Mast Cell Disorder Specialist in New Orleans

Mast Cell Activation Syndrome (MCAS)

Mast cell activation syndrome describes the inappropriate activation of mast cells with little or no observed increase in the number of mast cells throughout the body. Inappropriate triggers can include different foods, chemicals, fragrances, exercise, or stress. MCAS is not caused by an allergy nor is it secondary to other conditions that are known to activate normal mast cells.

Mast Cell Disorders, Hypermobile Ehlers-Danlos Syndrome, and Postural Orthostatic Tachycardia Syndrome

Hypermobile Ehlers-Danlos Syndrome (hEDS) is a rare inherited condition that affects connective tissue, resulting in very flexible joints and stretchy skin that breaks easily. This connective tissue disease can also affect other systems in the body including the digestive tract (heartburn, constipation), the heart (mitral valve prolapse), the bladder (stress incontinence), and other organs (organ prolapse).

Postural Orthostatic Tachycardia Syndrome (POTS) is a condition that causes an abnormal increase in heart rate after sitting up or standing. Patients with POTS often tend to describe discomfort when their heart rate increases, and can also experience lightheadedness and fainting. Symptoms are usually alleviated by sitting or lying back down.

In a study published in 2015, patients having a diagnosis of POTS and EDS were given a screening questionnaire to look for symptoms consistent with MCAS, and 66% of the respondents reported such symptoms. While much about these conditions and the nature of the relationships between them remain unknown, many researchers and medical professionals are now looking at these conditions together as a unique syndrome that’s relatively common in individuals with mast cell disorders.

Mastocytosis Symptoms

Mastocytosis can appear as distinct “spots” that look like freckles. These spots are called urticaria pigmentosa, and they can transform into hives and itch if stroked or irritated, or if the skin is exposed to sudden changes in temperature such as a hot shower.

Other symptoms of mastocytosis include:

• Anaphylaxis (severe allergic reaction)
• Shortness of breath
• Low blood pressure
• Hives and swelling
• Itching
• Nausea and vomiting
• Diarrhea
• Fainting
• Headache
• Uterine cramps / bleeding
• Flushing
• Musculoskeletal pain

MCAS Symptoms

An individual with MCAS often experiences repeated episodes of anaphylaxis that can affect different organ systems in the body. These include:

• Heart related symptoms: rapid pulse, low blood pressure, passing out
• Skin related symptoms: itching, hives, swelling, red skin
• Lung related symptoms: throat swelling, wheezing, shortness of breath, harsh noise when breathing
• Gastrointestinal tract symptoms: diarrhea, nausea with vomiting, crampy abdominal pain

Mastocytosis Diagnosis

If you suspect that you have a mast cell disorder, it’s important to seek care from an allergist / immunologist that is a mast cell disorder specialist. Diagnosis of mastocytosis can include:

Mastocytosis Management

• Antihistamines
• Cromolyn sodium
• Ketotifen
• Leukotriene-modifying agents

MCAS Management

• Certain antihistamine medications (type 1 receptor blockers) can be effective for itching, abdominal discomfort, and flushing, while others (type 2 receptor blockers) can help with abdominal pain and nausea
• Aspirin can reduce flushing
• Montelukast and zafirlukast can help reduce wheezing and abdominal cramping
• Corticosteroids can be helpful for edema, hives, and wheezing
• Omalizumab may help reduce anaphylactic episodes

If these treatments are ineffective, it’s unlikely that you have MCAS.

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