The following sections outline the recommended approach to evaluating pediatric patients with fever.

Important Considerations in Evaluation

Many rheumatic diseases are diagnoses of exclusion AND will need glucocorticoids as treatment.
  • Infectious diseases must be thoroughly ruled out.
  • Malignancy must be thoroughly ruled out.
    • Treatment with steroid will partially treat many malignancies and can have a very detrimental impact on likelihood of malignancy to be confirmed or cured; so low threshold for oncologic evaluation where appropriate
    • Many malignancies present with musculoskeletal pain
    • Features of malignancies that help differentiate from rheumatic disease:
      • Night waking with bone pain
      • Relative thrombocytopenia (i.e., low to low normal platelets in setting of elevated ESR)

Pattern of Fever

Persistent/recurrent fever or concern for periodic fever syndrome:

  • Advise family to keep fever diary that includes temperatures (oral or rectal) measured consistently at same time each day, as well as with subjective fevers; also note use of antipyretics and any associated symptoms
  • Daily high spikes (or twice daily) of fever with temperatures below normal between spikes (independent of administration of antipyretics) classic for systemic JIA
  • Persistent high fever without significant lapses concerning for Kawasaki disease, MIS-C, macrophage activation syndrome
  • Fever spikes lasting for 5-7 days at 4 week intervals concerning for PFAPA
  • Autoinflammatory syndromes also have specific patterns; see for specific details

Associated Symptoms

  • Rash
    • SJIA, SLE, vasculitis, sarcoidosis, Kawasaki disease: See specific disease pages for images
  • Arthritis
    • SJIA, SLE, vasculitis, sarcoidosis
  • Renal dysfunction
    • SLE, AAV
  • Upper/lower respiratory tract involvement (including sinuses)
    • AAV, sarcoid
  • Lymphadenopathy
    • SJIA, Kikuchi-Fujimoto
    • Sarcoidosis

Unique Features

  • Association with cold (familial cold associated autoinflammatory syndromes)
  • Family history (genetic autoinflammatory syndromes)
  • Cytopenias
  • Acute phase reactants
    • Elevated ESR + Elevated CRP: ongoing inflammation
    • Normal ESR + elevated CRP
      • If well appearing, may be acute illness
      • If ill appearing, consider MAS and look at additional testing
    • Elevated ferritin: consider SJIA, adult onset Still’s disease, macrophage activation syndrome, MIS-C

Patients with Known Rheumatic Disease

When evaluating patients with known rheumatic disease, providers should first consider the following: