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Non-arteritic anterior ischemic optic neuropathy (NAION) is after glaucoma the most common optic neuropathy in patients over 50 years. It is known that high blood pressure (HBP) is an important risk factor for the development of NAION. It is also known that malignant arterial hypertension (MAH) could be accompanied by optic disc edema. However, MAH has not classically been considered a cause of NAION. We report the case of a 32-year-old patient who presented irreversible visual loss with a pattern compatible with NAION as the only manifestation of a hypertensive crisis., (Copyright © 2024 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

Background: Malignant hypertension has not disappeared and remains the most severe form of hypertension. More than 100 years after its description, many points remain unanswered. Mechanisms, definitions, and optimal treatment are still controversial. In 2019, we decided to launch a prospective multicentre multidisciplinary cohort in France to try to fill these gaps., Method: This study aimed to describe the baseline characteristics of the first 302 included patients and compared these data to already published cohorts. We included patients with severe hypertension associated with severe hypertensive retinopathy and patients filling the HYP MOD (HYPertension MultiOrgan Damage) definition from a broad range of departments (cardiology, nephrology, neurology intensive care unit, emergency department, internal medicine). We collected clinical, biological, imaging, and target organ damage data at admission, along with social and demographic data. We also recorded diagnostic and therapeutic management, adverse events during hospitalization, and characteristics at discharge., Results: We enrolled 302 patients in 32 months (105/year) among 40 centres and different specialties. They mainly included young men (68%, mean age 48.7 ± 14.5 years). Target organ damage involved the eye in 86.7% of patients, kidney in 58.6%, heart in 50%, brain in 32.8%, and Thrombotic Microangiopathy stigmata in 15.6%. Patients with severe retinopathy shared characteristics similar to those included in the most important cohorts already published. We also reported several additional subgroups of interest: one-third of our patients were less than 40 years old, one-third were of non-European origin, 14.3% were included through the multiorgan damage definition, without fundus severe injuries, 22.8% were treated without the use of IV therapy, 40.9% had normal or low renin level, and almost all patients were not on antihypertensive therapy at the time of the enrolment., Conclusion: These preliminary findings already challenge long-standing dogma, raise numerous questions, and provide a solid basis to address them in ancillary studies of the cohort., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Rationale: Turner syndrome is characterized by complete or partial loss of the second sex chromosome. In patients with Turner syndrome, hypertension is well described. However, the literature regarding malignant hypertension is scarce. Therefore, an accurate and timely diagnosis and treatment are important., Patient Concerns: A 13-year-old female with Turner syndrome presented to the emergency department with malignant hypertension, headache, spraying vomiting, convulsion, and loss of consciousness. Considering her medical history, symptoms, and auxiliary examination, secondary hypertension (primary reninism) was suspected, but without any occupying or hyperplasia in renal and adrenal., Diagnosis: A type of secondary hypertension, primary reninism., Interventions: The patient was immediately transferred to the pediatric intensive care unit. Subsequently, she was given nifedipine 0.35 mg/kg and captopril 0.35mg/kg to reduce blood pressure (BP), mannitol and furosemide to reduce cranial pressure, and phenobarbital and midazolam to terminate restlessness successively. Three hours later, the BP was consistently higher than 170/120 mm Hg, sodium nitroprusside was pumped intravenously, then, giving oral drug transition. Finally, she was given Valsartan-Amlodipine Tablets (I) (80 mg valsartan and 5 mg amlodipine per day) and bisoprolol (2.5 mg per day)., Outcomes: For 2.5 years of follow-up, the BP reduced to 110-130/60-85 mm Hg, heart rate ranged between 65 and 80 bpm, and she could go to school without any headache, convulsion, and syncope., Lessons: The clinical phenotype of Turner syndrome is complex and varied, affecting multiple systems and organs. Turner syndrome with malignant hypertension is rare, so we should systematically evaluate secondary hypertension, target-organ damage, and accompanied by standard management when Turner syndrome presents with hypertension., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: The etiology of nephrotic syndrome can vary, with underlying metabolic diseases being a potential factor. Cobalamin C (cblC) defect is an autosomal recessive inborn error of metabolism caused by mutations in the MMACHC gene, resulting in impaired vitamin B12 processing. While cblC defect typically manifests with hematological and neurological symptoms, renal involvement is increasingly recognized but remains rare., Case Presentation: We describe a 7-month-old male patient presenting with fatigue and edema. His first laboratory findings showed anemia, thrombocytopenia, hypoalbuminemia and proteinuria and further examinations reveals hemolysis in peripheric blood smear. During his follow up respiratory distress due to pleural effusion in the right hemithorax was noticed. And fluid leakage to the third spaces supported nephrotic syndrome diagnosis. The patient's condition deteriorated, leading to intensive care admission due to, hypertensive crisis, and respiratory distress. High total plasma homocysteine and low methionine levels raised suspicion of cobalamin metabolism disorders. Genetic testing confirmed biallelic MMACHC gene mutations, establishing the diagnosis of cblC defect. Treatment with hydroxycobalamin, folic acid, and betaine led to remarkable clinical improvement., Discussion/conclusion: This case underscores the significance of recognizing metabolic disorders like cblC defect in atypical presentations of nephrotic syndrome. Early diagnosis and comprehensive management are vital to prevent irreversible renal damage. While cblC defects are more commonly associated with atypical hemolytic uremic syndrome, this case highlights the importance of considering cobalamin defects in the differential diagnosis of nephrotic syndrome, especially when associated with accompanying findings such as hemolysis. Our case, which has one of the highest homocysteine levels reported in the literature, emphasizes this situation again., (© 2024. The Author(s).)

