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Differences in Etiology and Treatment in Japan

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Pathogenesis and Treatment in IgA Nephropathy

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Abstract

IgA nephropathy is a major glomerulonephritis in Japan, 30–50 % of primary glomerulonephritis in adults and around 20 % in children. Around 70 % of patients are found as asymptomatic hematuria and/or proteinuria.

A number of investigations concerning tonsillar abnormality have been reported, which may reflect the characteristic pathogenesis of IgA nephropathy in Japan.

Since Kobayashi et al. firstly reported a significant effect of steroids on reducing proteinuria and prevention of progression in 1986, the steroid treatment was started to be used in patients with progressive IgA nephropathy.

The effect of tonsillectomy has been reported in IgA nephropathy with chronic tonsillitis. Since Hotta et al. reported the impact of tonsillectomy and steroid pulse therapy on the remission of urinary abnormality in 2001, tonsillectomy with steroid pulse therapy has been widely spread throughout of Japan as the first line of treatment of adult patients with IgA nephropathy. However, high-level evidence of the effectiveness of such treatment on long-term outcome has been required. In 2014, Kawamura et al. reported a significantly greater antiproteinuric effect in patients treated with tonsillectomy combined with steroid pulse therapy than in those with steroid pulse monotherapy in a multicenter, randomized, controlled trial (RCT). However, since the difference was marginal, the impact of tonsillectomy combined with steroid pulse therapy on the renal outcome remains unknown.

The treatment of Japanese childhood IgA nephropathy has been determined based on several RCTs in the different subset of children with IgA nephropathy by the Japanese Pediatric IgA Nephropathy Treatment Study Group.

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  1. Kidney Unit, National Fukuoka Higashi Medical Center, 1-1-1 Chidori, Koga-City, Fukuoka, 811-3195, Japan

    Ritsuko Katafuchi

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    Yasuhiko Tomino

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Katafuchi, R. (2016). Differences in Etiology and Treatment in Japan. In: Tomino, Y. (eds) Pathogenesis and Treatment in IgA Nephropathy. Springer, Tokyo. https://doi.org/10.1007/978-4-431-55588-9_11

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