Onset age is a risk factor for refractory pediatric IgA vasculitis: a retrospective cohort study

Abstract Background Though outcome differences between children and adults with immunoglobulin A vasculitis (IgAV) has been well documented, it remains unclear if disease features in pediatric IgAV patients vary with onset age. We aimed to explore clinical features and prognosis of pediatric IgAV st...

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Main Authors: Chun-Hua Liao, Melody Tsai, Yao-Hsu Yang, Bor-Luen Chiang, Li-Chieh Wang
Format: Article
Language:English
Published: BMC 2020-11-01
Series:Pediatric Rheumatology Online Journal
Subjects:
Online Access:http://link.springer.com/article/10.1186/s12969-020-00480-3
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spelling doaj-dbef945b7fd8404791e6d4b909b367462020-11-25T04:02:18ZengBMCPediatric Rheumatology Online Journal1546-00962020-11-0118111010.1186/s12969-020-00480-3Onset age is a risk factor for refractory pediatric IgA vasculitis: a retrospective cohort studyChun-Hua Liao0Melody Tsai1Yao-Hsu Yang2Bor-Luen Chiang3Li-Chieh Wang4Department of Pediatrics, National Taiwan University HospitalDepartment of Pediatrics, National Taiwan University HospitalDepartment of Pediatrics, National Taiwan University HospitalDepartment of Pediatrics, National Taiwan University HospitalDepartment of Pediatrics, National Taiwan University HospitalAbstract Background Though outcome differences between children and adults with immunoglobulin A vasculitis (IgAV) has been well documented, it remains unclear if disease features in pediatric IgAV patients vary with onset age. We aimed to explore clinical features and prognosis of pediatric IgAV stratified by onset age. Methods We retrospectively reviewed records of patients under 18 years old diagnosed with IgAV from January 1999 to December 2018 in one tertiary medical center in Taiwan. Patients were grouped by onset age: ≤ 6 years old, 6–12 years old (> 6, ≤ 12), and 12–18 years old (> 12, < 18). Demographics, laboratory data, incidence of gastrointestinal, renal, and joint involvement, corticosteroid dependence, recurrence, and refractory disease were analyzed. Recurrence was defined as disease flare-up after complete remission and discontinuation of all medications for at least 3 months. Corticosteroid dependence was defined by more than 6 weeks of daily oral corticosteroid intake. Refractory disease was defined as not achieving complete remission 6 months after disease onset. Statistical analysis was performed using R software (v3.6.0). Results There were 484 IgAV patients, with an onset age of 6.10 (4.72–8.58) (median (IQR)) years old. There were 234 (48.3%) patients ≤6 years old, 210 (43.4%) 6–12 years old, and 40 (8.3%) 12–18 years old. One hundred and thirty (26.9%) patients had renal involvement, which was more frequent in older children (≤ 6 years old, 18.4%; 6–12 years old, 31.0%; 12–18 years old, 55.0%; p <  0.001). There were 361 patients (74.6%) with joint involvement; younger children were affected more frequently (≤ 6 years old, 82.1%; 6–12 years old, 71.9%; 12–18 years old, 45.0%; p <  0.001). Gastrointestinal involvement was present in 311 (64.3%) patients, showing no difference among age groups. There were 46 patients (9.5%) with recurrent IgA vasculitis, 136 (28.1%) with corticosteroid dependent and 76 (15.7%) with refractory disease. Corticosteroid dependence and refractory disease occurred more frequently as onset age increased (p <  0.001). Conclusion Pediatric IgAV with different onset ages are associated with distinct clinical manifestations and outcomes. The risk of developing corticosteroid dependence, refractory disease and renal involvement increased with onset age.http://link.springer.com/article/10.1186/s12969-020-00480-3Pediatric IgA vasculitisOnset ageCorticosteroid dependenceRefractoryRenal involvement
