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

Table 2.

Therapeutic approaches 1.

Clinical Situations Classical Approach Recent Approaches
Recent-onset SJIA with auto-inflammatory syndrome ±NSAIDs first Anti-IL-1 treatment 2
High-dose steroids or anti-IL-6 treatment
±lower-dose steroids
Long-lasting systemic inflammation with limited joint involvement Anti-IL-1 treatment
Anti-IL-6 treatment		 JAK-inhibitor
±NSAID or low-dose steroids In refractory cases, discuss:
  • -

    thalidomide

  • -

    allogeneic hematopoietic stem cell transplantation
Severe MAS flare in a SJIA patient High-dose steroids
±cyclosporin		 High-dose steroids associated with:
  • -

    high-dose anakinra (±IV)
±etoposide
  • -

    or anti-IFNγ (emapalumab) ± anakinra or another SJIA treatment 3

  • -

    or a JAK-inhibitor
Remitting-relapsing MAS Steroids ± cyclosporine JAK-inhibitor 4, steroids.
In refractory cases, discuss:
  • -

    anti-INFγ (emapalumab)

  • -

    allogeneic hematopoietic stem cell transplantation
Diffuse polyarthritis Anti-TFN treatment
Anti-IL-1 treatment
Anti-IL-6 treatment		 Anti-IL-6 treatment (more evidence- based medicine than for other biologics or JAK-inhibitors)
±methotrexate ± low-dose steroids ±methotrexate ±low-dose steroids
In refractory cases, discuss allogeneic hematopoietic stem cell transplantation

SJIA, systemic juvenile idiopathic arthritis; NSAID, non-steroidal anti-inflammatory drug; IL, interleukin; JAK, janus kinase; MAS, macrophage activation syndrome; IFN, interferon. 1 case by case discussion with an expert center recommended. 2 more evidence-based medicine for anti-IL-1 treatment and in particular anakinra in this situation. 3 anti-IFNγ treatment may help controlling MAS but not the underlying systemic disease that may need other therapy in association. 4 may be active both on MAS and on the underlying systemic disease.