A man in his early 30s presented with sudden-onset respiratory distress, haemoptysis and reduced urine output. He was in volume overload with a blood pressure recording of 240/180 mm Hg. Pulmonary renal syndrome was suspected and he was initiated on plasmapheresis, followed by steroid pulse therapy. Chest radiography and the presence of fragmented red cells on the peripheral smear were unexplained. These were later explained by hypertensive nephropathy and thrombotic microangiopathy changes on renal biopsy. His respiratory and haematological parameters improved with blood pressure control. Malignant hypertension closely resembles pulmonary renal syndrome, which must be remembered in order to avoid plasmapheresis and high-dose immunosuppressive therapy., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Malignant hypertension (MHT) is a hypertensive emergency with excessive blood pressure (BP) elevation and accelerated disease progression. MHT is characterized by acute microvascular damage and autoregulation failure affecting the retina, brain, heart, kidney, and vascular tree. BP must be lowered within hours to mitigate patient risk. Both absolute BP levels and the pace of BP rise determine risk of target-organ damage. Nonadherence to the antihypertensive regimen remains the most common cause for MHT, although antiangiogenic and immunosuppressant therapy can also trigger hypertensive emergencies. Depending on the clinical presentation, parenteral or oral therapy can be used to initiate BP lowering. Evidence-based outcome data are spotty or lacking in MHT. With effective treatment, the prognosis for MHT has improved; however, patients remain at high risk of adverse cardiovascular and kidney outcomes. In this review, we summarize current viewpoints on the epidemiology, pathogenesis, and management of MHT; highlight research gaps; and propose strategies to improve outcomes., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: To describe the clinical characteristics, outcomes, and management of a large cohort of patients with concomitant malignant arterial hypertension and intracranial hypertension., Methods: Design: Retrospective case series., Subjects: Patients aged ≥ 18 years with bilateral optic disc oedema (ODE), malignant arterial hypertension and intracranial hypertension at five academic institutions. Patient demographics, clinical characteristics, diagnostic studies, and management were collected., Results: Nineteen patients (58% female, 63% Black) were included. Median age was 35 years; body mass index (BMI) was 30 kg/m 2 . Fourteen (74%) patients had pre-existing hypertension. The most common presenting symptom was blurred vision (89%). Median blood pressure (BP) was 220 mmHg systolic (IQR 199-231.5 mmHg) and 130 mmHg diastolic (IQR 116-136 mmHg) mmHg), and median lumbar puncture opening pressure was 36.5 cmH 2 O. All patients received treatment for arterial hypertension. Seventeen (89%) patients received medical treatment for raised intracranial pressure, while six (30%) patients underwent a surgical intervention. There was significant improvement in ODE, peripapillary retinal nerve fibre layer thickness, and visual field in the worst eye (p < 0.05). Considering the worst eye, 9 (47%) presented with acuity ≥ 20/25, while 5 (26%) presented with ≤ 20/200. Overall, 7 patients maintained ≥ 20/25 acuity or better, 6 demonstrated improvement, and 5 demonstrated worsening., Conclusions: Papilloedema and malignant arterial hypertension can occur simultaneously with potentially greater risk for severe visual loss. Clinicians should consider a workup for papilloedema among patients with significantly elevated blood pressure and bilateral optic disc oedema., (© 2023. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

