Abstract
Background: Systemic Juvenile Idiopathic Arthritis (SJIA)/Pediatric Still’s disease is associated with different phenotypes and outcomes from currently available treatments. Methods: A review of opinion, based on personal experience in a reference pediatric rheumatology center and key publications, to explore the most important questions regarding disease heterogeneity and treatment approaches. Results: A few situations deserve particular attention: 1/patients with recent-onset SJIA who may benefit from a treat-to-target approach with a key place for interleukin (IL)-1 inhibition; 2/SJIA patients refractory to Il-1 and IL-6 antagonists in whom several options may be discussed, including thalidomide or allogeneic hematopoietic stem cell transplantation; 3/SJIA patients with macrophage activation syndrome who may benefit from both well-used classical treatment and innovative approaches, such as anti-interferon gamma therapy or Janus Kinase (JAK) inhibitors; 4/SJIA with severe lung involvement, 5/SJIA patients who achieve complete remission on treatment, with some recent evidence that treatment may be reduced in intensity but not so easily withdrawn. Conclusions: a case-by-case discussion with expert teams is recommended in this heterogeneous, often difficult-to-treat population of patients.
Highlights
Systemic Juvenile Idiopathic Arthritis (SJIA) is defined according to the current International League of Associations for Rheumatology (ILAR) as a disease starting before the age of 16 years, active for at least 6 weeks, with at least 15 days of fever, a peculiar spiking fever pattern, arthritis, and at least 2 of the following features: skin rash, serositis, lymphadenopathy, hepatomegaly, or splenomegaly [1]
There is a continuum with Adult-Onset Still’s Disease (AoSD), which diagnosis can be made in patients without arthritis [15,16]
Several publications emphasize that some situations in SJIA patients deserve peculiar attention [5,18,19,26,27]:
Summary
Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affiliations. Less stringent diagnosis criteria were proposed to allow earlier diagnosis and treatment [2]. These new diagnosis criteria are undergoing a validation process. A provisional diagnosis of SJIA could be made after two weeks in a patient with typical systemic features and arthralgia, even in the absence of arthritis. There is a continuum with Adult-Onset Still’s Disease (AoSD), which diagnosis can be made in patients without arthritis [15,16] In both SJIA and AoSD, some patients develop life-threatening complications such as MAS or secondary amyloidosis [17]. Recent efforts may allow the distinction of some subpopulations of SJIA patients who are at higher risk of severe complications and may deserve specific therapeutic approaches. The author proposes to give his understanding of some key differences between different subpopulations of SJIA patients and their implications for patients’ prognosis and treatment
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