A importância do diagnóstico precoce da hipertensão maligna: relato de caso / The importance of early diagnosis of malignant hypertension - case report

A hipertensão maligna é uma síndrome constituída por hipertensão arterial grave, retinopatia com papiledema (com ou sem insuficiência renal) e necrose fibrinóide de arteríolas renais, a qual pode apresentar evolução clínica rapidamente progressiva e fatal.Nela ocorrem lesões vasculares que consistem predominantemente de proliferação miointimal e necrose fibrinóide arteriolar, as quais podem se desenvolver agudamente e comprometer o lúmen dos pequenos vasos. O prognóstico da hipertensão maligna é quase sempre fatal se não for reconhecida ou não for tratada adequadamente, apresentando uma mortalidade de cerca de 80% em dois anos, principalmente em decorrência da evolução para síndrome de insuficiência cardíaca e insuficiência renal terminal.

Malignant hypertension is a syndrome consisting of severe arterial hypertension, retinopathy with papilledema (with or without renal failure) and fibrinoid necrosis of renal arterioles, which may present a rapidly progressive and fatal clinical course. In this pathology may occur vascular lesions that consist mainly of myointimal proliferation and arteriolar fibrinoid necrosis, which can develop acutely and compromise the light from the small blood vessels. The prognosis of malignant hypertension is almost always fatal if it is not recognized or not adequately treated, with a mortality rate of about 80% in 2 years, mainly as a result of progression into heart failure syndrome and end-stage renal failure syndrome.

A importância do diagnóstico precoce da hipertensão maligna: Relato de caso / The importance of early diagnosis of malignant hypertension - Case report

A hipertensão maligna é uma síndrome constituída por hipertensão arterial grave, retinopatia com papiledema (com ou sem insuficiência renal) e necrose fibrinóide de arteríolas renais, a qual pode apresentar evolução clínica rapidamente progressiva e fatal.Nela ocorrem lesões vasculares que consistem predominantemente de proliferação miointimal e necrose fibrinóide arteriolar, as quais podem se desenvolver agudamente e comprometer o lúmen dos pequenos vasos. O prognóstico da hipertensão maligna é quase sempre fatal se não for reconhecida ou não for tratada adequadamente, apresentando uma mortalidade de cerca de 80% em dois anos, principalmente em decorrência da evolução para síndrome de insuficiência cardíaca e insuficiência renal terminal. (AU).

Malignant hypertension is a syndrome consisting of severe arterial hypertension, retinopathy with papilledema (with or without renal failure) and fibrinoid necrosis of renal arterioles, which may present a rapidly progressive and fatal clinical course. In this pathology may occur vascular lesions that consist mainly of myointimal proliferation and arteriolar fibrinoid necrosis, which can develop acutely and compromise the light from the small blood vessels. The prognosis of malignant hypertension is almost always fatal if it is not recognized or not adequately treated, with a mortality rate of about 80% in 2 years, mainly as a result of progression into heart failure syndrome and end-stage renal failure syndrome (AU).

Role of complement activation in the pathogenesis of severe cardiorenal injury in patients with primary malignant hypertension / 中华肾脏病杂志