A woman in her 30s presented with complaints of sudden onset of defective vision in the right eye for 2 days, with history of headache for a month. On examination, best corrected visual acuity was 20/40 in the right eye and 20/20 in the left eye. Anterior segment examination was normal. Fundus examination of both the eyes showed generalised arteriolar attenuation with diffuse, hyperaemic disc oedema and serous retinal detachment at macula in the right eye. Her blood pressure (BP) was 230/140 mm Hg. Other systemic evaluation was unremarkable. In the review visit, patient's BP reduced to 140/100 mm Hg, and visual acuity in the right eye improved to 20/20. Fundus in the right eye showed resolving disc oedema with macular star formation, and the left eye had developed soft exudates. This seemed to confirm the diagnosis of the disc oedema being caused by hypertension and a highly asymmetrical presentation of hypertensive retinopathy., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Rationale: This case report elucidates the management of a hypertensive crisis in a patient with underlying kidney disease, shedding light on the intricate interplay between these conditions. This unique case contributes valuable insights to the scientific literature., Patient Concerns: The patient exhibited severe headache, visual disturbances, and chest pain. Clinical evaluation revealed elevated blood pressure and impaired kidney function, emphasizing the importance of monitoring hypertension and renal health in such cases., Diagnoses and Interventions: The primary diagnoses included malignant hypertension and underlying kidney disease. Immediate interventions comprised intravenous antihypertensive agents and rigorous hemodynamic monitoring, yielding favorable outcomes. Blood pressure gradually returned to acceptable levels, and renal function improved during treatment., Conclusions: This case underscores the critical need for timely recognition and management of hypertensive crises in patients with preexisting kidney dysfunction. Simultaneously addressing both conditions is vital for successful outcomes. Healthcare practitioners must remain vigilant in assessing the intricate relationship between hypertension and kidney disease, employing tailored interventions for optimal results., Lesson Learned: The primary lesson from this case is the necessity of a comprehensive approach to managing hypertensive crises in individuals with underlying kidney disease. Early intervention and a multidisciplinary strategy are essential to achieve positive clinical outcomes and prevent potential complications., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Malignant hypertension triggers incremental renin activity, whereas primary aldosteronism suppresses such activity. We encountered a patient with malignant hypertension refractory to multiple anti-hypertensive agents. Repeated neurohormonal assessments, instead of a single one, eventually uncovered trends in an incremental aldosterone concentration, ranging from 221 up to 468 pg/mL, with a decline in the renin activity from 2.3 to <0.2 ng/mL/h. Adrenal venous sampling confirmed bilateral aldosterone secretion. Following the diagnosis of bilateral primary aldosteronism, we initiated a mineralocorticoid receptor antagonist, which improved his blood pressure. Repeated neurohormonal assessments are encouraged to correctly diagnose underlying primary aldosteronism with malignant hypertension.

Competing Interests: Footnotes and Disclosure The authors have no proprietary or commercial interest in any materials discussed in this essay. The data that support the findings of this study are available from the corresponding author upon request.

Background: Nephrosclerosis is one of the histopathological consequences of severe or malignant hypertension (MH), some of the pathophysiology of which has been extrapolated from essential polygenetic arterial hypertension. Despite our recent description of unsuspected ciliopathies with MH, causes of MH in young patients with severe renal impairment are poorly understood., Methods: To refine and better describe the MH phenotype, we studied clinical and prognostic factors in young patients receiving a kidney biopsy following their first episode of MH. Patients were identified retrospectively and prospectively from eight centres over a 35-year period (1985-2020). Keywords were used to retrospectively enrol patients irrespective of lesions found on renal biopsy., Results: A total of 114 patients were included, 77 (67%) of whom were men, average age 34 years, 35% Caucasian and 34% African origin. An isolated clinical diagnosis of severe nephrosclerosis was suggested in only 52% of cases, with 24% primary glomerulopathies. Only 7% of patients had normal renal function at diagnosis, 25% required emergency dialysis and 21% were eventually transplanted. Mortality was 1% at the last follow-up. Independent prognostic factors significantly associated with renal prognosis (6-month dialysis) and predictive of end-stage renal disease were serum creatinine on admission {odds ratio [OR] 1.56 [95% confidence interval (CI) 1.34-1.96], P < .001} and renal fibrosis >30% [OR 10.70 (95% CI 1.53-112.03), P = .03]. Astonishingly, the presence of any thrombotic microangiopathy lesion on renal biopsy was an independent, protective factor [OR 0.14 (95% CI 0.02-0.60), P = .01]. The histopathological hallmark of nephrosclerosis was found alone in only 52% of study patients, regardless of ethnicity., Conclusions: This suggests that kidney biopsy might be beneficial in young patients with MH., (© The Author(s) 2022. Published by Oxford University Press on behalf of the ERA.)

Background: We present a case of hypertensive choroidopathy due to malignant hypertension with exudative retinal detachment as a sole finding. We use OCT- angiography for initial diagnosis and report findings from extensive follow up., Case Presentation: A 51-year-old female with no past medical history, presented to our clinic with painless loss of vision in her left eye. Fundus examination revealed only exudative retinal detachment in her left eye that was confirmed with Optical Coherence Tomography. Fluorescein angiography showed hyperfluorescent spots with leakage in late phases. OCTA manifested a focal dark area in the choriocapillaris slab corresponding to flow signal voids, signifying regions of non-perfusion. Her blood pressure was 220/120 mmHG. Complete blood work -up failed to reveal any other possible etiology. During follow-up period of 9 months blood pressure normalized, patient regained visual function and choriocapillaris perfusion was completely restored., Discussions and Conclusions: Hypertensive choroidopathy with exudative retinal detachment can be the only sign of malignant hypertension and no pre-existing history of a systemic disease is required in order to become apparent. OCTA reveals areas of non-perfusion at choriocapillaris level, proving that it is an essential tool in the diagnosis and follow up of patients with hypertensive choroidopathy. Finally, we propose that early diagnosis prevents permanent damage of the RPE and leads to complete choroidal remodeling and better visual outcomes., (© 2023. The Author(s).)

