Takayasu Arteritis Presenting as Renovascular Hypertension and Renal Failure in a Patient with Factor VII Deficiency.

Sercin Ozkok,Yildiz Gulseren,Ahmet Aslan,Mehmet Ali Agirbasli,Gonul Kutlu Aciksari

doi:10.5152/eurasianjmed.2018.17324

Sercin Ozkok, Yildiz Gulseren + Show 3 more

Open Access

https://doi.org/10.5152/eurasianjmed.2018.17324

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Abstract

A 23-year-old female patient with factor VII (FVII) deficiency was admitted with severe hypertension and renal failure. Brachial arterial pressures were 230/120 and 220/115 mm/Hg on the right and left arms, respectively. There was no blood pressure difference between the arms. Renal artery Doppler ultrasonography revealed bilateral severe renal artery stenosis (RAS). Contrast-enhanced magnetic resonance imaging angiography (CE-MRA) revealed severe mural irregularities, contrast enhancement in the aorta and its branches, and long-segment stenosis starting in the abdominal aorta and extending into the proximal renal arteries. The diagnosis of Takayasu arteritis (TA) complicated by RAS in a patient with FVII deficiency was established. This is the first case of concomitant TA and factor VII deficiency in the literature. In conclusion, TA complicated with RAS should be kept in mind in the etiology of secondary hypertension, even when there is no blood pressure difference between the arms in patients. CE-MRA is an accurate, sensitive, and safe imaging method for diagnosing vasculitis, even in the early phases of the disease, and should be considered for evaluating the activity and response to treatment in patients with TA.

Highlights

Takayasu arteritis (TA) is granulomatous vasculitis of the aorta and medium-sized arteries
TA complicated with renal artery stenosis (RAS) should be kept in mind in the etiology of secondary hypertension, even when there is no blood pressure difference between the arms in patients
We aimed to present the first case of factor VII (FVII) deficiency together with TA complicated by renovascular hypertension and renal failure secondary to renal artery stenosis (RAS)

Summary

Introduction

Takayasu arteritis (TA) is granulomatous vasculitis of the aorta and medium-sized arteries. The clinical presentation of patients with TA depends on the involvement of the arterial segment and the severity of the disease. The most common presentation of patients with TA involves the subclavian and renal arteries [1]. Factor VII (FVII) deficiency is a rare coagulation disorder that can be congenital or acquired. Acquired FVII deficiency is very rare and has been reported to occur due to sepsis, myeloproliferative diseases, and drugs [3, 4]. 3-4% of patients with FVII deficiency present with thrombotic events [6]. We aimed to present the first case of FVII deficiency together with TA complicated by renovascular hypertension and renal failure secondary to renal artery stenosis (RAS)

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