RESUMO
We report a case of a 16-year-old Hispanic male, without history of systemic illness, who presented with altered mental status and fevers since two weeks prior to evaluation. Further history revealed one-month complaints of headaches, nocturnal fevers, right knee and elbow pain, fatigue, loss of appetite, transient finger discoloration, and a nine-pound weight loss. Physical exam was remarkable for a thin male with pale mucosa, petechia on palate and distal extremities, malar rash that included nasal bridge and cervical and posterior lymphadenopathy. Laboratory work-up showed pancytopenia, with elevated ferritin value of 11,320 ng/mL. The patient was diagnosed with juvenile-onset systemic lupus erythematosus (JSLE) with macrophage activation syndrome (MAS) and suspected antiphospholipid syndrome (APS). Our patient's predominant presentation were neurological symptoms. These can be seen in up to one-third of patients with MAS. They can range from headache, seizures, altered mental status, irritability, and lethargy. Other symptoms are fevers, lymphadenopathy, and hepatosplenomegaly. Ferritin values above 10,000 are highly specific and sensitive for MAS. Albeit a more common presentation in juvenile idiopathic arthritis, MAS can also present across other auto-immune diseases.
