Pathogenesis and Treatment of Refractory Disease Courses in Systemic Juvenile Idiopathic Arthritis: Refractory Arthritis, Recurrent Macrophage Activation Syndrome and Chronic Lung Disease
Erkens, Remco; Esteban, Ysabella; Towe, Christopher; Schulert, Grant; Vastert, Sebastiaan
(2021) Rheumatic Disease Clinics of North America, volume 47, issue 4, pp. 585 - 606
(Article)
Abstract
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
... read more
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.
show less
Download/Full Text
The full text of this publication is not available.
Keywords: Lung disease, Macrophage activation syndrome, Refractory disease, Systemic JIA, Translational research, Biological Therapy, Macrophage Activation Syndrome/diagnosis, Arthritis, Juvenile/complications, Humans, Lung Diseases, Child, Rheumatology, Review, Journal Article, Research Support, N.I.H., Extramural
ISSN: 0889-857X
Publisher: W.B. Saunders Ltd
Note: 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.
(Peer reviewed)