Clinical features of macrophage activation syndrome in the adult northern Chinese population

Objective: Macrophage activation syndrome (MAS) is a relatively rare but potentially fatal complication of childhood rheumatic illnesses. We sought to provide insight for the timely recognition and diagnosis of MAS and efficacious disease management in adults with rheumatic diseases.

Methods: Clinical files for eight adult MAS patients treated at the Second Affiliated Hospital of Harbin Medical University were analyzed for clinical manifestations, laboratory investigations, therapeutic measurements and clinical outcomes.

Results: The study included male and female patients with ages ranging from 16 to 59 years old. All patients were diagnosed with underlying rheumatic diseases with five patients having adult-onset Still's disease (AOSD), two patients having systemic lupus erythematosus (SLE) and one patient having Sjögren's syndrome (SS). The interval from fever onset to MAS diagnosis varied from seven days to 40 days. The most common clinical presentations were prolonged high fever, respiratory symptoms and jaundice. No patients had symptoms involving the central nervous system (CNS). Laboratory findings showed peripheral cytopenias, elevated liver enzymes, elevated triglycerides, hypofibrinogenemia and bone marrow hemophagocytosis. Potential effective treatments for MAS include glucocorticoid plus immunoglobulin therapy, but delays in diagnosis and treatment may lead to a fatal disease course.

Conclusion: MAS in adults may not be as rare as was once thought, although the clinical features of MAS in adults often differ from those seen in children. The MAS mortality in adults is far higher than that for children. A diagnosis of MAS should be considered when a patient with rheumatic disease presents with prolonged high fever, peripheral cytopenia and liver failure. Collection of bone marrow aspirates is critical for accurate diagnosis and MAS therapy should begin as early as possible.