What are the symptoms of MAS?
Signs and symptoms of MAS
- Continuous fevers (persistent fevers)
- Feeling tired and low energy.
- Headaches, feeling confused or ‘foggy’ (mental status changes)
- Large lymph nodes (lymphadenopathy)
- Large liver and spleen (hepatosplenomegaly)
Is macrophage activation syndrome an autoimmune disease?
MAS is a secondary HLH, which is associated with autoimmune diseases. The most common autoimmune diseases associated with MAS are systemic juvenile idiopathic arthritis (SJIA), followed by systemic lupus erythematosus (SLE), Kawasaki disease (KD), and juvenile dermatomyositis (JDM).
How is ferritin an inflammatory marker?
Serum ferritin is an important inflammatory disease marker, as it is mainly a leakage product from damaged cells. Metallomics.
What causes macrophage activation syndrome?
Dr. Grom says about half of MAS cases are triggered by infections. Others can be caused by a disease flare, when symptoms worsen, or by certain drugs, including those used to treat SJIA such as NSAIDs and biologics. Often, there are no known triggers, although evidence suggests certain gene mutations may play a role.
How common is macrophage activation syndrome?
Although considered a rare complication, macrophage activation syndrome is probably more common than previously thought. In a retrospective study from a tertiary care center, 7 of the 103 children (6.7%) diagnosed with SJIA over a 20-year period developed macrophage activation syndrome.
What happens when a macrophage is activated?
A macrophage that is activated through Toll-like receptors and interferon-γ. These cells exhibit enhanced killing of intracellular microorganisms, increased secretion of cytokines and mediators, and higher expression of co-stimulatory molecules.
Is macrophage activation syndrome fatal?
The term Macrophage Activation Syndrome (MAS) identifies a potentially fatal complication of rheumatic diseases. It occurs usually in the context of systemic Juvenile Idiopathic Arthritis (sJIA), but it may occur also, albeit more rarely, in systemic Lupus Erythematosus and Kawasaki disease.
Why is ferritin high with inflammation?
Ferritin molecules help sequester this free iron, preventing its participation in this reaction and subsequent free radical-mediated cellular damage. Beyond this protective role in redox biology and iron homeostasis, free serum ferritin is increased in the setting of ongoing inflammation.
What diseases cause low ferritin levels?
However, ferritin levels are unreliable in:
- Acute or chronic inflammation.
- CKD.
- Heart failure.
- Liver disease.
- Excessive alcohol intake.
- Malignancy.
- Hyperthyroidism.
Can macrophage activation syndrome be cured?
The treatment of macrophage activation syndrome (MAS) is traditionally based on the parenteral administration of high doses of corticosteroids. However, some fatalities have been reported, even among patients treated with massive doses of corticosteroids.
How is macrophage activation syndrome treated?
What happens inside a macrophage?
Macrophage function in detail Once engulfed, cellular enzymes inside the macrophage destroy the ingested particle. Some macrophages act as scavengers, removing dead or necrotic cells while others provide host immunity by engulfing microbes.
What are the diseases associated with high ferritin levels?
Four uncommon immune mediated conditions may be associated with high ferritin levels: macrophage activation syndrome (MAS), adult onset Still’s disease (AOSD), catastrophic antiphospholipid syndrome (cAPS) and septic shock.
Which is worse, hyperferritinemia or macrophage activation?
Hyperferritinemia is associated with a multitude of clinical conditions and with worse prognosis in critically ill patients.
How is ferritin related to pro-inflammatory cytokines?
As described above, pro-inflammatory cytokines can induce ferritin expression; in turn, ferritin may induce the expression of pro-inflammatory cytokines. Moreover, ferritin induction of anti-inflammatory cytokines (IL-10) is an important mechanism underlying the immunosuppressive effects of ferritin.
How is the expression of ferritin under delicate control?
The expression of ferritin is under delicate control (Figure 2 ). The amount of cytoplasmic ferritin is regulated by the translation of H- and L-ferritin mRNAs in response to an intracellular pool of “chelatable” or “labile” iron.