Objective:To investigate the role of complement activation in the pathogenesis of primary malignant hypertension (MHT) with nephrosclerosis complicated with severe cardiorenal injury.Methods:Data of MHT patients with nephrosclerosis proven by biopsy from January 2010 to December 2020 in the Beijing Anzhen Hospital, Capital Medical University were retrospectively analyzed. The expressions of complement-related component C4d, C1q, complement factor H-related protein 5, C3c and C5b-9 were detected by immunohistochemical staining. According to whether the patients were complicated with acute heart failure (AHF) and/or acute kidney injury (AKI), they were divided into severe cardiorenal injury group and non-severe cardiorenal injury group. The differences of clinicopathological data between the two groups were compared. According to the degree of C4d deposition in renal tissues, patients were divided into C4d diffused deposition group and non-C4d diffused deposition group. The severity of cardiorenal injury and the pathological characteristics of thrombotic microangiopathy in renal tissues were compared between the two groups.Results:A total of 33 patients were enrolled in this study, of which 17 cases (51.5%) were complicated with severe cardiorenal injury; AHF occurred in 16 patients (48.5%), AKI occurred in 8 patients (26.7%), and AHF and AKI were combined in 7 patients (21.2%). Compared with non-severe cardiorenal injury group, patients in severe cardiorenal injury group had higher levels of baseline lactate dehydrogenase [326.0 (217.0, 366.0) IU/L vs 197.0 (165.0, 220.0) IU/L, Z=37.000, P=0.002] and hemoglobin [(143.6±24.0) g/L vs (106.4±24.7) g/L, t=38.500, P<0.001], lower levels of 12 h urinary incontinence osmolality [400.0 (342.5, 504.0) mmol/L vs 476.0 (432.3, 616.5) mmol/L, Z=72.000, P=0.021] and serum albumin [(36.2±9.4) g/L vs (43.2±6.2) g/L, t=6.423, P=0.017], and thicker left ventricular posterior wall [(14.0±2.1) mm vs (12.1±1.1) mm, t=6.552, P=0.018]. The immunohistochemical results of kidney tissue showed that the proportions of C4d and C5b-9 diffused deposition in severe cardiorenal injury group were significantly higher than those in non-severe cardiorenal injury group (5/16 vs 0/15, P=0.043; 12/16 vs 5/15, P=0.032). Compared with non-C4d diffused deposition group, C4d diffused deposition group had higher incidence of AHF (5/5 vs 10/26, P=0.018), poorer heart function, more severe ventricular remodeling, and shorter history of hypertension [2.0 (0, 12.0) months vs 48.0 (9.5, 84.0) months, Z=22.500, P=0.022]. Conclusions:The incidence of severe cardiorenal injury in MHT patients with nephrosclerosis is about 51.5%. The proportion of diffuse deposition of complement activated components in renal tissues in patients with severe cardiorenal injury is higher than that in patients with non-severe cardiorenal injury. Overactivation of complement may be involved in the pathogenic process of severe heart and kidney injury caused by MHT.

Role of alternative complement pathway overactivation in malignant nephrosclerosis / 中华肾脏病杂志

Objective To study the role of alternative complement pathway overactivation in malignant nephrosclerosis.Methods (1) Fifty patients with confirmed malignant nephrosclerosis by renal needle biopsy were enrolled.Meanwhile,twenty-five cases of time-zero renal needle biopsy were enrolled as control subjects.Enzyme linked immunosorbent assay (ELISA) was used to detect alternative complement pathway of the complement initiation factor B,positive regulation factor P,negative regulation factor H,and the complement end products C3a and C5a in the plasma and urine.(2) Immunohistochemistry was used to detect the deposition of the complement end product C5b-9,C4d and mannan binding lectin (MBL) of lectin pathway in the renal biopsies.Double immunofluorescence labeling method was used to assay the deposition of C5b-9 and CD34 (endothelial cell marker) in the arteriolar endothelium and glomerular capillary endothelium.Results (1) The plasma and urine levels of complement factor B,factor P,C3a and C5a in malignant nephrosclerosis patients were significantly higher than those in control subjects (all P ＜ 0.05),while the plasma and urine levels of complement factor H in malignant nephrosclerosis patients were lower than those in control subjects (all P ＜ 0.05).(2) The plasma level of factor P was positively correlated with 24 h urine protein (rs=0.465,P=0.001).Urinary factor B/urinary creatinine,urinary factor P/urinary creatinine and urinary C3a/urinary creatinine were positively correlated with serum creatinine in malignant nephrosclerosis patients (rs=0.483,P ＜ 0.001;rs=0.352,P=0.012;rs=0.319,P=0.024),while urinary factor H/urinary creatinine was negatively correlated with serum creatinine and 24 h urine protein (rs=-0.299,P=0.035;rs=-0.342,P=0.015).Urinary C5a/urinary creatinine was positively correlated with serum creatinine and 24 h urine protein (rs=0.525,P ＜ 0.001;rs=0.496,P ＜ 0.001).(3) Immunohistochemical results showed that there were C5b-9 deposited in the arterioles and glomerular capillary wall in malignant nephrosclerosis patients,and no deposition in control renal tissues.Meanwhile,the semi-quantitative scores showed that C5b-9 deposition intensity was positively correlated with serum creatinine and 24 h urine protein (rs=0.791,P＜ 0.001;rs=0.345,P=0.014).The double immunofluorescence labeling analysis showed that the C5b-9 and CD34 deposited in the arteriolar endothelium and glomerular capillary endothelium.(4) Plasma level of factor B in malignant nephrosclerosis patients was positively correlated with plasma C3a level (r=0.331,P=0.022).Plasma level of factor P was positively correlated with C5b-9 score (rs=0.300,P=0.034).Urinary B was positively correlated with urinary C3a,C5a and C5b-9 score (rs=0.311,P=0.028;rs=0.465,P=0.001;rs=0.428,P=0.002).Urinary factor P was also positively correlated with urinary C3a and C5a (rs=0.307,P=0.030;rs=0.442,P=0.001).Immunohistochemical result showed that there were C4d deposited in the arterioles and glomerular,and no deposition of MBL.Conclusion Complement activation via the alternative pathway may be involved in malignant nephrosclerosis and related to the severity of the disease.

