Pediatric systemic juvenile idiopathic arthritis-related lung disease: Description of clinical cohort and review of management.
Pediatr Pulmonol
; 59(3): 724-733, 2024 Mar.
Article
em En
| MEDLINE
| ID: mdl-38155594
ABSTRACT
CONTEXT Since the early 2010s, there has been an increased awareness of interstitial lung disease in systemic juvenile idiopathic arthritis (sJIA-LD) in pediatric patients. Despite the increase in prevalence of sJIA-LD, little is known about this disease process and effective therapeutic management. OBJECTIVES:
To identify and characterize the disease process and management of interstitial lung disease related to systemic juvenile idiopathic arthritis. STUDYDESIGN:
In this single-center, retrospective case series of 9 patients, we analyze demographic, clinical, radiographic, and laboratory data to corroborate common clinical characteristics and describe an approach for diagnosis and monitoring of sJIA-LD. DATA EXTRACTION All data was extracted through electronic medical records and individually reviewed by two pediatric pulmonologists and two pediatric rheumatologists.RESULTS:
Our results were similar to other described cases of sJIA-LD as patients in our cohort were more likely to be younger, have a history of macrophage activation syndrome and prior use of biologic therapies. In contrast to prior studies, they did not present with peripheral lymphadenopathy and hepatosplenomegaly.LIMITATIONS:
The cohort size was small and data is reflective of one center's approach to management of a rare lung disease process.CONCLUSION:
Interstitial lung disease due to sJIA is rare and management can be difficult in these complex patients.More research is necessary to understand the increased incidence and treatment of sJIA-LD in pediatric population.Palavras-chave
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Artrite Juvenil
/
Doenças Pulmonares Intersticiais
/
Síndrome de Ativação Macrofágica
Idioma:
En
Ano de publicação:
2024
Tipo de documento:
Article
País de afiliação:
Estados Unidos