Macrophage activation syndrome
Macrophage Activation Syndrome (MAS) is a severe, potentially life-threatening complication of systemic juvenile idiopathic arthritis (sJIA) and other autoimmune diseases. It is characterized by excessive activation and proliferation of macrophages and T lymphocytes, leading to overwhelming inflammation and tissue damage.
Pathophysiology[edit | edit source]
MAS is a form of secondary hemophagocytic lymphohistiocytosis (HLH), a syndrome of excessive immune activation. In MAS, the dysregulated immune response leads to the overproduction of cytokines, such as interleukin-1, interleukin-6, and tumor necrosis factor-alpha. This cytokine storm results in the activation of macrophages and T cells, which then attack the body's own tissues.
Clinical Features[edit | edit source]
The clinical presentation of MAS includes:
- Persistent high fever
- Hepatosplenomegaly (enlarged liver and spleen)
- Cytopenias (reduction in the number of blood cells)
- Elevated levels of ferritin
- Hypertriglyceridemia (high levels of triglycerides in the blood)
- Coagulopathy (impaired blood clotting)
Diagnosis[edit | edit source]
The diagnosis of MAS is challenging due to its overlap with other inflammatory conditions. It is often based on clinical criteria and laboratory findings, including:
- Elevated serum ferritin levels
- Decreased platelet count
- Elevated liver enzymes
- Hypertriglyceridemia
- Hypofibrinogenemia (low levels of fibrinogen)
Treatment[edit | edit source]
Early recognition and treatment of MAS are crucial. Treatment typically involves:
- High-dose corticosteroids
- Cyclosporine
- Intravenous immunoglobulin (IVIG)
- Biologic agents targeting specific cytokines, such as anakinra (an interleukin-1 receptor antagonist)
Prognosis[edit | edit source]
The prognosis of MAS depends on the promptness of diagnosis and initiation of treatment. With timely and appropriate therapy, the outcome can be favorable, but delayed treatment can lead to significant morbidity and mortality.
Related Pages[edit | edit source]
- Systemic juvenile idiopathic arthritis
- Hemophagocytic lymphohistiocytosis
- Cytokine storm
- Autoimmune diseases
- Macrophage
- T lymphocytes
Search WikiMD
Ad.Tired of being Overweight? Try W8MD's physician weight loss program.
Semaglutide (Ozempic / Wegovy and Tirzepatide (Mounjaro / Zepbound) available.
Advertise on WikiMD
|
WikiMD's Wellness Encyclopedia |
| Let Food Be Thy Medicine Medicine Thy Food - Hippocrates |
Translate this page: - East Asian
中文,
日本,
한국어,
South Asian
हिन्दी,
தமிழ்,
తెలుగు,
Urdu,
ಕನ್ನಡ,
Southeast Asian
Indonesian,
Vietnamese,
Thai,
မြန်မာဘာသာ,
বাংলা
European
español,
Deutsch,
français,
Greek,
português do Brasil,
polski,
română,
русский,
Nederlands,
norsk,
svenska,
suomi,
Italian
Middle Eastern & African
عربى,
Turkish,
Persian,
Hebrew,
Afrikaans,
isiZulu,
Kiswahili,
Other
Bulgarian,
Hungarian,
Czech,
Swedish,
മലയാളം,
मराठी,
ਪੰਜਾਬੀ,
ગુજરાતી,
Portuguese,
Ukrainian
Medical Disclaimer: WikiMD is not a substitute for professional medical advice. The information on WikiMD is provided as an information resource only, may be incorrect, outdated or misleading, and is not to be used or relied on for any diagnostic or treatment purposes. Please consult your health care provider before making any healthcare decisions or for guidance about a specific medical condition. WikiMD expressly disclaims responsibility, and shall have no liability, for any damages, loss, injury, or liability whatsoever suffered as a result of your reliance on the information contained in this site. By visiting this site you agree to the foregoing terms and conditions, which may from time to time be changed or supplemented by WikiMD. If you do not agree to the foregoing terms and conditions, you should not enter or use this site. See full disclaimer.
Credits:Most images are courtesy of Wikimedia commons, and templates Wikipedia, licensed under CC BY SA or similar.
Contributors: Prab R. Tumpati, MD