Background: Thrombotic microangiopathy (TMA) is a complication of malignant hypertension (mHTN) attributed to high blood pressure (BP). However, no studies have investigated in patients with mHTN of different aetiologies whether the presence of TMA is associated with specific causes of mHTN., Methods: We investigated the presence of TMA (microangiopathic haemolytic anaemia and thrombocytopenia) in a large and well-characterized cohort of 199 patients with mHTN of different aetiologies [primary HTN 44%, glomerular diseases 16.6%, primary atypical haemolytic uraemic syndrome (aHUS) 13.1%, renovascular HTN 9.5%, drug-related HTN 7%, systemic diseases 5.5%, endocrine diseases 4.5%]. Outcomes of the study were kidney recovery and kidney failure., Results: Patients with TMA [40 cases (20.1%)] were younger, were more likely female and had lower BP levels and worse kidney function at presentation. Their underlying diseases were primary aHUS (60%), drug-related mHTN (15%), glomerular diseases [all of them immunoglobulin A nephropathy (IgAN); 10%], systemic diseases (10%) and primary HTN (5%). The presence of TMA was 92.3% in primary aHUS, 42.9% in drug-related HTN, 36.4% in systemic diseases, 12.1% in glomerular diseases and 2.3% in primary HTN. No patient with renovascular HTN or mHTN caused by endocrine diseases developed TMA, despite BP levels as high as patients with TMA. A higher proportion of TMA patients developed kidney failure as compared with patients without TMA (56.4% versus 38.9%, respectively)., Conclusions: The presence of TMA in patients with mHTN should guide the diagnosis towards primary aHUS, drug-related mHTN, some systemic diseases and IgAN, while it is exceptional in other causes of mHTN., (© The Author(s) 2022. Published by Oxford University Press on behalf of the ERA.)

Renal arteriovenous fistula (RAVF) is a rare vascular disease and is usually presented with severe hypertension. Renin-angiotensin-aldosterone system (RAAS) activation was proposed to play a key role in RAVF-induced hypertension but the data was inconsistent. We reported a case of RAVF presented as malignant hypertension, which was detected by contrast-enhanced ultrasonography and successfully managed by interventional embolization. A 35-year-old male was presented with a headache and blurred vision. His blood pressure was up to 220/110 mmHg, with significantly elevated serum creatinine and proteinuria. Hypertensive target organ impairments were noted. A RAVF was detected by contrast-enhanced renal ultrasonography. He underwent renal artery angiography and renal arteriovenous fistula embolization. RAAS activation was also evaluated by separate renal vein sampling. The patient's blood pressure and target-organ damage improved after RAVF embolization and blood pressure control. This is a rare case of renal arteriovenous fistula with malignant hypertension. Contrast-enhanced ultrasonography can be useful for diagnosis., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Alveolar hemorrhage presents with severe respiratory failure, requiring prompt diagnosis and treatment. Alveolar hemorrhage is often caused by autoimmune diseases accompanied by progressive renal dysfunction. However, few cases without autoimmune diseases occur, making diagnosis difficult. Here, we report a case of alveolar hemorrhage with hypertensive emergency., Patient Concerns: A 28-year-old man presented with dyspnea and bloody sputum. His blood pressure was 200/120 mm Hg., Diagnosis: The chest computed tomography showed suggestive of alveolar hemorrhage. Renal dysfunction and proteinuria were observed. However, autoantibodies were not detected. Echocardiogram revealed left ventricular function decrease. Ejection fraction was 20% to 30% with no ventricular asynergy or any valvular diseases. Brain magnetic resonance imaging showed hyperintense lesions on fluid-attenuated inversion recovery in the white matter of both cerebral and right cerebellar hemispheres, which were compatible with posterior reversible encephalopathy syndrome. Renal biopsy did not reveal any immune-mediated glomerulonephritis or vasculitis, but hypertensive nephropathy was diagnosed., Interventions: Blood pressure was controlled with combination therapy using calcium channel blocker, angiotensin II receptor blocker, α1 blocker, and β blocker., Outcomes: Alveolar hemorrhage and proteinuria improved with strict blood pressure control., Conclusion: This case indicates that severe hypertension can possibly cause alveolar hemorrhage. Accumulating these cases is important for general physicians to diagnose the alveolar hemorrhage with hypertensive emergency in its early stage and to avoid unnecessary treatment such as immunosuppressive therapy., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Pheochromocytomas are neoplasms originating from neuroectodermal chromaffin cells leading to excess catecholamine production. They are notorious for causing a triad of headaches, palpitations, and sweats. Though the Menard triad is one to be vigilant of, symptomatic presentation can vary immensely, hence the tumor earning the label "the great masquerader.", Case Presentation: We report a case of pheochromocytoma initially presenting with cortical blindness secondary to posterior reversible encephalopathy syndrome and thrombotic microangiopathy from malignant hypertension. Our patient was seen in our facility less than a week prior to this manifestation and discharged after an unremarkable coronary ischemia work-up. In the outpatient setting, she had been prescribed multiple anti-hypertensives with remarkably elevated blood pressure throughout her hospitalization history., Conclusion: Pheochromocytoma presenting with malignant hypertension and hypertensive encephalopathy should be expected if left untreated; nonetheless, the precipitation of cortical blindness is rare in the literature. This case contributes an additional vignette to the growing literature revolving adrenal tumors and their symptomatic presentation along with complex management. It also serves to promote increased diagnostic suspicion among clinicians upon evaluating patients with refractory hypertension., (© 2022. The Author(s).)

