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- Table 1.
Comparison of typical clinical and laboratory features of Kawasaki disease and macrophage activation syndrome (MAS).
Feature Kawasaki Disease MAS Fever pattern Remittent Continuous Rash Erythematous maculopapular, scarlatiniform or erythema multiforme-like Petechial, purpuric, or erythematous maculopapular Lymphadenopathy Cervical and unilateral Generalized Hepatomegaly ± ++ Splenomegaly – ++ Arthralgias/arthritis ± – Serositis + – Hemorrhages Cracked bleeding lips only + CNS involvement ++ Common: irritability with aseptic meningitis. Rare: transient peripheral facial nerve palsy, profound sensorineural hearing loss + Headache, lethargy, disorientation, hallucinations, seizures, coma White blood cell count ↑↑ ↓ Hemoglobin Normal or ↓ ↓ Platelet count Normal or ↑ ↓ ESR ↑↑ *Normal or ↑ CRP ↑↑ ↑ Transaminases Normal or ↑ ↑↑ Bilirubin Normal or ↑ ↑ Lactate dehydrogenase Normal or ↑ ↑↑ Triglycerides Normal ↑ Albumin Normal or ↓ ↓↓ Fibrinogen ↑ ** ↓ D-dimer ↑ ↑↑ ↵* ESR tends to decrease in MAS.
↵** The absolute value of fibrinogen may be normal or still elevated in MAS; however, its decreasing trend helps with the recognition of MAS versus another inflammatory process. CNS: central nervous system; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein.