collection DOAJ
language English
format Article
sources DOAJ
author Chun-Hua Liao
Melody Tsai
Yao-Hsu Yang
Bor-Luen Chiang
Li-Chieh Wang
spellingShingle Chun-Hua Liao
Melody Tsai
Yao-Hsu Yang
Bor-Luen Chiang
Li-Chieh Wang
Onset age is a risk factor for refractory pediatric IgA vasculitis: a retrospective cohort study
Pediatric Rheumatology Online Journal
Pediatric IgA vasculitis
Onset age
Corticosteroid dependence
Refractory
Renal involvement
author_facet Chun-Hua Liao
Melody Tsai
Yao-Hsu Yang
Bor-Luen Chiang
Li-Chieh Wang
author_sort Chun-Hua Liao
title Onset age is a risk factor for refractory pediatric IgA vasculitis: a retrospective cohort study
title_short Onset age is a risk factor for refractory pediatric IgA vasculitis: a retrospective cohort study
title_full Onset age is a risk factor for refractory pediatric IgA vasculitis: a retrospective cohort study
title_fullStr Onset age is a risk factor for refractory pediatric IgA vasculitis: a retrospective cohort study
title_full_unstemmed Onset age is a risk factor for refractory pediatric IgA vasculitis: a retrospective cohort study
title_sort onset age is a risk factor for refractory pediatric iga vasculitis: a retrospective cohort study
publisher BMC
series Pediatric Rheumatology Online Journal
issn 1546-0096
publishDate 2020-11-01
description Abstract Background Though outcome differences between children and adults with immunoglobulin A vasculitis (IgAV) has been well documented, it remains unclear if disease features in pediatric IgAV patients vary with onset age. We aimed to explore clinical features and prognosis of pediatric IgAV stratified by onset age. Methods We retrospectively reviewed records of patients under 18 years old diagnosed with IgAV from January 1999 to December 2018 in one tertiary medical center in Taiwan. Patients were grouped by onset age: ≤ 6 years old, 6–12 years old (> 6, ≤ 12), and 12–18 years old (> 12, < 18). Demographics, laboratory data, incidence of gastrointestinal, renal, and joint involvement, corticosteroid dependence, recurrence, and refractory disease were analyzed. Recurrence was defined as disease flare-up after complete remission and discontinuation of all medications for at least 3 months. Corticosteroid dependence was defined by more than 6 weeks of daily oral corticosteroid intake. Refractory disease was defined as not achieving complete remission 6 months after disease onset. Statistical analysis was performed using R software (v3.6.0). Results There were 484 IgAV patients, with an onset age of 6.10 (4.72–8.58) (median (IQR)) years old. There were 234 (48.3%) patients ≤6 years old, 210 (43.4%) 6–12 years old, and 40 (8.3%) 12–18 years old. One hundred and thirty (26.9%) patients had renal involvement, which was more frequent in older children (≤ 6 years old, 18.4%; 6–12 years old, 31.0%; 12–18 years old, 55.0%; p <  0.001). There were 361 patients (74.6%) with joint involvement; younger children were affected more frequently (≤ 6 years old, 82.1%; 6–12 years old, 71.9%; 12–18 years old, 45.0%; p <  0.001). Gastrointestinal involvement was present in 311 (64.3%) patients, showing no difference among age groups. There were 46 patients (9.5%) with recurrent IgA vasculitis, 136 (28.1%) with corticosteroid dependent and 76 (15.7%) with refractory disease. Corticosteroid dependence and refractory disease occurred more frequently as onset age increased (p <  0.001). Conclusion Pediatric IgAV with different onset ages are associated with distinct clinical manifestations and outcomes. The risk of developing corticosteroid dependence, refractory disease and renal involvement increased with onset age.
topic Pediatric IgA vasculitis
Onset age
Corticosteroid dependence
Refractory
Renal involvement
url http://link.springer.com/article/10.1186/s12969-020-00480-3
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