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 https://www.nomidalliance.org/compchart.php 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
  • Upper/lower respiratory tract involvement (including sinuses)
  • 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:

If yes, consider the following:

  • Increased risk of viral/bacterial/fungal infection
    • If no obvious source, consider blood/urine cultures in addition to standard workup (guided by clinical presentation)
    • Class of medication may impact infection risk
      • Anti-CD20 medication: B cells – think bacteria
      • T-cell inhibitors: Think viruses and fungi
      • Steroids: At risk for all types of infections, especially at higher doses
  • Medications may alter classic response
    • IL-6 inhibitors may falsely lower CRP
    • Glucocorticoids may limit inflammatory response causing attenuation of exam findings
  • Medication may need to be held
    • For moderate to severe infection, if rheumatic disease well controlled, strongly consider holding medication until patient is fever-free with improving symptoms x24 hours (discuss with treating rheumatologist)
  • Glucocorticoids may require stress dosing for patients at risk of adrenal suppression with moderate to severe illness (reference: https://www.chop.edu/clinical-pathway/steroid-stress-dosing-and-weaning-clinical-pathway provides guidance)
  • IL-1 inhibitor usually not held during infections.
  • SLE: Higher risk for infection with encapsulated organisms (e.g. streptococcus pneumoniae, salmonella)
  • SLE, Vasculitis: Renal disease may result in loss of immunoglobulins, complement, and other proteins in the urine
  • SLE, SJIA/MAS: Cytopenias, particularly neutropenia, can occur and result in serious infections

See linked disease pages for full description of testing to determine if fever is likely to be part of disease flare.

Fevers are a common symptom in all of the following:

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