identify omissions and clarify ambiguities regarding the items in the survey. In round 2, participants were asked to rate the validity and feasibility of the items using Likert-type scales ranging from 1 to 9 (where 1 = very invalid/unfeasible, 5 = uncertain, and 9 = very valid/feasible). In round 3, participants reviewed the results and comments from round 2 and were asked to provide final ratings. Items rated as highly valid and feasible (median scores ≥7 for each) in round 3 were selected as the provisional core set of items. A consensus meeting using a nominal group technique was conducted to further reduce the core set of items. RESULTS: Ninety-nine experts from 16 countries participated in the Delphi exercise. Of the 31 items in the survey, consensus was achieved on 13, in the categories hypertension, renal insufficiency, proteinuria, and hemolysis. Eleven experts took part in the nominal group technique discussion, where consensus was achieved in 5 domains: blood pressure, acute kidney injury, microangiopathic hemolytic anemia, target organ dysfunction, and renal histopathology. CONCLUSION: A core set of items that characterize SRC was identified using consensus methodology. This core set will be used in future data-driven phases of this project to develop classification criteria for SRC.

[Severe hypertension: definition and patients profiles]. / Définition et profil des patients ayant une hypertension artéirielle sévere.

Severe arterial hypertension gathers relatively different clinical situations explained by the heterogeneity of the definitions of this clinical setting. From a medical point of view, severe hypertension is a short course situation defined by very high values of blood pressure corresponding to grade 3 hypertension. In France, until 2011, the social security also included in the definition of severe HTA chronic situations characterized by moderate blood pressure values requiring at least triple anthihypertensive therapies associated with a clinical or infraclinical target organ damages. These clinical profiles, much more frequent than grade 3 hypertension, allowed the full reimbursement of care costs for these patients. In France, it is estimated that 10% of hypertensive patients present a severe form with an annual incidence of 50,000 patients. The patients with severe hypertension have an increased cardiovascular morbidity justifying a closer clinical monitoring. From an economic point of view, these severe forms of hypertension have a higher cost of care, explained primarily by a more frequent need of specialized referrals, radiological exams and hospitalizations. This excess cost justified the existence of a full coverage of induced costs by the social security, since the incidence of severe hypertension is more frequent in the low social categories, and in patients with economic fragility.

[Management of hypertension recorded as at least 180/110 mmHg]. / Prise en charge d'une hypertensìon artérielle supérieure ou égale a 180/110 mmHg.

Stage 3 hypertension (severe) is far from rare. It may be part of a previous hypertension condition which is difficult to control, or occur more acutely, in which case it will be harder for the patient to bear. When it is symptomatic and a fortiorione or more organs targeted by hypertension are affected, management must be fast and appropriate. It may take the form of a hypertensive urgency, in which case the investigations and treatment usually take place in outpatients, with oral treatment. it may also be a hypertensive emergency for which treatment involves hospitalization in an intensive care unit with intravenous anti-hypertensive treatment. A reduction in blood pressure must be obtained rapidly but not suddenly; it must be more or less significant depending on the clinical situation, and also progressive.

[Hypertensive crisis in kidney patients]. / Le crisi ipertensive nel paziente nefropatico.

The classification and management of hypertensive crisis have been recently reviewed in the context of both European and American guidelines. The key points for proper blood pressure control in severe arterial hypertension are: 1 - Distinction between urgent intervention and emergencies 2 - Choice of the best drug(s) 3 - Choice of the correct route of administration. In patients with renal disease, beside the common causes of hypertension/ hypertensive crises, kidney-specific causes should be taken into account such as renal parenchymal hypertension, renovascular hypertension, sclerodermic crises, and preeclampsia.

Hipertensäo maligna, doença de Bright e como surgiu o conceito de hipertensäo arterial essencial / Malignant hypertension, Bright's disease and how the concept of essential hypertension was origined

A hipertensäo maligna é uma complicaçäo grave da hipertensäo arterial, caracterizada por elevaçäo importante e aguda da pressäo arterial, insuficiência renal rapidamente progressiva, retinopatia com exsudatos, hemorragias e papiledema, culminando com a morte do paciente em poucos meses se näo tratada adequadamente. Foi descrita em 1914 por Volhard e Fahr, que reportaram os achados anatomopatológicos renais presentes nessa patologia, como a endarterite obliterante e a necrose fibrinóide de arteríolas renais. A relaçäo entre hipertensäo e doença renal foi sugerida pela primeira vez por Bright, em 1836, antes de ser conhecida a pressäo arterial do homem, ao descrever a cardiopatia de pacientes portadores de nefropatia grave. A partir desse estudo, diferentes autores demonstraram a provável origem vascular da doença de Bright e, em 1911, surgiu o termo hipertensäo essencial. Por muitos anos, o aumento da pressäo arterial foi considerado essencial para forçar o sangue através de artérias estreitadas pelo envelhecimento, e apenas após a metade do século XX é que se mostrou que a hipertensäo arterial, mesmo leve ou moderada, poderia causar graves complicações cardiovasculares se näo tratada. Atualmente, a hipertensäo essencial é definida como hipertensäo arterial com causa desconhecida, mas com o melhor conhecimento dos fatores ambientais e genéticos envolvidos na gênese da hipertensäo, a proporçäo de pacientes com esse diagnóstico será muito menor

Short-term course of renal function in accelerated hypertension.

