Macrophage activation syndrome in a patient with axial spondyloarthritis on adalimumab

Abstract

Macrophage activation syndrome (MAS) is a rare and potentially fatal condition characterized by excessive activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to overwhelming systemic inflammation and cytokine release. MAS has been reported with viral infections, autoimmune disorders, malignancies, and medications. We describe a case of a patient with axial spondyloarthritis (axSpA) treated with adalimumab, who presented with MAS.

Macrophage Activation Syndrome Upon Initiation of Adalimumab in a Patient With Longstanding Rheumatoid Arthritis

“…While a definitive link between biologics and MAS has not been formally established, several cases in the literature report macrophage activation syndrome after initiation of etanercept, canakinumab, infliximab [ 8 ] and adalimumab. The development of MAS in a similar temporal relationship to adalimumab and without a detectable infectious trigger was also described by Baker et al in an adult patient with axial spondyloarthritis [ 9 ]. The pathophysiologic mechanism behind this syndrome is not well understood, but it appears to involve modulation of toll-like receptors (TLRs) by biologic agents.…”

Immunopathology of and potential therapeutics for secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a translational perspective

“… Target Drugs Combined drugs Condition Trigger Clinical and laboratory criteria Results Ref. IL-1 Anakinra Methylprednisolone, Ciclosporin, Prednisolone sJIA Methylprednisolone ↑Fever, ↑Splenomegaly, ↑Ferritin, ↓PLT, ↑AST, ↑Fibrinogen, ↑sCD25 Ferritin/PLT/AST/Fibrinogen normal range 95 IVIG, Aspirin, Infliximab, Methylprednisolone, Prednisolone KD, HLH Methylprednisolone, Prednisolone ↑Ferritin, ↑WBC, ↑PLT, ↑AST, ↑ALT, ↑TG Inflammatory marker recovery 96 Methylprednisolone, Ciclosporin, Prednisolone, Dexamethasone AOSD, HLH Methylprednisolone ↑Fever, ↑Hepatomegaly, Cervical lymphadenopathy, ↑Neutrophilic leukocytosis, ↑Ferritin, ↑AST, ↑TG, ↑Hematopoietic cells Ferritin/Inflammatory marker improvement 97 Methylprednisolone, Corticosteroids, Tocilizumab AOSD, HLH Anakinra (100 mg/day), Methylprednisolone, Tocilizumab ↑Fever, ↑Hepatomegaly, ↑Splenomegaly, ↑Ferritin, ↓PLT, ↑AST, ↑ALT, ↓Leucocyte count, lymphopenia, ↑Hematopoietic cells Ferritin/systemic inflammation and cytolysis improvement 98 Methylprednisolone, Infliximab, Prednisolone, MTX, Dexamethasone sJIA Infliximab, Prednisolone, MTX ↑Fever, ↑Splenomegaly, ↑Ferritin, ↓PLT Ferritin/WBC/PLT normal range 99 Prednisone, Adalimumab axSpA Adalimumab ↑Fever, ↑Splenomegaly, Lymphopenia, ↓PLT, ↑AST, ↑ALT, ↑Ferritin ESR/CRP/ferritin/fibrinogen/inflammatory marker improvement 100 Canakinumab Prednisone, Methylprednisolone, Anakinra, Cyclosporine A sJIA Prednisone, Methylprednisolone, Anakinra ↑Fever, ↑Ferritin, ↑PLT, ↑AST, ↑TG, ↑Fibrinogen Ferritin decrease, AST normal range 101 ...…”