Background: Perirenal hematomas are most frequently caused by neoplasm, followed by polyarteritis nodosa (PN), but may also be a rare complication of MPA. Case Presentation: A 77-year-old woman who presented with rapidly progressive glomerulonephritis, proteinuria, and mononeuropathy multiplex, and was positive for serum myeloperoxidase-anti-neutrophil cytoplasmic antibody, was diagnosed with vasculitis as a probable microscopic polyangiitis (MPA). She developed a spontaneous right-sided perirenal hematoma, along with back pain, on the day of referral to the nephrology unit and a spontaneous left-sided perirenal hematoma ten days later. Treatment with 30 mg/day prednisolone stabilized the hematomas and controlled the progression of probable MPA. Conclusion: Nephrectomy or embolization is commonly required in patients with perirenal hematoma associated with neoplasm or PN, but radical invasive management may not be necessary in patients with MPA because smaller blood vessels are affected, resulting in less extensive bleeding and normal clotting. Accurate differential diagnosis between MPA and PN is essential to avoid invasive management of perirenal hematomas in patients with MPA. The presence of glomerulonephritis suggests injury to capillary vessels, a characteristic of MPA.
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