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A case of chronic relapsing ANCA-associated microscopic polyangiitis successfully treated with plasma exchange
Yuko Akioka1, Motoshi Hattori, Hiroshi Kawaguchi
1Department of Pediatric Nephrology, Kidney Center, Tokyo Women's Medical University School of Medicine, Tokyo, Japan.
Abstract:
We report the case of a 4.5-year-old girl with microscopic polyangiitis (MPA) manifesting antineutrophil cytoplasmic autoantibody (ANCA)-positive necrotizing crescentic glomerulonephritis and pulmonary hemorrhage. She was initially on induction therapy with corticosteroids and azathioprine. Plasma exchange (PE) combined with immunosuppressants was used to treat an episode of recurrent pulmonary hemorrhage, and achieved remission. At 9.8 years of age her kidney disease relapsed, associated with renal dysfunction and increased proteinuria. To minimize the toxic effects of immunosuppressants, she was treated with PE again, and her renal dysfunction resolved. Plasma exchange was effective in reducing the risk of death and preserving long-term renal function without the severe adverse effects of immunosuppressants. Our preliminary results indicate that PE is likely to be a treatment option for children in acute phase of ANCA-associated MPA, who should be protected from the toxic effects of immunosuppressants.
Insights
Plasma exchange (PE) effectively treated a child with microscopic polyangiitis (MPA), resolving kidney and lung issues. This approach minimized toxic immunosuppressant effects, preserving long-term kidney function.
Area of Science:
- Pediatric Nephrology
- Rheumatology
- Immunology
Background:
- Microscopic polyangiitis (MPA) is a rare autoimmune vasculitis affecting small blood vessels, often leading to severe kidney and lung damage.
- Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis in children requires effective treatment strategies to prevent end-organ damage and long-term sequelae.
Observation:
- A 4.5-year-old girl presented with ANCA-positive necrotizing crescentic glomerulonephritis and pulmonary hemorrhage, initially treated with corticosteroids and azathioprine.
- Recurrent pulmonary hemorrhage necessitated plasma exchange (PE) combined with immunosuppressants, achieving remission.
- Relapse of kidney disease at 9.8 years, with renal dysfunction and proteinuria, was managed with PE to mitigate immunosuppressant toxicity, leading to resolution of renal dysfunction.
Findings:
- Plasma exchange (PE) demonstrated efficacy in managing acute episodes of pulmonary hemorrhage and renal relapse in a pediatric patient with ANCA-associated MPA.
- PE was effective in preserving long-term renal function and reducing the risk of mortality.
- Treatment with PE minimized the severe adverse effects associated with prolonged immunosuppressant therapy.
Implications:
- Plasma exchange may represent a valuable therapeutic option for children experiencing the acute phase of ANCA-associated MPA.
- This approach offers a strategy to protect pediatric patients from the cumulative toxic effects of conventional immunosuppressants.
- Further investigation into PE as a primary or adjunctive therapy in pediatric ANCA-associated vasculitis is warranted.