BACKGROUND Malignant hypertension (MHT), one of the severest forms of hypertension, can have deleterious effects on various organs, such as renal failure, retinopathy, and encephalopathy. These types of organ damage are common complications of MHT, but in several previous cases, damage to other organs, such as the gastrointestinal tract or pancreas, resulting from small vessel lesions, has also been reported, and these cases have had severe clinical outcomes and a poor prognosis. CASE REPORT A 32-year-old male patient with untreated hypertension of a 5-year duration presented with breathlessness and edema. His blood pressure was 220/144 mmHg, and he had renal dysfunction, congestive heart failure, and hypertensive retinopathy. He immediately received treatment, including antihypertensive agents and intermittent hemodialysis, but experienced epigastric pain for several days. A cystic lesion appeared in the pancreatic head, and his serum pancreatic enzymes were elevated. Based on these findings, acute pancreatitis with a cystic lesion was diagnosed. He first received fluid management, pain control, and parenteral nutrition but experienced 2 relapses. Finally, he received transpapillary endoscopic drainage for the cystic lesion with suspected walled-off necrosis. Thereafter, his symptoms improved. CONCLUSIONS The present case of MHT is the first to demonstrate acute necrotizing pancreatitis and it illustrates the difficulty of treatment. Therefore, if a patient with MHT presents with abdominal pain, a thorough workup, including contrast-enhanced computed tomography, should be performed to rule out significant organ involvement.

Competing Interests: Competing interests: None declared.

Competing Interests: Competing interests: None declared.

Background: Malignant hypertension is macrovascular and microvascular endothelial injury responsible for multiple organ damage. Considering the anatomical and functional homologies between the posterior pole of the eye and the kidney, ophthalmological explorations may inform clinicians on the mechanisms underpinning concurrent kidney injury in this condition. More specifically, we investigated whether the wall-to-lumen ratio (WLR) of retinal arterioles measured by adaptive optics ophthalmoscopy could be correlated to WLR of kidney arterioles as determined by pathology. We sought to estimate the incidence of retinal arteriole occlusion a supposedly uncommon complication of malignant hypertension., Methods: All patients hospitalized in our renal Intensive Care Unit for malignant hypertension between 2016 and 2019 were referred to ophthalmological examinations., Results: Twenty-seven patients were included. Median retinal WLR was 0.39 [0.31-0.47] and was correlated with initial systolic (r = 0.56, P = 0.003) and mean blood pressure (r = 0.46, P = 0.02) upon admission. The retinal WLR was not correlated to renal pathological findings, as assessed by juxtaglomerular WLR (r = 0.38, P = 0.2), ratio of glomerulosclerosis (r = -0.39, P = 0.2), or tubulointerstitial fibrosis (r = -0.45, P = 0.08). Retinal WLR was not associated with neurological or cardiovascular end-organ damage. Branch retinal artery occlusion was detected in 18.5% of patients and exudative retinal detachment (ERD) in 29.6% of patients, without any significant correlation with canonical signs of retinal hypertension including optic disc swelling., Conclusions: In the setting of malignant hypertension, we failed to demonstrate a significant relationship between WLR and other meaningful end-organ injuries. However, branch retinal artery occlusion and ERD may have been hitherto underestimated., (© American Journal of Hypertension, Ltd 2020. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Malignant hypertension, a form of hypertensive emergency, causes acute damage in vital organs such as the brain, eyes, and kidneys. We aimed to examine the concurrency of acute hypertensive damage across the target organs to elucidate the underlying analogous pathophysiology. This single-center retrospective study evaluated the characteristics of organ damage, short-term clinical course, and interorgan relationships in patients with malignant hypertension treated between 2008 and 2019. Baseline characteristics of 20 patients who met our inclusion criteria were mean age 48 ± 13 years and blood pressure 222 ± 18/142 ± 16 mmHg; the median estimated glomerular filtration rate and urinary protein level were 49 mL/min/1.73 m 2 (interquartile range [IQR] 27-79) and 1.9 g/g creatinine (IQR 0.2-4.0), respectively. Posterior reversible encephalopathy syndrome (PRES) was found in 60% of patients with major involvement and a wide variety of distribution patterns in the brainstem. In the fundus, serous retinal detachment was found in 60% of patients. Patients with PRES and serous retinal detachment showed higher levels of urinary protein than those without symptoms (P = 0.007 and 0.02, respectively), and proteinuria >1 g/g creatinine highly complicated both PRES and serous retinal detachment (91%). Matrix analysis also showed that the three symptoms were highly associated with each other. These results demonstrate the close relationship and concurrency of hypertensive acute organ damage in the brain, eyes, and kidneys. A common analogous mechanism, such as hyperperfusion-induced capillary leakage in each organ, implies an underlying pathophysiology of PRES, serous retinal detachment, and proteinuria.

