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Arduini, A., Marasco, E., Marucci, G., Pardeo, M., Insalaco, A., Caiello, I., et al. (2019) An Unusual Presentation of Purine Nucleoside Phosphorylase Deficiency Mimicking Systemic Juvenile Idiopathic Arthritis Complicated by Macrophage Activation Syndrome. Pediatric Rheumatology, 17, Article No. 25.
https://doi.org/10.1186/s12969-019-0328-3

has been cited by the following article:

• TITLE: Current Review of Systemic Juvenile Idiopathic Arthritis: What Do Paediatricians Need to Know?

  AUTHORS: Asma R. Albaker

  KEYWORDS: Juvenile Idiopathic Arthritis, Systemic Juvenile Idiopathic Arthritis, Autoinflammatory, Arthritis, Biologics

  JOURNAL NAME: Open Journal of Pediatrics, Vol.10 No.4, December 31, 2020

  ABSTRACT: Systemic juvenile idiopathic arthritis is classified as an autoimmune entity and a subtype of juvenile idiopathic arthritis, although it has many features of autoinflammatory-type of diseases. This review article will elaborate on the disease’s pathogenesis and its proposed relation to autoinflammatory diseases including defective innate immunity and phagocytosis response leading excessive cytokine release. It also explains the disease’s epidemiology, clinical phenotype, diagnostic challenges, complications and current advancements in the treatment of systemic juvenile idiopathic arthritis, such as IL-1 and IL-6 antagonists and their impact on the disease trajectory. Care of patients with systemic juvenile idiopathic arthritis requires a comprehensive multidisciplinary team to optimize the care and avoid complications of the disease itself such as growth impairment, macrophage activation syndrome or the complications of immunosuppressant and immunomodulatory treatments.