Role of alternative complement pathway overactivation in malignant nephrosclerosis / 中华肾脏病杂志

Objective@#To study the role of alternative complement pathway overactivation in malignant nephrosclerosis.@*Methods@#(1) Fifty patients with confirmed malignant nephrosclerosis by renal needle biopsy were enrolled. Meanwhile, twenty-five cases of time-zero renal needle biopsy were enrolled as control subjects. Enzyme linked immunosorbent assay (ELISA) was used to detect alternative complement pathway of the complement initiation factor B, positive regulation factor P, negative regulation factor H, and the complement end products C3a and C5a in the plasma and urine. (2) Immunohistochemistry was used to detect the deposition of the complement end product C5b-9, C4d and mannan binding lectin (MBL) of lectin pathway in the renal biopsies. Double immunofluorescence labeling method was used to assay the deposition of C5b-9 and CD34 (endothelial cell marker) in the arteriolar endothelium and glomerular capillary endothelium.@*Results@#(1) The plasma and urine levels of complement factor B, factor P, C3a and C5a in malignant nephrosclerosis patients were significantly higher than those in control subjects (all P<0.05), while the plasma and urine levels of complement factor H in malignant nephrosclerosis patients were lower than those in control subjects (all P<0.05). (2) The plasma level of factor P was positively correlated with 24 h urine protein (rs=0.465, P=0.001). Urinary factor B/urinary creatinine, urinary factor P/urinary creatinine and urinary C3a/urinary creatinine were positively correlated with serum creatinine in malignant nephrosclerosis patients (rs=0.483, P<0.001; rs=0.352, P=0.012; rs=0.319, P=0.024), while urinary factor H/urinary creatinine was negatively correlated with serum creatinine and 24 h urine protein (rs=-0.299, P=0.035; rs=-0.342, P=0.015). Urinary C5a/urinary creatinine was positively correlated with serum creatinine and 24 h urine protein (rs=0.525, P<0.001; rs=0.496, P<0.001). (3) Immunohistochemical results showed that there were C5b-9 deposited in the arterioles and glomerular capillary wall in malignant nephrosclerosis patients, and no deposition in control renal tissues. Meanwhile, the semi-quantitative scores showed that C5b-9 deposition intensity was positively correlated with serum creatinine and 24 h urine protein (rs=0.791, P<0.001; rs=0.345, P=0.014). The double immunofluorescence labeling analysis showed that the C5b-9 and CD34 deposited in the arteriolar endothelium and glomerular capillary endothelium. (4) Plasma level of factor B in malignant nephrosclerosis patients was positively correlated with plasma C3a level (r=0.331, P=0.022). Plasma level of factor P was positively correlated with C5b-9 score (rs=0.300, P=0.034). Urinary B was positively correlated with urinary C3a, C5a and C5b-9 score (rs=0.311, P=0.028; rs=0.465, P=0.001; rs=0.428, P=0.002). Urinary factor P was also positively correlated with urinary C3a and C5a (rs=0.307, P=0.030; rs=0.442, P=0.001). Immunohistochemical result showed that there were C4d deposited in the arterioles and glomerular, and no deposition of MBL.@*Conclusion@#Complement activation via the alternative pathway may be involved in malignant nephrosclerosis and related to the severity of the disease.

