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Published on: June 18, 2020
Renal Medullary Angiitis Associated with Cutaneous Leukocytoclastic Vasculitis
Shoko Miura1, Kan Katayama2, Kensuke Joh3
1Department of Nephrology, Kuwana City Medical Center, Japan.
Abstract:
Renal medullary angiitis is characterized by interstitial hemorrhaging in the medulla with neutrophil infiltration. An 81-year-old man presented with a fever, kidney dysfunction, and purpura of the legs, which was diagnosed as leukocytoclastic vasculitis. Proteinase 3 antineutrophil cytoplasmic antibodies were weakly positive. A kidney biopsy showed severe tubulointerstitial hemorrhaging with neutrophilic infiltration in the perivascular areas surrounding the vasa recta in the medulla without crescent formation in the glomeruli. An immunofluorescence analysis was negative, and electron microscopy revealed no immune-dense deposits, ruling out immunoglobulin A vasculitis. Intravenous methylprednisolone for three days and plasma exchange followed by oral prednisolone improved his general condition.
Insights
Renal medullary angiitis, a rare condition causing kidney bleeding and neutrophil infiltration, was diagnosed in an elderly man with vasculitis. Treatment with steroids and plasma exchange led to clinical improvement.
Area of Science:
- Nephrology
- Pathology
- Rheumatology
Background:
- Renal medullary angiitis is a rare vasculitic condition.
- It is characterized by interstitial hemorrhaging and neutrophil infiltration in the renal medulla.
Purpose of the Study:
- To present a case of renal medullary angiitis in an elderly male patient.
- To highlight the diagnostic challenges and treatment outcomes.
Main Methods:
- Case report of an 81-year-old male with fever, kidney dysfunction, and purpura.
- Diagnostic workup included serological tests (Proteinase 3 antineutrophil cytoplasmic antibodies), kidney biopsy with immunofluorescence, and electron microscopy.
- Treatment involved intravenous methylprednisolone, plasma exchange, and oral prednisolone.
Main Results:
- The patient presented with symptoms suggestive of leukocytoclastic vasculitis.
- Kidney biopsy revealed severe tubulointerstitial hemorrhaging and neutrophilic infiltration in the medulla, without glomerular crescents.
- Immunofluorescence and electron microscopy ruled out immunoglobulin A vasculitis.
- The patient showed clinical improvement after treatment.
Conclusions:
- Renal medullary angiitis can present as kidney dysfunction and purpura in the elderly.
- A comprehensive diagnostic approach is crucial to differentiate it from other vasculitides.
- Prompt treatment with corticosteroids and plasma exchange can lead to favorable outcomes.
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