"It's hard to imagine a disease more complex than mast cell activation disease..." - Dr. Afrin
Mast cells, a type of blood cell, plays a critical role in the body’s immune system. Mast cells reside in all body tissues and form part of the body’s defense system by being responsible for immediate allergic reactions. Mast cells create and store mediators and releases them when a foreign substance is present inside of the body, an allergen, or when there is an injury. For a healthy person, these chemical mediators act beneficially to heal and protect the body. However, for a person who has MCAS, these chemicals are inappropriately triggered and released, resulting in a negative effect on the body. When someone has MCAS, their body interprets different triggers (foods, exercise, chemicals, fragrances, stress, etc.) as a foreign substance/allergen and then the body releases mediators to initiate an allergic reaction. This essentially means that people with MCAS have sensitive mast cells and can have an allergic reaction to any and many things. MCAS causes a wide range of unpleasant, sometimes debilitating, symptoms that often affect several different systems of the body at the same time. It is important for patients to identify and avoid their triggers, but this is can be extremely difficult and often times the process of discovering new triggers never really ends.
Quick facts
Prevalence: Rare disorder, but the specific number is unknown. MCAS affects both children and adults.
Often Associated With:
Ehlers-Danlos Syndrome
Postural Orthostatic Tachycardia Syndrome (POTS)
Autism
Chronic Fatigue Syndrome
Lupus
Fibromyalgia
Lyme Disease
Multiple Sclerosis
Symptoms: (wide ranging and depends on the individual)
Itching, rashes, hives, skin turning red, swelling
Inflammation
Digestive discomfort, abdominal pain, nausea, vomiting, diarrhea
Chronic pain
Tachycardia (rapid pulse)
Hypotension (low blood pressure)
Dizziness/fainting
Wheezing, shortness of breath, cough, chest pain
Anxiety
Difficulty concentrating
Headaches
Brain fog
Fatigue
Insomnia
Itching, tightness/closure, and hoarseness of the throat
Triggers: (depends on the individual and can change over time)
Chemicals
Food/drinks
Heat/cold
Exercise
Medications
Stress (emotional and physical)
Fatigue
Infections
Venoms
Sun
Fragrances
Areas Affected: Affects all systems, organs, and tissue of the body! Specifically affecting…
Skin
Liver
Spleen
Lymph nodes
Bones/bone marrow
Gastrointestinal system
Lungs
Eyes
Blood
Heart
Severity: Unpleasant, to completely debilitating, to anaphylaxis (life-threatening)
Diagnosis: It is often difficult for patients to receive a diagnosis and to find a doctor who has the time and experience with MCAS. The diagnosis criteria relies on symptoms, evidence of mediator release and response to medication, as well as ruling out any other potential diseases. There is no definitive test for MCAS, but blood work (looking at tryptase, histamine, and prostaglandin) can support a diagnosis.
Treatment: There is no cure for MCAS, but avoiding triggers can help improve quality of life. There are also medications that can help limit aspects of reactions from occurring.
Epinephrine (EpiPen): In the case of anaphylaxis, should be used in addition to seeking medical attention
Antihistamines: Can be effective for itching, abdominal pain, and flushing (second generation antihistamines are preferable due to fewer side effects)
Histamine type 2 receptor blockers: Can be helpful for abdominal pain and nausea
Aspirin: Can reduce flushing
Montelukast and Zafirlukast: Can help reduce wheezing and abdominal cramping
Corticosteroids: Helpful for edema, hives, and wheezing (but should only be used as a last resort)
Omalizumab: Helps reduce mast cell reactivity and sensitivity to activation, which can reduce anaphylactic episodes
Timeline: The onset of MCAS is often sudden and patients commonly notice a dramatic change in symptoms after years of mild symptoms. Symptoms are often episodic and vary in intensity. For some patients, symptoms worsen over time.
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