Perianeurysmal retroperitoneal fibrosis presenting as acute kidney injury: A case report. 2021

Daniel Z Hodson, and Lloyd G Cantley, and Sudhir Perincheri, and Nikhil Singh

Retroperitoneal fibrosis and chronic periaortitis describe overlapping groups of rare diseases characterized by inflammation and fibrosis involving the aorta. The presentation is often non-specific, and while obstructive nephropathy is a common complication, these entities are an uncommon cause of renal failure necessitating dialysis. A 57-year-old man presented multiple times with acute kidney injury, even requiring hemodialysis, with repeated abrupt resolution. Renal ultrasound repeatedly did not reveal acute hydronephrosis. Renal biopsy on his first admission showed acute tubular injury attributed to hypovolemia. Computed tomography finally revealed a retroperitoneal soft tissue mass encasing the infrarenal abdominal aorta and partially encasing the bilateral ureters. Bilateral nephrostomy tubes were placed, steroids were initiated, and the patient experienced rapid and remarkable improvement in renal function. Chronic periaortitis should be considered in older patients with acute kidney injury, even in the absence of ultrasonographic evidence of obstruction. Additional studies are needed to describe the test characteristics of renal sonography for periaortitis, the long-term sequelae of acute kidney injury secondary to periaortitis, and the optimal management to preserve long-term renal function.

UI MeSH Term Description Entries
D007668 Kidney Body organ that filters blood for the secretion of URINE and that regulates ion concentrations. Kidneys
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012185 Retroperitoneal Fibrosis A slowly progressive condition of unknown etiology, characterized by deposition of fibrous tissue in the retroperitoneal space compressing the ureters, great vessels, bile duct, and other structures. When associated with abdominal aortic aneurysm, it may be called chronic periaortitis or inflammatory perianeurysmal fibrosis. Ormond Disease,Perianeurysmal Fibrosis, Inflammatory,Periaortitis, Chronic,Fibrosis, Inflammatory Perianeurysmal,Fibrosis, Perianeurysmal Inflammatory,Idiopathic Retroperitoneal Fibrosis,Inflammatory Fibrosis, Perianeurysmal,Inflammatory Perianeurysmal Fibrosis,Ormond's Disease,Perianeurysmal Inflammatory Fibrosis,Chronic Periaortitides,Chronic Periaortitis,Disease, Ormond,Disease, Ormond's,Fibroses, Retroperitoneal,Fibrosis, Retroperitoneal,Periaortitides, Chronic,Retroperitoneal Fibroses
D058186 Acute Kidney Injury Abrupt reduction in kidney function. Acute kidney injury encompasses the entire spectrum of the syndrome including acute kidney failure; ACUTE KIDNEY TUBULAR NECROSIS; and other less severe conditions. Acute Kidney Failure,Acute Kidney Insufficiency,Acute Renal Failure,Acute Renal Injury,Acute Renal Insufficiency,Kidney Failure, Acute,Kidney Insufficiency, Acute,Renal Failure, Acute,Renal Insufficiency, Acute,Acute Kidney Failures,Acute Kidney Injuries,Acute Kidney Insufficiencies,Acute Renal Failures,Acute Renal Injuries,Acute Renal Insufficiencies,Kidney Failures, Acute,Kidney Injuries, Acute,Kidney Injury, Acute,Kidney Insufficiencies, Acute,Renal Failures, Acute,Renal Injuries, Acute,Renal Injury, Acute,Renal Insufficiencies, Acute

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