Introduction: Thrombotic microangiopathy (TMA) is characterized by endothelial injury followed by formation of multiple microthrombi in the target organs due to various causes, including malignant hypertension (MHT). Here, we reported a rare case of MHT with splenic TMA changes., Case Concerns: A 27-year-old Chinese Han male with a history of hypertension and proteinuria, admitted to our hospital because of renal failure with MHT and thrombocytopenia., Diagnosis: This case diagnosed with TMA based on the patient's MHT and thrombocytopenia. The patient final diagnosis was confirmed by the spleen pathological findings, other differential diagnoses were ruled out., Interventions: The patient was treated with hemodialysis and intravenous antihypertensive agents, and his condition gradually improved. However, he suddenly complained of abdominal pain and went into hemorrhagic shock, which was due to spontaneous spleen rupture on the third day of hospitalization. The pathological evidence after splenectomy showed splenic TMA., Outcomes: Hemodialysis was continued and the blood pressure was under control until his discharge from our hospital., Conclusion: Spontaneous splenic rupture could be a rare and critical complication associated with MHT-induced TMA, and it requires careful clinical attention.

Background: Interleukin-11 (IL-11) is a pleiotropic cytokine of the interleukin-6 family. Recent studies revealed its crucial role in the development of cardiovascular fibrosis. In this study we examined IL-11 expression levels in the heart and the kidney exposed to high blood pressure in renovascular hypertensive rats and their correlations to fibrotic markers and kidney injury., Methods: Two-kidney, one-clip renovascular hypertension (2K1C) was induced in rats. IL-11 expression was measured by real-time polymerase chain reaction in the left ventricle and the right kidney. The correlation of cardiac IL-11 expression with biomarkers of renal fibrosis was assessed. We further investigated IL-11 expression in 2K1C rats grouped into rats with malignant vs. nonmalignant hypertension (distinguishing criteria: weight loss, number of fibrinoid necrosis, and onion skin lesions)., Results: Thirty-five days after clipping, mean arterial pressure was significantly increased in 2K1C. Renal IL-11 expression was elevated in 2K1C. In the heart there was only a trend toward higher IL-11 expression in 2K1C. IL-11 in the kidney in 2K1C correlated with the expression of transforming growth factor (TGF)-β1/2, collagens, fibronectin, osteopontin, as well as tissue inhibitors of metalloprotease 1/2. There were also correlations of IL-11 with tissue collagen expansion, number of activated fibroblasts and serum creatinine, but no correlation with mean arterial pressure. Renal expression of IL-11 was highest in rats with malignant hypertension., Conclusions: Renal IL-11 expression of renovascular hypertensive rats is markedly increased and correlates with profibrotic markers and loss of function and might therefore serve as a biomarker for the severity of hypertensive nephrosclerosis., (© American Journal of Hypertension, Ltd 2019. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Hypertensive emergency can cause thrombotic microangiopathy (TMA) in the kidneys with high rates of end-stage renal disease (ESRD) and vice versa. The conundrum of hypertension as the cause of TMA or consequence of TMA on the background of defects in complement regulation remains difficult. Patients with hypertensive emergency and TMA on kidney biopsy were tested for ex vivo C5b9 formation on the endothelium and rare variants in complement genes to identify complement-mediated TMA. We identified factors associated with defects in complement regulation and poor renal outcomes. Massive ex vivo C5b9 formation was found on resting endothelial cells in 18 (69%) out of 26 cases at the presentation, including the 9 patients who carried at least one rare genetic variant. Thirteen (72%, N =18) and 3 (38%, N =8) patients with massive and normal ex vivo complement activation, respectively, progressed to ESRD ( P =0.03). In contrast to BP control, inhibition of C5 activation prevented ESRD to occur in 5 (83%, N =6) patients with massive ex vivo complement activation. TMA-related graft failure occurred in 7 (47%, N =15) donor kidneys and was linked to genetic variants. The assessment of both ex vivo C5b9 formation and screening for rare variants in complement genes may categorize patients with hypertensive emergency and TMA into different groups with potential therapeutic and prognostic implications. We propose an algorithm to recognize patients at the highest risk for defects in complement regulation.

