Age-dependent survival in rapidly progressive glomerulonephritis: A nationwide questionnaire survey from children to the elderly.

Eri Muso,Hitoshi Sugiyama,Shuzo Kaneko,Pasqual Barretti,Seiichi Matsuo,Takashi Wada,Yoshihiro Arimura,Kunihiro Yamagata,Hirofumi Makino,Mayumi Takahashi-Kobayashi,Kosaku Nitta,Joichi Usui

doi:10.1371/journal.pone.0236017

Abstract

BackgroundRapidly progressive glomerulonephritis (RPGN) has been known to have a poor prognosis. Although evidence across adult RPGN cases has accumulated over many years, the number of case series in adolescents and young adults has been limited, requiring further studies.MethodsA total of 1,766 cases from 1989 to 2007 were included in this nationwide questionnaire survey, led by Intractable (former name, Progressive) Renal Diseases Research, Research on intractable disease, from the Ministry of Health, Labour and Welfare of Japan. To elucidate age-related differences in 2-year patient and renal survival rates, the cases were divided into the following four groups: children (0–18 years), young adults (19–39 years), the middle-aged (40–64 years), and the elderly (over 65 years).ResultsOf the 1,766 total RPGN cases, antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis comprised 1,128 cases (63.9% of all RPGN cases), showing a tendency to increase with age. Two-year patient survival for RPGN was 93.9% among children, 92.6% in young adults, 83.2% in the middle-aged, and 68.8% in the elderly. The younger group (children plus young adults) showed a clearly higher survival rate compared to the older group (middle-aged plus elderly) (p<0.05). ANCA-associated glomerulonephritis also showed similar age-related results with all RPGN cases. The comparison of renal prognosis showed no statistically significant differences both in RPGN and in ANCA-associated GN.ConclusionThe present study described the age-dependent characteristics of the classification of RPGN, especially focusing on a better prognosis of the younger group in patient survival both in RPGN and in ANCA-associated GN.

Highlights

Progressive glomerulonephritis (RPGN) is characterized by clinical features of progressive deterioration of renal function and glomerulonephritis, accompanied by active urinary sediment and crescent formation in histopathology [1]
Of the 1,766 total Rapidly progressive glomerulonephritis (RPGN) cases, antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis comprised 1,128 cases (63.9% of all RPGN cases), showing a tendency to increase with age
The present study described the age-dependent characteristics of the classification of RPGN, especially focusing on a better prognosis of the younger group in patient survival both in RPGN and in ANCA-associated GN

Summary

Introduction

Progressive glomerulonephritis (RPGN) is characterized by clinical features of progressive deterioration of renal function and glomerulonephritis, accompanied by active urinary sediment and crescent formation in histopathology [1]. As the previous reports on this topic have mainly focused on adults, the studied cases in the relatively young population including children have been limited in number. In this context, age-based analysis, especially in pediatric and young adult cases, was determined to be the main purpose of this study. Progressive glomerulonephritis (RPGN) has been known to have a poor prognosis. Evidence across adult RPGN cases has accumulated over many years, the number of case series in adolescents and young adults has been limited, requiring further studies

Methods

Results

Conclusion