Quartier Pierre
Unité d'Immunologie-Hématologie et Rhumatologie Pédiatrique, RAISE Reference Centre, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, 149 Rue de Sèvres, 75015 Paris, France.
INSERM 1231, Université de Paris, 45 Rue des Saints-Pères, 75006 Paris, France.
J Clin Med. 2022 Mar 1;11(5):1357. doi: 10.3390/jcm11051357.
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complete remission on treatment, with some recent evidence that treatment may be reduced in intensity but not so easily withdrawn.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.
全身型幼年特发性关节炎（SJIA）/儿童斯蒂尔病具有不同的表型，且现有治疗方法的疗效各异。
基于参考儿科风湿病中心的个人经验及关键出版物进行观点综述，以探讨有关疾病异质性和治疗方法的最重要问题。
有几种情况值得特别关注：1/近期发病的SJIA患者可能受益于以白细胞介素（IL）-1抑制为关键的达标治疗方法；2/对IL-1和IL-6拮抗剂难治的SJIA患者，可讨论多种选择，包括沙利度胺或异基因造血干细胞移植；3/患有巨噬细胞活化综合征的SJIA患者可能受益于常用的经典治疗方法和创新方法，如抗干扰素γ治疗或 Janus激酶（JAK）抑制剂；4/伴有严重肺部受累的SJIA；5/治疗后实现完全缓解的SJIA患者，近期有证据表明治疗强度可降低，但不易停药。
对于这类异质性强、通常难以治疗的患者群体，建议与专家团队进行逐案讨论。
