Systemic Vasculitis: An Important and Underestimated Cause of Malignant Hypertension

Abstract

Objectives: Malignant hypertension (MHT) is defined as severe hypertension accompanied by ischemic failure of one or more organs. The aims of this study were to evaluate the current clinical and etiologic profiles of MHT. Methods: As a retrospective study, we selected all patients admitted to our center from January 2013 to December 2016. Seventy patients with MHT were included. Results: The average age of the patients was 40 years, and more than half of the patients were male (78.57%). There were 24 patients with essential hypertension, accounting for 34.29% of the patients, and 46 with secondary hypertension, accounting for 65.71% of the patients. For secondary MHT, systemic vasculitis (25.57%) was the most common cause, followed by severe obstructive sleep apnea syndrome (15.71%), primary renal parenchymal hypertension (11.43%), primary aldosteronism (7.14%), and Cushing syndrome (1.43%) and nutcracker phenomenon (1.43%). Twenty patients with systemic vasculitis were characterized by severe hypertension accompanied by damage to two or more target organs of differing severity. The levels of white blood cells, hypersensitive C-reactive protein, serum creatinine, and 24-hour urinary protein were above their normal range. Conclusion: Systemic vasculitis may be one of the main causes of MHT, and has been underestimated in the past. In future clinical work, clinicians need to pay more attention to patients with systemic vasculitis.

Fatal hemoperitoneum due to rupture of mesenteric artery in remission state of microscopic polyangiitis, concomitant with severe hypertension and posterior reversible encephalopathy syndrome: an autopsy case report

“…Treatment-resistant severe hypertension led to PRES and the rupture of the mesenteric artery. Systemic vasculitis has recently been identified as an important cause of secondary malignant hypertension, which is a life-threatening manifestation of hypertension [10]. The pathophysiology of hypertension by small-vessel vasculitis is as follows:…”

“…(1) activation of the renin-angiotensin-aldosterone system due to renal artery stenosis, (2) reduction in glomerular filtration area caused by ischemic damage of small arterioles, and (3) vascular endothelial cell damage [10]. In addition, Sada et al reported that 19% of elderly patients with AAV who were treated with high-dose glucocorticoids developed hypertension, which was the most frequent complication in this type of vasculitis, compared to patients treated with medium-dose glucocorticoids [11].…”

Increased Urinary CD163 Levels in Systemic Vasculitis with Renal Involvement

“…In some cases, hypertension is one of the main clinical manifestations of vasculitis. The study has shown that vasculitis is one of the most important causes of malignant hypertension [ 3 ]. If untreated, this group of diseases can pose a threat to patient's life.…”