Distinct characteristics and outcomes in elderly-onset IgA vasculitis (Henoch-Schönlein purpura) with nephritis: Nationwide cohort study of data from the Japan Renal Biopsy Registry (J-RBR)

Hiroyuki Komatsu, Shouichi Fujimoto, Shoichi Maruyama, Masashi Mukoyama, Hitoshi Sugiyama, Kazuhiko Tsuruya, Hiroshi Sato, Jun Soma, Junko Yano, Seiji Itano, Tomoya Nishino, Toshinobu Sato, Ichiei Narita, Hitoshi Yokoyama

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Article numbere0196955
JournalPLoS One
Volume13
Issue number5
DOIs
Publication statusPublished - May 1 2018

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Schoenlein-Henoch Purpura
vasculitis
nephritis
Nephritis
Biopsy
Vasculitis
cohort studies
Immunoglobulin A
Registries
biopsy
Japan
Cohort Studies
kidneys
Kidney
renal function
Angiotensins
Renin
Creatinine
Adrenal Cortex Hormones
renin-angiotensin system

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Agricultural and Biological Sciences(all)

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Distinct characteristics and outcomes in elderly-onset IgA vasculitis (Henoch-Schönlein purpura) with nephritis : Nationwide cohort study of data from the Japan Renal Biopsy Registry (J-RBR). / Komatsu, Hiroyuki; Fujimoto, Shouichi; Maruyama, Shoichi; Mukoyama, Masashi; Sugiyama, Hitoshi; Tsuruya, Kazuhiko; Sato, Hiroshi; Soma, Jun; Yano, Junko; Itano, Seiji; Nishino, Tomoya; Sato, Toshinobu; Narita, Ichiei; Yokoyama, Hitoshi.

In: PLoS One, Vol. 13, No. 5, e0196955, 01.05.2018.

Research output: Contribution to journalArticle

Komatsu, H, Fujimoto, S, Maruyama, S, Mukoyama, M, Sugiyama, H, Tsuruya, K, Sato, H, Soma, J, Yano, J, Itano, S, Nishino, T, Sato, T, Narita, I & Yokoyama, H 2018, 'Distinct characteristics and outcomes in elderly-onset IgA vasculitis (Henoch-Schönlein purpura) with nephritis: Nationwide cohort study of data from the Japan Renal Biopsy Registry (J-RBR)', PLoS One, vol. 13, no. 5, e0196955. https://doi.org/10.1371/journal.pone.0196955
Komatsu, Hiroyuki ; Fujimoto, Shouichi ; Maruyama, Shoichi ; Mukoyama, Masashi ; Sugiyama, Hitoshi ; Tsuruya, Kazuhiko ; Sato, Hiroshi ; Soma, Jun ; Yano, Junko ; Itano, Seiji ; Nishino, Tomoya ; Sato, Toshinobu ; Narita, Ichiei ; Yokoyama, Hitoshi. / Distinct characteristics and outcomes in elderly-onset IgA vasculitis (Henoch-Schönlein purpura) with nephritis : Nationwide cohort study of data from the Japan Renal Biopsy Registry (J-RBR). In: PLoS One. 2018 ; Vol. 13, No. 5.
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abstract = "Background The clinical presentation and prognosis of adult and elderly patients with IgA vasculitis (Henoch-Sch{\"o}nlein 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.",
author = "Hiroyuki Komatsu and Shouichi Fujimoto and Shoichi Maruyama and Masashi Mukoyama and Hitoshi Sugiyama and Kazuhiko Tsuruya and Hiroshi Sato and Jun Soma and Junko Yano and Seiji Itano and Tomoya Nishino and Toshinobu Sato and Ichiei Narita and Hitoshi Yokoyama",
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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

PY - 2018/5/1

Y1 - 2018/5/1

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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