Renovascular Hypertension: A Unique Cause of Unilateral Focal Segmental Glomerulosclerosis

Alchi, Bassam; Shirasaki, Arimasa; Narita, Ichiei; Nishi, Shinichi; Ueno, Mitsuhiro; Saeki, Takako; Miyamura, S; Gejyo, Fumitake

doi:10.1291/hypres.29.203

Cited by 15 publications

(17 citation statements)

References 12 publications

(11 reference statements)

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“…Only a few clinical studies described the development of FSGS in malignant hypertension [12][13][14], and these studies reported that FSGS may occur in primary malignant hypertension with nephrotic-range proteinuria, and may contribute to renal dysfunction. In addition, a few cases of renal artery stenosis with malignant hypertension and nephrotic syndrome have been reported [15][16][17][18][19][20]. In such cases, histopathological examination of the contralateral kidney often shows lesions of FSGS with benign or malignant nephrosclerosis.…”

Section: Discussionmentioning

confidence: 99%

A case of secondary focal segmental glomerulosclerosis associated with malignant hypertension

et al. 2012

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Focal segmental glomerulosclerosis (FSGS) is associated with various clinicopathological conditions, including hypertension. We report here a case of secondary FSGS associated with malignant hypertension. A 33-yearold man with a 1-month history of visual impairment and headache visited the Department of Ophthalmology at our hospital and was found to have hypertensive retinopathy and severe hypertension (230/160 mmHg). He was referred to our department based on suspected renal dysfunction. His blood pressure on admission was 250/130 mmHg. Physical examination and laboratory tests revealed hypertensive cardiac dysfunction, focal brain edema, renal dysfunction (serum creatinine, Cr 7.07 mg/dl, blood urea nitrogen, BUN 49.9 mg/dl), massive proteinuria (10.7 g/day), and thrombotic microangiopathy. Funduscopy showed exudate, hemorrhage, and papilledema. The cause of secondary hypertension could not be identified. He was treated for primary malignant hypertension, but required hemodialysis 3 days after admission due to anuria. Treatment with antihypertensive agents resulted in the gradual recovery of renal function, although heavy proteinuria continued with nephrotic syndrome. Renal biopsy performed 1 month after admission showed features of malignant nephrosclerosis with secondary FSGS. Hemodialysis was discontinued following further improvement in renal function and the most recent laboratory tests showed proteinuria 1.8 g/day and persistent renal dysfunction (BUN 36.5 mg/dl, Cr 3.14 mg/ dl). Malignant hypertension may cause various injuries, including glomerular endothelial and epithelial cell injuries in glomerular hypertension and hyperfiltration, increase of the renin-angiotensin-aldosterone system, and endothelialepithelial interaction, resulting in the development of secondary FSGS and heavy proteinuria.

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Section: Discussionmentioning

confidence: 99%

A case of secondary focal segmental glomerulosclerosis associated with malignant hypertension

et al. 2012

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“…In existing medical literature there have been a few well-documented cases of unilateral RAS associated with NS [1, 2, 3, 4, 5, 6]. The mechanism has been postulated to be hemodynamic (hyperfiltration) injury to the nonstenosed kidney with protection from this injury in the stenosed kidney.…”

Section: Discussionmentioning

confidence: 99%

“…Renovascular hypertension with its associated activation of the renin-angiotensin-aldosterone system (RAAS) has been reported under certain circumstances, to be associated with nephrotic syndrome (NS) [1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12]. This has most often been in cases of unilateral stenosis [1, 2, 3, 4, 5].…”

Section: Introductionmentioning

confidence: 99%

“…This has most often been in cases of unilateral stenosis [1, 2, 3, 4, 5]. The mechanism is not fully elucidated but some authors have postulated that unilateral renal artery stenosis (RAS) produces hyperfiltration injury and secondary focal and segmental glomerulosclerosis (FSGS) in the unaffected kidney with demonstration of protection of the stenotic kidney from hemodynamic injury [1, 2, 3, 4, 5, 12]. This, however, could not account for the cases of NS where bilateral RAS is present [10, 11].…”

Section: Introductionmentioning

confidence: 99%

“…These findings suggest that circulating factors related to RAAS activation may play a primary role. In most cases, the NS resolved either with revascularization, nephrectomy of the stenotic kidney or angiotensin blockade [1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11]. We are reporting a case presenting for evaluation of NS that was incidentally discovered to have bilateral RAS due to fibromuscular dysplasia (FMD).…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Steroid-Responsive Nephrotic Syndrome and Bilateral Renal Artery Stenosis: A Possible Role for Angiotensin-Mediated Podocyte Injury

Bhardwaj¹,

Dosani²,

Clark³

2012

Case Rep Nephrol Urol

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Nephrotic syndrome (NS) associated with renal artery stenosis is not widely recognized or investigated as a cause of the NS. The mechanisms are incompletely understood, but have largely focused on hemodynamic factors resulting in hyperfiltration injury-induced focal and segmental glomerulosclerosis (FSGS) in the nonstenosed kidney with sparing of the stenotic kidney protected from hemodynamic stress. However, separation of hemodynamic from circulating factors (such as angiotensin II) as the cause of the nephrosis remains difficult. We report a patient presenting with NS who was incidentally discovered to have high-grade bilateral renal artery stenosis from fibromuscular dysplasia. Kidney biopsy revealed FSGS. Proteinuria in our patient did not initially respond to angiotensin-converting enzyme inhibition (ACEI) and correction of stenoses with angioplasties. There was prompt response to steroid treatment. A brief relapse several months later (without associated hypertension) responded to ACEI alone. This is the first reported case of an association between fibromuscular dysplasia and steroid-responsive nephrotic syndrome due to FSGS. This may shed insight into the nature of podocyte injury in patients with high angiotensin states and suggest a possible role for activated renin-angiotensin-aldosterone system (RAAS) triggering an immune-mediated injury, rather than hemodynamic insult. Furthermore the lack of initial response to angioplasty and ACEI suggests that RAAS-activated injury may in some cases require more aggressive immune modulatory therapy with steroids over and above angiotensin inhibition alone. This case also highlights the importance of being aware of possible occult renovascular disease contributing to idiopathic NS with FSGS even when hypertension is only modest.

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ROS in Atherosclerotic Renovascular Disease

Zhu

Lerman

2016

Studies on Atherosclerosis

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Renovascular Hypertension: A Unique Cause of Unilateral Focal Segmental Glomerulosclerosis

Cited by 15 publications

References 12 publications

A case of secondary focal segmental glomerulosclerosis associated with malignant hypertension

A case of secondary focal segmental glomerulosclerosis associated with malignant hypertension

Steroid-Responsive Nephrotic Syndrome and Bilateral Renal Artery Stenosis: A Possible Role for Angiotensin-Mediated Podocyte Injury

ROS in Atherosclerotic Renovascular Disease

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