Case Presentation: 41-year-old African American female with a history of Systemic lupus erythematosus (SLE) with antiphospholipid syndrome and cocaine abuse presents with acute onset of atypical chest pain, upper respiratory tract infection symptoms, myalgia, and arthralgia with an unremarkable physical examination. The labs were significant for urine drug screen positive for cocaine, elevated troponin, and D-dimer. Electrocardiogram showed new Q waves and T- wave inversion in lead III. Computerized tomography (CT) angiography was negative for pulmonary embolism. Her chest pain was diagnosed to be secondary to cocaine use which subsided in one day. She had recurrent fever and rash. Initially, it was thought to be secondary to lamotrigine, which was discontinued. The infectious workup was negative. However, she had low C3 and C4, elevated CRP, low fibrinogen of 142, hypertriglyceridemia of 589, pancytopenia (hemoglobin of 8.2 g/dl, leucopenia of 3000 and thrombocytopenia of 130,000), abnormal liver function tests (LFT) with highest values reaching alanine transaminase (ALT) of 425, aspartate transaminase (AST) of 2157, gamma-glutamyl transferase (GGT) of 517, alkaline phosphate (ALP) of 509 and normal total bilirubin. In view of recurrent fever, ferritin was obtained which was significantly elevated at approximately around 7800, which continued to peak around 15000. Macrophage activation syndrome (MAS) was diagnosed given her underlying history of SLE and laboratory abnormalities. She was started on high-dose pulse methylprednisolone followed by prednisone taper. The fever and her aforementioned laboratory findings improved with this treatment.
Discussion: Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory condition with unregulated immune activation. It has 2 forms, primary which is seen more in children is caused by genetic mutations, whereas the secondary form is an acquired condition that can be from infections, malignancies, and autoimmune diseases, which is seen more in adults. Secondary HLH from autoimmune disorders is called Macrophage activation syndrome (MAS). In patients with recurrent fever and hyperferritinemia, one should also, consider HLH especially with a history of rheumatological disorders. Ferritin is an acute-phase reactant that can be seen elevated in infection, inflammation, and malignancy. The presence of hyperferritinemia with levels more than 2000 microgram/liter should raise the suspicion of MAS. Our patient had recurrent fevers and given her underlying history of SLE and HLH probability scores of 98.5%, MAS was diagnosed.
Conclusions: Early recognition and diagnosis of MAS-HLH are essential for efficacious management. Ferritin level should be considered in patients with recurrent fevers without identifiable infections and malignancies.