Plasma exchange combined with immunosuppressive treatment in a child with rapidly progressive IgA nephropathy |
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Authors: | Shuichiro Fujinaga Yoshiyuki Ohtomo Daisuke Umino Hiroshi Mochizuki Hitohiko Murakami Toshiaki Shimizu Yuichiro Yamashiro Kazunari Kaneko |
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Affiliation: | (1) Division of Nephrology, Saitama Children’s Medical Center, 2100 Magome, Iwatsuki-ku, Saitama-city Saitama 339 8551, Japan;(2) Department of Pediatrics, Juntendo Nerima Hospital, Tokyo, Japan;(3) Division of Pathology, Saitama Children’s Medical Center, Saitama, Japan;(4) Department of Pediatrics, Juntendo University School of Medicine, Tokyo, Japan;(5) Department of Pediatrics, Kansai Medical University, Osaka, Japan |
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Abstract: | Although diffuse crescentic formation in immunoglobulin A (IgA) nephropathy, histologically characterized by extensive extracapillary proliferation, is assumed to have a poor prognosis, there has still been no established treatment because of the low prevalence of the condition, especially in pediatric patients. This paper reports on a 5-year-old boy with rapidly progressive IgA nephropathy requiring dialysis for 1 month. He had been treated with plasma exchange (PE) combined with immunosuppressive treatment, including steroids and mizoribine, because renal function deteriorated rapidly despite initial treatment with intravenous methylprednisolone pulse. The histological findings at that time revealed IgA nephropathy, with large circumferential cellular crescent formation in approximately 80% of the glomeruli. Three weeks after PE initiation, serum levels of creatinine and IgA-containing immune complexes returned to normal, and urinary protein excretion gradually decreased. The second renal biopsy taken 7 months later demonstrated mild IgA nephropathy with small fibrocellular crescents. This case report indicates that PE combined with immunosuppressive treatment may benefit children with rapidly progressive IgA nephropathy, even when extensive crescent formations are present. |
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Keywords: | Rapidly progressive IgA nephropathy Plasma exchange Immunosuppressive treatment |
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