Systemic mastocytosis (SM) rarely affects the eyes, but if it does, it can lead to loss of vision.
When it does occur, it is usually following disease progression to a more serious form of SM, notably aggressive systemic mastocytosisMastocytosis Rare disease caused by the buildup of mast cells. Cutaneous mastocytosis primarily affects the skin and is more common in children, while systemic mastocytosis affects internal organs and is more common in adults. (ASM). Often referred to as ocular mastocytosis, symptoms can include red, itchy, watery eyes and blurry vision.
What is SM?
Systemic mastocytosis (SM) is a rare hematological disease characterized by mast cells that are overactive and accumulate in different parts of the body such as the bone marrow, liver, spleen, gastrointestinal tract and lymph nodes.
Different subtypes of SM
When we speak about SM, we usually refer to the most common subtype of SM, indolent systemic mastocytosis (ISM). This is the mildest version of SM, with a manageable symptom burden and less effect on life expectancy. But there are several other subtypes possible as the disease progresses.
Read more about SM signs and symptoms
Smoldering systemic mastocytosis (SSM) has a greater effect on internal organs and less positive outlook than ISM. Aggressive systemic mastocytosis (ASM) is a more severe version of SM, with greater complications and lower survival rates, as well as possible ocular symptoms.
If the disease continues to progress to the most serious subtypes — systemic mastocytosis with an associated hematological neoplasm and mast cell leukemiaMast cell leukemia The most severe subtype of SM, caused by the rapid buildup of immature mast cells in the bone marrow and blood. Mast cell leukemia accounts for less than 5% of SM cases and has a poor prognosis. — the disease burden increases; symptoms become harder to manage, and life expectancy is dramatically reduced.
Eye-related SM symptoms
HistamineHistamine A chemical released by mast cells when they are activated. Histamine causes many of the allergy-type symptoms in mastocytosis, including flushing, itching and hives. release as a result of mast cellMast cell A type of white blood cell produced in the bone marrow. They help defend against infections and play a key role in allergic reactions. In SM, mast cells become overactive and build up throughout the body. activity can cause symptoms in the eyes, such as blurred vision or red, itchy, watery eyes. Eye-related symptoms are not common in SM, but the effect on your vision can be progressively debilitating if the eyes are involved.
In the eye, mast cells accumulate in the conjunctiva, the mucus membrane that forms a protective film over the eye.
Managing SM symptom triggers
Triggers for the onset of SM symptoms are varied and may include alcohol, certain foods and medications, emotional stress, physical exertion, sudden changes in temperature, pollen, strong perfumes, skin friction and insect stings.
Triggers that activate mast cells in the eyes are usually pollen, animal dander and mold spores. Avoiding such triggers will avoid ocular symptoms and the eventual deterioration or loss of vision.
Treating eye SM symptoms
Your doctor will advise you on the appropriate medication for SM-related eye symptoms.
The most common treatment is a mast cell stabilizerMast cell stabilizer Medications such as cromolyn sodium that prevent mast cells from releasing histamine and other chemicals that cause symptoms. known as cromolyn eye drops, which prevent mast cells from releasing histamine and other chemicals that cause the allergic reaction.
Antihistamine eye drops are also effective in managing ocular symptoms. Topical or oral steroids may also be prescribed.
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