TY - JOUR
T1 - Pathogenesis and Treatment of Refractory Disease Courses in Systemic Juvenile Idiopathic Arthritis
T2 - Refractory Arthritis, Recurrent Macrophage Activation Syndrome and Chronic Lung Disease
AU - Erkens, Remco
AU - Esteban, Ysabella
AU - Towe, Christopher
AU - Schulert, Grant
AU - Vastert, Sebastiaan
N1 - Funding Information:
The authors thank Dr Kathryn Wikenheiser-Brokamp, Division of Pathology and Laboratory Medicine, Cincinnati Children’s Hospital Medical Center and University of Cincinnati College of Medicine, for providing histopathological images. Dr. Schulert was supported by the National Institutes of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health (K08-AR072075).
Funding Information:
The authors thank Dr Kathryn Wikenheiser-Brokamp, Division of Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine, for providing histopathological images. Dr. Schulert was supported by the National Institutes of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health (K08-AR072075). Dr. Schulert has received consulting fees from Novartis.
PY - 2021/11
Y1 - 2021/11
N2 - Systemic juvenile idiopathic arthritis is a distinct and heterogeneous disease presently classified under the umbrella of juvenile idiopathic arthritis, with some patients following a monophasic remitting course, whereas others have persistent disease with chronic organ- and life-threatening complications. Although biologic therapies have revolutionized treatment, recent follow-up studies report significant numbers of children with persistently active disease on long term follow-up. This review focuses on refractory disease courses, specifically refractory arthritis, systemic juvenile idiopathic arthritis with recurrent, or longstanding signs of macrophage activation syndrome, and systemic juvenile idiopathic arthritis associated with suspected, probable, or definite lung disease.
AB - Systemic juvenile idiopathic arthritis is a distinct and heterogeneous disease presently classified under the umbrella of juvenile idiopathic arthritis, with some patients following a monophasic remitting course, whereas others have persistent disease with chronic organ- and life-threatening complications. Although biologic therapies have revolutionized treatment, recent follow-up studies report significant numbers of children with persistently active disease on long term follow-up. This review focuses on refractory disease courses, specifically refractory arthritis, systemic juvenile idiopathic arthritis with recurrent, or longstanding signs of macrophage activation syndrome, and systemic juvenile idiopathic arthritis associated with suspected, probable, or definite lung disease.
KW - Lung disease
KW - Macrophage activation syndrome
KW - Refractory disease
KW - Systemic JIA
KW - Translational research
KW - Biological Therapy
KW - Macrophage Activation Syndrome/diagnosis
KW - Arthritis, Juvenile/complications
KW - Humans
KW - Lung Diseases
KW - Child
UR - http://www.scopus.com/inward/record.url?scp=85113338544&partnerID=8YFLogxK
U2 - 10.1016/j.rdc.2021.06.003
DO - 10.1016/j.rdc.2021.06.003
M3 - Review article
C2 - 34635293
AN - SCOPUS:85113338544
SN - 0889-857X
VL - 47
SP - 585
EP - 606
JO - Rheumatic Disease Clinics of North America
JF - Rheumatic Disease Clinics of North America
IS - 4
ER -