A 32 year-old man who presented with severe elevation of blood pressure after failure of kidney transplant. The patient had multiple serous retinal detachments, venous dilation, arteriolar narrowing, retinal haemorrhages, as well as optic disc oedema. Due to the impossibility of performing a fluorescein angiography, an angiotomography and en-face optical coherence tomography images were used to identify the vascular alterations in the retina, choroid, and choriocapillaris. Angiotomography and en face-optical coherence tomography mode images are very useful in cases where it is not possible to perform fluorescein angiography., (Copyright © 2019 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.)

Objectives: To compare and evaluate the characteristics of hypertensive choroidopathy with serous retinal detachment in preeclampsia and malignant hypertension (HTN) and explore choroidal ischemia as a pathogenesis using multimodal imaging., Methods: A retrospective multicenter case series. Medical charts were reviewed. Clinical characteristics and multimodal imaging, including optical coherence tomography (OCT) and OCT angiography (OCTA), were evaluated., Results: Fifty-three eyes of 29 preeclampsia patients and 45 eyes of 24 HTN patients were included. There were no differences in age, follow-up duration, baseline visual acuity, central macular thickness (CMT), or subfoveal choroidal thickness (CT) between the two groups. Blood pressure parameters, including systolic blood pressure, diastolic blood pressure, and pulse rate, were significantly higher in the HTN group. After serous retinal detachment resolved, both CMT (p < 0.001) and CT (p = 0.003) decreased more in the preeclampsia group. Hypertensive retinopathy features, including hemorrhage, exudates, cotton-wool spots, and optic disc edema, were predominantly found in the HTN group (p = 0.001). Final visual acuity was better in the preeclampsia group than in the HTN group (p = 0.048). Poor visual prognostic factors included the presence of retinopathy features (p = 0.005) and retinal detachment in the macula (p = 0.017)., Conclusion: Choroidal circulation may be affected earlier than retinal circulation by elevated blood pressure, presumably because of anatomical differences and autoregulatory mechanisms in the retinal vasculature. Serous retinal detachment with hypertensive choroidopathy presented with choroidal thickening that decreased after resolution, but the residual flow defects observed in the choriocapillaris on OCTA confirmed the long-hypothesized notion that ischemia is a mechanism underlying hypertensive choroidopathy.

Hypertensive emergency, which occurs even in young adults, induces systemic organ damage and results in a poor prognosis. We herein report the case of a 27-year-old man who developed alveolar hemorrhaging with hypertensive emergency. He presented with bloody sputum, renal failure, and extremely high blood pressure (200/128 mmHg). Chest computed tomography revealed diffuse bilateral alveolar infiltrates suggestive of diffuse alveolar hemorrhaging. After intensive therapy with anti-hypertensive drugs, the alveolar hemorrhaging disappeared. Renal impairment was partially reversed. Therefore, we conclude that hypertensive emergency should be considered as a possible cause of hemoptysis, even in young adults.

Background: Malignant hypertension, the most severe form of hypertension, is defined by high blood pressure and acute ischemic organ damage. It has a worse prognosis than other forms of hypertension, especially in black patients. New tools to assess organ damage, especially that of the heart and brain, are now available and may contribute to a better evaluation of these patients. This report improves knowledge of the characteristics of involved organs to facilitate diagnosis and to evaluate the effectiveness of our treatment protocol., Method: The Bordeaux registry, started in 1995, recruited 168 patients. In addition to evaluations of their eyes and kidneys, these patients had a systematic evaluation of their hearts with ECG and echocardiography and, since 2007, a systematic brain MRI. Blood pressure was lowered with a protocol based on blockers of the renin-angiotensin system started at a very low-dose with forced titration over 48 h. Only an oral route was used for antihypertensive medication., Results: Systematic MRIs found significant brain damage in 93% of patients. Heart involvement was highly prevalent: 82% had left ventricular mass more than 60 g/m, and 56% had systolic dysfunction (estimated by global longitudinal strain). Renal involvement and thrombotic microangiopathy were respectively present in 55 and 15% of patients. Median follow-up was 48 months. Renal survival at 5 years was 90.8%, similar to other studies., Conclusion: Malignant hypertension is a systemic disease causing severe damage to the brain, heart, kidneys and eyes, even in absence of symptoms. Renin-angiotensin system blockers seem to be the cornerstone of treatment.