Malignant Hypertension with Pulmonary Alveolar Hemorrhage Needing Dialysis / 이화의대지

A 35-year-old man presented with progressive dyspnea and hemoptysis. His blood pressure was 230/140 mmHg and serum creatinine level was 20.13 mg/dL. Chest radiography and computed tomography revealed pulmonary hemorrhage. His renal function was low, thus emergent renal replacement therapy was required. Malignant hypertension and acute kidney injury were diagnosed, and antihypertensive therapy and hemodialysis started immediately. Renal biopsy was performed to examine the underlying disease. Typical pathological changes of malignant hypertension, fibrinoid necrosis of the afferent arterioles, and proliferative endoarteritis at the interlobular arteries were observed. His renal function improved gradually and pulmonary hemorrhage completely disappeared with administration of antihypertensive agents. Here, we report this rare case of malignant hypertension with pulmonary alveolar hemorrhage and speculate that the hemorrhage may be related to vascular injuries at the alveolar capillary level caused by malignant hypertension.

Hipertensão acelerada-maligna / Malignant hypertension

A hipertensão maligna é uma síndrome constituída por hipertensão arterial grave, retinopatia com papiledema, com ou sem insuficiência renal, necrose fibrinoide de arteríolas renais e que pode apresentar evolução clínica rapidamente progressiva e fatal. Ocorrem lesões vasculares que consistem predominantemente de proliferação miointimal e necrose fibrinóoide arteriolar, as quais podem se desenvolver agudamente e comprometer o lúmen dos pequenos vasos. O prognóstico da hipertensão maligna é quase sempre fatal se esta não for reconhecida ou não tratada adequadamente, com uma mortalidade de cerca de 80% em 2anos, principalmente em decorrência de insuficiência cardíaca e insuficiência renal terminal.

Malignant hypertension is a syndrome composed of severe hypertension, retinopathy with papilledema, with or without renal failure, renal arteriolar fibrinoid necrosis, and can provide rapidly progressive and fatal clinical outcome. Vascular lesions can occur, predominantly consisting of myointimal proliferation and arteriolar fibrinoid necrosis, which can develop sharply and compromise the lumen of the small vessels. The prognosis of malignant hypertension is almost always fatal if unrecognized and not properly treated, with a mortality rate of about 80% in 2 years, mainly due to heart failure and terminal renal failure.

A Case of Systemic Lupus Erythematosus Presenting as Malignant Hypertension with Hypertensive Retinopathy

The variability of cardiovascular abnormalities is one of the characteristics of systemic lupus erythematosus (SLE). Among the cardiovascular manifestations, hypertension is reported in 14% to 58.1% of patients in diverse ethnic populations, and remains a clinically important issue due to its close relationship with early mortality in patients with SLE. The development of hypertension in patients with SLE has been associated with advanced lupus-related renal disease and the medications used for the treatment of lupus. Malignant hypertension is a serious complication of hypertension; it rarely occurs in patients with SLE. However, it can occur in patients with other complicated medical conditions such as the antiphospholipid antibody syndrome (APS) or cardiac tamponade. Here, we report the case of a patient with SLE and malignant hypertension with hypertensive retinopathy that initially presented without clinical evidence of APS or hypertensive nephropathy.

Clinicopathological features of IgA nephropathy associated with malignant hypertension and their correlation to renal vascular lesions / 中华肾脏病杂志

