Complement activation is associated with crescent formation in IgA nephropathy
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ORIGINAL ARTICLE
Complement activation is associated with crescent formation in IgA nephropathy Hiroe Itami 1 & Shigeo Hara 2 & Kenichi Samejima 3 & Hideo Tsushima 3 & Katsuhiko Morimoto 4 & Keisuke Okamoto 5 & Takaaki Kosugi 6 & Takahiro Kawano 7 & Kengo Fujiki 7 & Hiromichi Kitada 8 & Kinta Hatakeyama 1 & Kazuhiko Tsuruya 3 & Chiho Ohbayashi 1 Received: 1 December 2019 / Revised: 11 March 2020 / Accepted: 18 March 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract IgA nephropathy (IgAN) is common chronic glomerulonephritis with variable prognosis, ranging from minor urinary abnormalities to end-stage renal disease. The revised Oxford classification of IgAN explains that cellular/fibrocellular crescents are associated with poor renal prognosis, proposing an extension to the MEST-C score. C3 immunofluorescent staining follows a distribution similar to IgA staining. Therefore, complement activation was reported to play a pivotal role in IgAN pathogenesis. This study included 132 IgAN patients diagnosed by renal biopsies. The clinical parameters at the time of the biopsies were obtained from patient data records. We classified the patients into C ≥ 1 and C0 groups, and compared clinical, light microscopic, and immunofluorescent features. In the C ≥ 1 group, 2 (1.5%) and 31 (23.5%) patients were assigned to C2 and C1, respectively. The remaining 99 patients (75%) were classified as C0. The C ≥ 1 group had lower average age and rate of hypertension, and higher score of urinary occult blood and E score. The C ≥ 1 group had significantly higher average immunofluorescence scores for IgA, C5b-9, mannoseassociated serine protease (MASP) 1/3, MASP2, properdin, factor B, and kappa. The steroid use rate was significantly higher in the C ≥ 1 group. During the follow-up period of 2.90 years on average, the rate of renal dysfunction was not significantly different between groups. Crescent formation in IgAN was associated with activation of the lectin and alternative pathways. The C ≥ 1 group had significantly increased use of steroids, which probably caused comparable renal function during the follow-up period. Keywords IgA nephropathy . Crescents . Complement . Light chain
Introduction IgA nephropathy (IgAN) is the most common chronic g l o m er u l o n e p h r i t i s c h ar a c t e r i z e d b y m e s an g i a l
deposition of IgA. IgAN is more common in Asia than in Europe and North America. In Japan, 40–60% of patients with chronic glomerulonephritis are diagnosed with IgAN [1].
This article is part of the Topical Collection on Quality in Pathology Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00428-020-02800-0) contains supplementary material, which is available to authorized users. * Hiroe Itami [email protected]
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Department of Nephrology, Nara Prefecture General Medical Center, Nara, Japan
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Department of Nephrology, Saiseikai Suita Hospital, Suita, Japan Department of Nephrology, Nara Prefectural Seiwa Medical Center, Sango, Japan
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