We used electronic health records (EHRs) data from 5658 ambulatory chronic kidney disease (CKD) patients with hypertension and prescribed antihypertensive therapy to examine antihypertensive drug prescribing patterns, blood pressure (BP) control, and risk factors for resistant hypertension (RHTN) in a real-world setting. Two-thirds of CKD patients and three-fourths of those with proteinuria were prescribed guideline-recommended renoprotective agents including an angiotensin-converting enzyme inhibitor (ACEI) or an angiotensin receptor blocker (ARB); however, one-third were not prescribed an ACEI or ARB. CKD patients, particularly those with stages 1-2 CKD, who were prescribed regimens including beta-blocker (BB) + diuretic or ACEI/ARB + BB + diuretic were more likely to have controlled BP (<140/90 mm Hg) compared to those prescribed other combinations. Risk factors for RHTN included African American race and major comorbidities. Clinicians may use these findings to tailor antihypertensive therapy to the needs of each patient, including providing CKD stage-specific treatment, and better identify CKD patients at risk of RHTN., (©2018 Wiley Periodicals, Inc.)

Introduction: Intravitreal anti VEGF agents are used in a variety of retinal pathologies to decrease the VEGF levels resulting due to breakdown of the blood retinal barrier hence decrease the exudation from vessels which causes macular edema (ME)., Case: A 61year old patient presented with sudden decrease in vision in both eyes with a history of systemic malignant hypertension leading to macular edema as documented on optical Coherence Tomography (OCT) in both eyes. The foveal thickness (FT)of 536 and 328 microns (μ) were observed in the right and left eye each. He was advised intravitreal anti vascular endothelial growth factor (VEGF) in both eyes and advised complete systemic evaluation with the physician. Following one month postintravitreal bevacizumab ( IVB) injection in right eye, marked visual improvement was noted with concomitant significant reduction in macular edema in both eyes., Observation: Single Bevacizumab injection with control of hypertension in our patient resulted in rapid resolution of the macular edema and early visual recovery. Intravitreal anti VEGF is an effective treatment option in eyes due to hypertensivemaculopathy especially to gain speedy visual recovery., Conclusion: The case gives a unique outlook to the course of ME in the single patient with malignant HTN with or without IVB injection. We believe that anti VEGF injections may result in rapid recovery in vision and minimize the risk of permanent vision loss in eyes with malignant hypertension., (© NEPjOPH.)

Introduction: Capillary leak syndrome (CLS) is characterized by hypoproteinemia, diffused pitting edema, noncardiogenic pulmonary edema, and hypotension. By far, there are no related reports of CLS secondary to malignant hypertension (MHT). A 33-year-old male was admitted to our hospital with the diagnosis of CLS on the background of MHT., Patient Concerns: A 33-year-old male was admitted with a 6-day history of worsening dyspnea, chest distress, and diffused pitting edema accompanied by very high blood pressure (200/145 mm Hg)., Diagnoses: The tests and examinations showed hypoalbuminemia (26.7 g/L), pulmonary edema, and normal heart function. However, the expected massive proteinuria was absent (1.5 g/24 h). After diuretic and other antihypertensive therapy, the blood pressure reduced gradually; meanwhile, the symptoms of dyspnea and chest distress were improved quickly, and edema in his legs was also reduced. It is surprising that there was no change of pulmonary edema signs on imaging scan, and hypoalbuminemia remained with only mild proteinuria. Thus, our provisional diagnosis of this patient was CLS secondary to MHT., Interventions and Outcomes: We administered intravenous immunoglobulin, sulodexide, and renin-angiotensin system inhibitor to the patient for repairing vascular endothelium and improving the function of vascular endothelium. Before discharge, the patient's edema disappeared and the chest X-ray turned to normal. The level of serum albumin also increased to 35.1 g/L along with the overall improvement. Finally, the renal biopsy revealed malignant hypertensive glomerulosclerosis. All these clinical manifestations were consistent with CLS caused by MHT., Lessons: Up to now, there has been no case report of CLS caused by MHT. We should pay more attention to CLS induced by MHT, try to diagnose it as soon as possible, and give prompt treatment to CLS and primary disease.

Malignant hypertension can cause thrombotic microangiopathy (TMA) characterized by hemolytic anemia and thrombocytopenia. On the other hand, severe hypertension is sometimes associated with hemolytic uremic syndrome (HUS) or thrombotic thrombocytopenic purpura (TTP). Distinguishing these entities is important because of therapeutic implications. Plasmapheresis should be initiated as soon as possible if we are dealing with TTP. We describe the case of a 30-year-old man referred to our hospital with malignant hypertension, severe renal failure and TMA: haemoglobin=9g/dL, total bilirubin=0.4mg/dL, haptoglobin≤10mg/dL, platelet count=59,000/μL and schistocytes on peripheral smear. He required initiation of hemodialysis. Additionally, we considered that the possible cause of TMA was malignant hypertension according to the presence of hypertensive retinopathy and thrombocytopenia which remitted only with blood pressure control, hence, plasmapheresis was not given. Renal function did not improve and the patient remained chronic hemodialysis. Intensive therapy for hypertension with a combination of antihypertensive drugs including spironolactone successfully lowered his blood pressure without developing hyperkalemia., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)