TY - JOUR
T1 - Distinct characteristics and outcomes in elderly-onset IgA vasculitis (Henoch-Schönlein purpura) with nephritis
T2 - Nationwide cohort study of data from the Japan Renal Biopsy Registry (J-RBR)
AU - Komatsu, Hiroyuki
AU - Fujimoto, Shouichi
AU - Maruyama, Shoichi
AU - Mukoyama, Masashi
AU - Sugiyama, Hitoshi
AU - Tsuruya, Kazuhiko
AU - Sato, Hiroshi
AU - Soma, Jun
AU - Yano, Junko
AU - Itano, Seiji
AU - Nishino, Tomoya
AU - Sato, Toshinobu
AU - Narita, Ichiei
AU - Yokoyama, Hitoshi
N1 - Funding Information:
This study was supported in part by the Japanese Society of Nephrology, by a Grant-in-Aid for Intractable Renal Diseases Research, Research on Rare and Intractable Diseases, and by Health and Labour Sciences Research Grants from the Ministry of Health, Labour and Welfare of Japan. The authors greatly acknowledge the help and assistance of our colleagues in the affiliate hospitals with collection of data for the study: Koji Inagaki (Nagoya University), Masataka Adachi (Kumamoto University), Toshio Yamashiro (Okayama University), Hisako Yoshida (Kyushu University), Izaya Nakaya (Iwate Prefectural Central Hospital), Kei Fukami (Kurume University), Tamaki Sasaki (Kawasaki Medical School), Tadashi Uramatsu (Nagasaki University), Yoko Obata (Nagasaki University), Shin Goto (Niigata University).
Publisher Copyright:
© 2018 Komatsu et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
PY - 2018/5
Y1 - 2018/5
N2 - Background The clinical presentation and prognosis of adult and elderly patients with IgA vasculitis (Henoch-Schönlein purpura) accompanied by nephritis (IgAV-N) have not been investigated in detail. We therefore surveyed the features and outcomes of IgAV-N based on nationwide data derived from the Japan Renal Biopsy Registry (J-RBR). Methods This multi-center cohort study compared the clinicopathological parameters at diagnosis, initial therapies and outcomes between 106 adult (age 19–64 years) and 46 elderly (65 years) patients with IgAV-N who were registered in the J-RBR between 2007 and 2012. The primary end-points comprised a 50% increase in serum creatinine (sCr) values or end-stage kidney disease. Factors affecting a decrease in renal function were assessed using Cox proportional hazards models. Results Rates of hypertension, impaired renal function, hypoalbuminemia and crescentic glomerulonephritis were significantly higher among the elderly, than the adult patients. About 80% and 60% of the patients in both groups were respectively treated with corticosteroid and a renin-angiotensin system (RAS) blockade. Both groups had favorable renal survival rates for nine years (93.6% and 91.4% of the adult and elderly patients, respectively). Significantly more elderly than adult patients developed a 50% increase in sCr during a mean observation period of 3.9 years (21.7% vs. 4.7%, p = 0.012), and significantly fewer elderly, than adult patients achieved clinical remission (23.9% vs. 46.2%, p = 0.016). Multivariate analysis selected advanced age (65 years) and lower serum albumin values as independent prognostic factors for a decline in renal function, whereas steroid pulse therapy helped to preserve renal function. Conclusions The renal prognosis of adult and elderly patients with IgAV-N was favorable when treated aggressively with corticosteroid and RAS blockade. However, the course of renal function should be carefully monitored in patients aged over 65 years and those with hypoalbuminemia.
AB - Background The clinical presentation and prognosis of adult and elderly patients with IgA vasculitis (Henoch-Schönlein purpura) accompanied by nephritis (IgAV-N) have not been investigated in detail. We therefore surveyed the features and outcomes of IgAV-N based on nationwide data derived from the Japan Renal Biopsy Registry (J-RBR). Methods This multi-center cohort study compared the clinicopathological parameters at diagnosis, initial therapies and outcomes between 106 adult (age 19–64 years) and 46 elderly (65 years) patients with IgAV-N who were registered in the J-RBR between 2007 and 2012. The primary end-points comprised a 50% increase in serum creatinine (sCr) values or end-stage kidney disease. Factors affecting a decrease in renal function were assessed using Cox proportional hazards models. Results Rates of hypertension, impaired renal function, hypoalbuminemia and crescentic glomerulonephritis were significantly higher among the elderly, than the adult patients. About 80% and 60% of the patients in both groups were respectively treated with corticosteroid and a renin-angiotensin system (RAS) blockade. Both groups had favorable renal survival rates for nine years (93.6% and 91.4% of the adult and elderly patients, respectively). Significantly more elderly than adult patients developed a 50% increase in sCr during a mean observation period of 3.9 years (21.7% vs. 4.7%, p = 0.012), and significantly fewer elderly, than adult patients achieved clinical remission (23.9% vs. 46.2%, p = 0.016). Multivariate analysis selected advanced age (65 years) and lower serum albumin values as independent prognostic factors for a decline in renal function, whereas steroid pulse therapy helped to preserve renal function. Conclusions The renal prognosis of adult and elderly patients with IgAV-N was favorable when treated aggressively with corticosteroid and RAS blockade. However, the course of renal function should be carefully monitored in patients aged over 65 years and those with hypoalbuminemia.
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U2 - 10.1371/journal.pone.0196955
DO - 10.1371/journal.pone.0196955
M3 - Article
C2 - 29738576
AN - SCOPUS:85046640783
VL - 13
JO - PLoS One
JF - PLoS One
SN - 1932-6203
IS - 5
M1 - e0196955
ER -