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. 2022 Mar 1;11(5):1357.
doi: 10.3390/jcm11051357.

Systemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches

Pierre Quartier  1   2
Affiliations

Systemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches

Pierre Quartier. J Clin Med. .

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complete remission on treatment, with some recent evidence that treatment may be reduced in intensity but not so easily withdrawn.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.

Keywords: Janus Kinase antagonists; Still’s disease; Systemic Juvenile Idiopathic Arthritis; allogeneic hematopoietic stem cell transplantation; biotherapy; interleukin-1; interleukin-18; interleukin-6; interstitial lung disease; macrophage activation syndrome.

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Conflict of interest statement

The author received consultancy or speaking fees from AbbVie, Bristol-Myers Squibb, Chugai-Roche, Lilly, Novartis, Novimmune, Pfizer and Swedish Orphan Biovitrum; and participated in a data safety monitoring board for Sanofi.

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