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

Table 1.

Peculiar situations.

Clinical Situations Main Questions
Recent-onset SJIA symptoms Differential diagnosis:
Macrophage activation
Syndrome (MAS)
  • -

    Infections

  • -

    Post-infectious (Covid19, …)

  • -

    Vasculitis (Kawasaki, PAN, …)

  • -

    Inflammatory bowel disease

  • -

    Autoimmune disease (lupus, hepatitis, …)

  • -

    Monogenic autoinflammatory syndrome

  • -

    Neoplasia (rare)

Therapeutic emergency 1:
  • -

    Macrophage activation syndrome

  • -

    Dyspnea on arythenoid arthritis (rare)

  • -

    Myocarditis, marked pericarditis

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    Marked inflammation and pain

  • -

    Early, diffuse polyarthritis

Differential diagnosis:
  • -

    Infection (EBV, Leishmania …)

  • -

    ±monogenic immune disease (familial lymphohistiocytosis, Purtilo’s syndrome … or combined immunodeficiency) 1

  • -

    Neoplasia (T-, NK-cell lymphoma, …)

SJIA subtype at risk of lung disease:
  • -

    Early-onset SJIA, recurrent MAS

  • -

    ±chromosomal abnormality (trisomy 21 …)

  • -

    “hypersensitivity” to biologics

Urgent, ±intensive treatment 1:

SJIA, systemic juvenile idiopathic arthritis; PAN, periarteritis nodosa; Epstein–Barr virus. 1 case by case discussion with an expert center recommended.