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Macrophage Activation Syndrome (MAS) in Adults

A life-threatening hyperinflammatory complication of rheumatic disease characterized by uncontrolled macrophage and T-cell activation.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Macrophage Activation Syndrome (MAS) in Adults?

Macrophage activation syndrome is a form of secondary hemophagocytic lymphohistiocytosis (HLH) seen in adult rheumatologic disorders, most commonly adult-onset Still's disease, systemic lupus erythematosus and adult systemic vasculitides. It results from uncontrolled activation and proliferation of T lymphocytes and macrophages with massive cytokine release.

Clinical hallmarks include unremitting fever, hepatosplenomegaly, lymphadenopathy, cytopenias, marked hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia and elevated transaminases. Bone marrow examination may show hemophagocytosis but is not always required for diagnosis.

Without prompt recognition and treatment, MAS can progress rapidly to multi-organ failure and disseminated intravascular coagulation. Therapy combines high-dose corticosteroids, calcineurin inhibitors, anakinra and, in refractory cases, etoposide-based protocols.

Symptoms

High persistent fever
Hepatosplenomegaly
Pancytopenia
Mucosal bleeding
Hepatic dysfunction
Encephalopathy and seizures
Multi-organ dysfunction

Risk Factors

Active AOSD or systemic JIA in adulthood
Lupus flare
Concurrent infections (EBV, CMV)
Drug triggers including biologics
Underlying malignancy
Genetic HLH gene variants
Severe hyperferritinemia

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent unexplained fever in known rheumatic disease
  • Rapidly falling cell counts
  • Marked rise in ferritin and triglycerides
  • Coagulopathy or hypofibrinogenemia
  • Mental status change
  • Worsening organ function

Treatment Methods

01
High-dose intravenous methylprednisolone
02
Cyclosporine A
03
Anakinra (IL-1 blockade)
04
Etoposide for refractory disease
05
Plasmapheresis or intravenous immunoglobulin
06
Treatment of underlying triggers and infection
07
Multidisciplinary intensive care

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.