Atypical Presentation of Systemic Arthritis in a Toddler with Down Syndrome

Systemic juvenile idiopathic arthritis (sJIA) is a chronic, inflammatory disease of childhood, which is characterized by the combination of arthritis, serositis, daily, high-spiking fevers, and evanescent macular rash and can present with the life-threatening complication of macrophage activation sy...

Full description

Saved in:
Bibliographic Details
Main Authors: Emily Worley, Weijie Li, Jordan T. Jones
Format: Article
Language:English
Published: Wiley 2021-01-01
Series:Case Reports in Pediatrics
Online Access:http://dx.doi.org/10.1155/2021/6567770
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1841524688158720000
author Emily Worley
Weijie Li
Jordan T. Jones
author_facet Emily Worley
Weijie Li
Jordan T. Jones
author_sort Emily Worley
collection DOAJ
description Systemic juvenile idiopathic arthritis (sJIA) is a chronic, inflammatory disease of childhood, which is characterized by the combination of arthritis, serositis, daily, high-spiking fevers, and evanescent macular rash and can present with the life-threatening complication of macrophage activation syndrome (MAS). Children with Down syndrome (DS) have complex medical challenges related to abnormalities in their immune system, which can cause a broad spectrum of disease manifestations, which can occur atypically. Children with DS are at increased risk for arthritis and interstitial lung disease (ILD) associated with sJIA that has high mortality. This case report outlines an atypical presentation of sJIA in a 21-month-old male with DS in which fever was not part of the initial presentation of sJIA and then later developed MAS and ILD. Due to broad spectrum of disease and atypical presentation in children with DS, this case report was created to increase awareness of atypical presentations of rheumatic disease in children with DS.
format Article
id doaj-art-7ef7c0a91e3947c991ab1b453319c22e
institution Kabale University
issn 2090-6803
2090-6811
language English
publishDate 2021-01-01
publisher Wiley
record_format Article
series Case Reports in Pediatrics
spelling doaj-art-7ef7c0a91e3947c991ab1b453319c22e2025-02-03T05:47:38ZengWileyCase Reports in Pediatrics2090-68032090-68112021-01-01202110.1155/2021/65677706567770Atypical Presentation of Systemic Arthritis in a Toddler with Down SyndromeEmily Worley0Weijie Li1Jordan T. Jones2University of Kansas School of Medicine, Kansas City, KS, USAChildren’s Mercy Kansas City, Kansas City, MO, USAUniversity of Kansas School of Medicine, Kansas City, KS, USASystemic juvenile idiopathic arthritis (sJIA) is a chronic, inflammatory disease of childhood, which is characterized by the combination of arthritis, serositis, daily, high-spiking fevers, and evanescent macular rash and can present with the life-threatening complication of macrophage activation syndrome (MAS). Children with Down syndrome (DS) have complex medical challenges related to abnormalities in their immune system, which can cause a broad spectrum of disease manifestations, which can occur atypically. Children with DS are at increased risk for arthritis and interstitial lung disease (ILD) associated with sJIA that has high mortality. This case report outlines an atypical presentation of sJIA in a 21-month-old male with DS in which fever was not part of the initial presentation of sJIA and then later developed MAS and ILD. Due to broad spectrum of disease and atypical presentation in children with DS, this case report was created to increase awareness of atypical presentations of rheumatic disease in children with DS.http://dx.doi.org/10.1155/2021/6567770
spellingShingle Emily Worley
Weijie Li
Jordan T. Jones
Atypical Presentation of Systemic Arthritis in a Toddler with Down Syndrome
Case Reports in Pediatrics
title Atypical Presentation of Systemic Arthritis in a Toddler with Down Syndrome
title_full Atypical Presentation of Systemic Arthritis in a Toddler with Down Syndrome
title_fullStr Atypical Presentation of Systemic Arthritis in a Toddler with Down Syndrome
title_full_unstemmed Atypical Presentation of Systemic Arthritis in a Toddler with Down Syndrome
title_short Atypical Presentation of Systemic Arthritis in a Toddler with Down Syndrome
title_sort atypical presentation of systemic arthritis in a toddler with down syndrome
url http://dx.doi.org/10.1155/2021/6567770
work_keys_str_mv AT emilyworley atypicalpresentationofsystemicarthritisinatoddlerwithdownsyndrome
AT weijieli atypicalpresentationofsystemicarthritisinatoddlerwithdownsyndrome
AT jordantjones atypicalpresentationofsystemicarthritisinatoddlerwithdownsyndrome