CLINICAL COURSE OF TWO CASES DIAGNOSED WITH RENAL FIBROMUSCULAR DYSPLASIA PRESENTING WITH HYPERTENSION AND RENAL DYSFUNCTION

 

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CLINICAL COURSE OF TWO CASES DIAGNOSED WITH RENAL FIBROMUSCULAR DYSPLASIA PRESENTING WITH HYPERTENSION AND RENAL DYSFUNCTION

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Hiroki
Okushima
Hiroki Okushima okuringo@hotmail.co.jp Osaka general medical center Division of Kidney and Hypertension Osaka Japan *
Miyuu Tsuchida h.okushima@kid.med.osaka-u.ac.jp Osaka general medical center Division of Kidney and Hypertension Osaka Japan -
Shuzo Makino 05a14078@gmail.com Osaka general medical center Division of Kidney and Hypertension Osaka Japan -
Akito Takashima okushimahi@gh2.opho.jp Osaka general medical center Division of Kidney and Hypertension Osaka Japan -
Hiromitsu Miyakawa mi8kawa1009@gmail.com Osaka general medical center Division of Kidney and Hypertension Osaka Japan -
Nobuhiro Hashimoto bosuhiro2005@yahoo.co.jp Osaka general medical center Division of Kidney and Hypertension Osaka Japan -
Terumasa Hayashi t-hayashi@abelia.ocn.ne.jp Osaka general medical center Division of Kidney and Hypertension Osaka Japan -
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Renal fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vascular disease that can cause renovascular hypertension and, in some cases, progressive renal impairment. We report two cases of renal FMD presenting with hypertension and subsequent decline in renal function during long-term follow-up.

Two cases presentation

Case 1: A man in his 30s had been hypertensive since his 20s and was treated with antihypertensive agents, maintaining home blood pressure around 130/90 mmHg. An incidental renal artery aneurysm detected on abdominal CT led to further evaluation. Laboratory findings revealed elevated plasma renin activity (18.7 ng/mL/h) and aldosterone (202 pg/mL). Contrast-enhanced CT showed beaded stenoses in both renal arteries and wedge-shaped cortical scars, consistent with FMD. Initial renal function was normal (Cr 0.74 mg/dL, eGFR 99 mL/min/1.73 m²), but despite adequate blood pressure control with renin–angiotensin system inhibition, renal function gradually declined over 15 years to Cr 1.93 mg/dL and eGFR 31 mL/min/1.73 m², accompanied by cortical thinning on ultrasonography.
Case 2: A man in his 20s was referred for secondary hypertension after elevated plasma renin activity (30.2 ng/mL/h) was detected. Contrast-enhanced CT revealed multiple beaded stenoses and occlusions in the distal branches of both renal segmental arteries and the celiac artery, leading to a diagnosis of FMD. Renal function was initially preserved (Cr 0.69 mg/dL, eGFR 112 mL/min/1.73 m²), but mild cortical atrophy and a decline in eGFR to 79 mL/min/1.73 m² were observed over 10 years, despite effective antihypertensive therapy.

Discussion:Although renal function is usually maintained in FMD, 2.8–8.9% of patients develop renal impairment, potentially due to hypertension or repeated renal infarctions. Both cases showed progressive cortical thinning and renal dysfunction despite adequate blood pressure control, suggesting ischemic injury as a key contributing factor. While percutaneous transluminal angioplasty may be considered in select patients, an established effective therapy for diffuse or distal lesions is lacking.


Renal FMD can lead to progressive renal dysfunction through hypertension and recurrent renal infarctions. Careful long-term follow-up and strict blood pressure control are essential for optimal management.

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