A case of polyarteritis nodosa presenting with acute renal failure due to extensive interlobular and arcuate arterial occlusion

 

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A case of polyarteritis nodosa presenting with acute renal failure due to extensive interlobular and arcuate arterial occlusion

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Taito
Miyake
Taito Miyake taito06090713@yahoo.co.jp Kouseiren Takaoka Hospital Nephrology and Rheumatology Takaoka Japan *
Wataru Sone watarusone6131@gmail.com Kouseiren Takaoka Hospital Nephrology and Rheumatology Takaoka Japan -
Takuya Suda tkysd0911@gmail.com Kouseiren Takaoka Hospital Nephrology and Rheumatology Takaoka Japan -
Shouhei Makiishi Shohei.makiishi@gmail.com Kouseiren Takaoka Hospital Nephrology and Rheumatology Takaoka Japan -
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Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that predominantly affects small to medium-sized arteries. Patients with PAN usually present with systemic manifestations such as fever and weight loss. Their typical presentations also include mononeuritis multiplex, skin lesions (e.g., ulcers and livedo reticularis), and gastrointestinal bleeding. However, reports describing detailed renal involvement in PAN remain scarce.

We report a case of PAN presenting with acute renal failure, focusing on its clinical course and characteristic renal pathology.

A 78-year-old Japanese woman was admitted to our hospital with complaints of lower leg edema, weight loss, and loss of appetite. Urinalysis revealed proteinuria and microscopic hematuria. Laboratory tests showed severe renal impairment (serum creatinine 8.36 mg/dL, BUN 79.5 mg/dL), elevated CRP (8.36 mg/dL), and anemia (Hb 8.0 g/dL). Computed tomography showed no renal atrophy or urinary tract obstruction.Rapidly progressive glomerulonephritis was initially suspected, and glucocorticoid therapy along with hemodialysis was initiated. However, the patient suddenly died on day 7 due to intra-abdominal hemorrhage.

Autopsy revealed almost normal glomeruli and only mild interstitial inflammation and fibrosis in the kidney. In contrast, most interlobular and arcuate arteries exhibited fibrointimal occlusion, with fibrinoid necrosis noted in some interlobular arteries. The cause of death was attributed to splenic arterial bleeding, although no microaneurysm was identified. Based on these findings, a diagnosis of PAN was established. We hypothesized that severe renal dysfunction resulted from dehydration-induced reduction in renal blood flow superimposed on preexisting occlusion of the renal interlobular and arcuate arteries.

We encountered a rare case of PAN in which extensive occlusion of the interlobular and arcuate arteries led to severe acute renal failure precipitated by dehydration. PAN typically causes necrotizing vasculitis in small to medium-sized arteries; within the kidney, the arcuate and interlobular arteries correspond to small muscular arteries. Although acute renal failure due to PAN has been reported previously, such cases remain rare, and detailed pathological descriptions are limited. PAN should be recognized as a potential cause of small arterial occlusion within the kidneys, contributing to the rapid progression of acute renal failure.

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