Abstract
Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases. This case demonstrates the significant challenges and therapeutic considerations in adult-onset Still’s disease (AOSD) complicated with MAS at initial presentation, which will be discussed. MAS in our patient was refractory to the first-line therapy with high-dose corticosteroids, early administration of anakinra at a standard dosage and subsequent add-on treatments with cyclosporine A, IVIG, etoposides and tocilizumab. At 2 months after presentation, the patient was still critically ill with clinical, laboratory and histological signs of an active uncontrolled MAS. Notably, adoption of anakinra at a high dosage finally induced remission. This case confirms that adjusted dosage of anakinra is an effective therapeutic strategy in a severe AOSD-related MAS. It is tempting to speculate that anakinra at a high dosage, if used earlier, would have significantly changed the course of the disease in our patient and could have led to earlier remission.
Highlights
Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases
MAS or ‘rheumatic haemophagocytic lymphohistiocytosis (HLH)’ is classified among the secondary forms of HLH occurring in the context of a rheumatic disease.[5]
Both the primary and secondary forms of HLH are characterized by an uncontrolled activation and proliferation of macrophages and T lymphocytes with hypersecretion of pro-inflammatory cytokines, tissue infiltration, haemophagocytosis and tissue damage
Summary
Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases.
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