biostudies-literatureUnknown11(5)Quartier PSystemic Juvenile Idiopathic Arthritis (SJIA)/Pediatric Still's disease is associated with different phenotypes and outcomes from currently available treatments. 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. 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complete remission on treatment, with some recent evidence that treatment may be reduced in intensity but not so easily withdrawn. a case-by-case discussion with expert teams is recommended in this heterogeneous, often difficult-to-treat population of patients.Journal of clinical medicine1357https://www.ebi.ac.uk/biostudies/studies/S-EPMC8911482biostudies-literatureSystemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches.PMC8911482Quartier PfalseSystemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches.Systemic Juvenile Idiopathic Arthritis (SJIA)/Pediatric Still's disease is associated with different phenotypes and outcomes from currently available treatments. 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. 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. a case-by-case discussion with expert teams is recommended in this heterogeneous, often difficult-to-treat population of patients.2022-01-01T00:00:00Z2022 Mar2025-04-18T12:12:22.351Z2025-04-06T21:48:42.785ZS-EPMC89114823526844910.3390/jcm11051357