Thirty-four patients with primary accelerated hypertension were studied regarding the effect of BP on renal function and the association between fundal grade and renal function before treatment; and the changes in renal function on normalization of BP in 12 patients. Mean arterial pressure was negatively correlated with serum creatinine (r = -0.44, P < 0.02) and there was no significant difference in serum creatinine or in the occurrence of renal failure between patients with grade III (n = 21) and grade IV (n = 13) retinopathy, (P = n.s. and X2 = 0.172 P = n.s. respectively). Twenty-two patients required dialysis at presentation. Serum creatinine rose in 12 other patients on reduction of BP; 7 patients with serum creatinine level < or = 3.2 mg/dL (283 umol/L) at presentation never required dialysis in the 3 months following reduction of BP while those with levels > or = 3.6 mg/dL (319 umol/L) did. In accelerated hypertension, renal function is partly maintained by the blood pressure and dialysis may be required soon after presentation or normalization of BP.

Perinatal hypertensive crisis.

Women with severe pregnancy-induced hypertension or chronic hypertension with superimposed preeclampsia are at risk for the development of hypertensive crisis. Hypertensive crisis is an emergent situation that carries great maternal and fetal morbidity and mortality. Effective assessment and comprehensive care of the patient in hypertensive crisis requires a thorough understanding of the underlying disease preeclampsia, common hemodynamic findings, and therapies available. Nurses in any perinatal environment must be prepared to respond immediately to this critical obstetrical circumstance.

Histopathology of severe renal vascular damage in blacks.

During the 1970s renal biopsies were obtained after blood pressure had been controlled in 41 black patients in Memphis who had severe hypertension plus excretory renal failure. An additional 13 binephrectomy specimens were also studied. This material yielded significant information on the state of the renal arteries--arterioles under these circumstances. Fibrinoid necrosis of the afferent glomerular arteriole and proliferative glomerulitis were not noted. Rather, the vascular lesion characterized by the accumulation of smooth muscle cells and mucopolysaccharide in the intima attended by a marked narrowing of the lumen was dominant. This lesion caused pronounced ischemia associated with obsolescence of glomeruli, atrophy, and fibrosis (end-stage kidney). This lesion has been renamed musculomucoid intimal hyperplasia as a result of changes revealed by electron microscopic and histochemical studies. Since this study the incidence of this severe vascular disease of the kidney in the same geographic area has been markedly reduced. There are a number of possible reasons for this change in incidence, but a major one appears to be improved treatment of hypertension and better compliance with antihypertensive therapy. Why such extreme changes occur in a subset of hypertensive blacks is not known. It is apparent that without improved antihypertensive treatment, this type of end-stage renal disease due to severe vascular damage will continue to be encountered.

Hypertensive emergency: case criteria, sociodemographic profile, and previous care of 100 cases.

To study the frequency, cost, sociodemographic profile, and previous care correlates of hospital admissions for hypertensive emergency, we used specific case criteria to identify a series of 100 cases at Presbyterian Hospital in New York City. Approximately 58 cases were admitted per year. Mean length of hospital stay was 11.8 days, 75 per cent of patients received intensive care, and estimated annual hospital charges were $438,828 (1986 dollars). Cases had severe hypertension on admission (mean systolic blood pressure, 229.8 mmHg; mean diastolic blood pressure, 143 mmHg). Two-thirds had clinical evidence of acute arteriolitis. Cases were predominantly young, male, Black or Hispanic, and of lower socioeconomic status. At least 93 per cent of cases were previously diagnosed, and at least 83 per cent were aware of their diagnosis of hypertension. Improved management of chronic hypertension rather than more intensive screening may be a useful strategy to reduce the incidence of hypertensive emergency.

Malignant hypertension--improving prognosis in a rare disease.

A follow-up was made of 69 patients diagnosed as having malignant hypertension during 1969--76, essential in 26, secondary in 39 and unclassified in 4. A clear male dominance was seen (41 men, 28 women), particularly in the group with essential hypertension (18 men, 8 women). The mortality in this series was less than in previously published series. Thus, the 5-year survival rate was 75% in the patients with essential and 72% in those with secondary hypertension. In part this was due to haemodialysis and renal transplantation. The importance of renal function at the time of diagnosis was evident in this study. In most patients with essential hypertension and serum creatinine levels below 300 mumol/l, renal function could be maintained or improved when antihypertensive treatment was instituted, whereas progression of the renal damage was seen in those with serum creatinine levels above 300 mumol/l in spite of antihypertensive treatment with 3 or more drugs. The incidence of new cases of malignant hypertension tended to decrease during the observation period, particularly as regards essential hypertension.