Objective To explore the clinicopathological features of IgA nephrolpathy associated with malignant hypertension (IgAN-MHT) and to analyze their correlation with renal vascular lesions. Methods Twenty-nine patients of IgAN-MHT were screened from 2000 biopsy-proven eases with primary IgA nephropathy (IgAN) in our department from April 1997 to May 2007. Data of clinicopathology and follow-up of these 29 patients were collected. Semi- quantitative analysis was performed to evaluate the pathological changes. Inner lumen, outer lumen, intimal thickness, tunica media-to-internal lumen ratio of 436 arterioles, 124 interlobular arteries and 5 arcuate arteries were measured. The primary endpeint was the composite of a doubling of serum creatinine level and ESRD. Correlations of renal vascular lesions with clinical manifestation, pathological change and prognosis were examined by Spearman and Cox methods. Results 1.5% of all the IgAN patients presented malignant hypertension. The common clinical features were renal failure (100%), hyperurieacidemia (62.7%) and hypertriglyceridemia (51.7%). The average amount of urine protein excretion was 2.8 g/d. The common pathological changes were moderate mesangial proliferation, severe global sclerosis, severe interstitial inflammation and severe interstitial- tubular fibrosis. The small arteries (arcuate arteries and interlobular arteries) and arterioles (afferent arterioles) were both involved in IgAN-MHT. The characteristic lesions of intrarenal arteries included vascular occlusion, media thickening, proliferative endarteritis (onionskin lesion, musculomucoid intimal hyperplasia), hyaline arteriosclerosis, but mainly vascular occlusion (86.2%). The arteriole lesion was negatively correlated with age and total protein level; vascular occlusion was positively correlated with uric acid level. The average foUow-up period was 21.1 months. Forteen patients reached the endpoint. The arteriole lesion was the main independent risk factor for the progression of IgAN-MHT (RR=10.21, 95%CI=1.16～89.67). Conclusions The main clinical feature of IgAN-MHT is renal failure. The main histological feature of intrarenal vascular lesions is occludes arterioles. Arteriole lesion is the main independent risk factor for the progression of IgAN-MHT.

Tratamento percutâneo da hipertensão renovascular: relato de caso / Percutaneous treatment of renovascular hypertension: case record

A doença renovascular decorre da estenose uni ou bilateral da artéria renal ou um de seus ramos. Causa hipertensão arterial secundária em crianças e adultos. Será relatado caso de mulher jovem com doença fibrodisplásica e hipertensão renovascular submetida a angioplastia com cateter balão que, treze anos depois, retorna com níveis pressóricos elevados. Angiograficamente constatou-se lesão arterial renal contra-lateral e realizou-se nova intervenção percutânea com stent. Após o procedimento, evoluiu com redução e estabilização das cifras tensionais na fase hospitalar sem uso de medicamentos. O presente artigo relata o caso e revisa etiologia, incidência, manifestações clínicas, diagnóstico e tratamento.

The renovascular disease results of a unit or bilateral renal stenosis or arterial ramification. It's one of the causes of secondary hypertension in both children and adults. It reports a case of renovascular hypertension due to fibromuscular dysplasia in a young female patient treated with angioplasty, who, thirteen years later, returns with high blood levels. Then,she was submitted to percutaneous intervention with stent in treatment of opponent renal artery. The patient has evolved with normal blood pressure, while in the hospital without pharmacological therapy. This article describes a case of renovascular hypertension and its etiology, incidence, clinical presentation, diagnosis and treatment are reviewed.

Clinical features and prognostic factors of chronic glomerulonephritis with malignant hypertension / 中华肾脏病杂志

Objective To find out the clinical characteristics and prognostic risk factors of malignant hypertension (MHT) with pre existed chronic glomerulonephritis.Method The clinical and renal pathological data of 38 chronic glomerulonephritis patients with MHT hospitalized in our department from 1990 to 2002,and the associated factors for renal prognosis were analyzed. Results All the patients suffered from chronic glomerulonephritis.The ratio of male to female was 3 75∶1.The average age was (29 5?7 74)years old. The rates of recognition, therapy and control of hypertension before MHT onset were 75 7%, 13 5%and 5 4%respectively. IgA nephropathy was the most common pathological type.Of 38 cases,3 maintained normal renal function, 6 presented acute renal failure,13 presented chronic renal failure, and 16 had acute attack based on chronic renal failure. Twenty four patients were followed up for 1～6 months after antihypertensive therapy and renal function of 10(41 7%) was improved. 1 year renal survival rate of 20 patients was 55%. The analysis of risk factors showed that those patients who were possessed of positive hypertension family history(P=0 03), high SBP/DBP levels(P=0 023,0 047), and high scores of chronic index of renal pathology(P=0 032) had worse prognosis. The level of highest serum creatinine and the scores of chronic index of renal pathology were associated with 1 year renal survival(P=0 031,0 037).Conclusions MHT occuring in young patients with chronic glomerulonephritis is not rare, which becomes an important reason inducing rapidly decrease of renal function, especially in patients with IgA nephropathy. Actively antihypertensive therapy is helpful for renal function recovery. The low rates of recognition, therapy and control of pre existed renal hypertension may play key roles in the development of MHT. It is important to recognize and treat the hypertension at early stage of renal disease to improve the renal survival.