Coughing, shortness of breath and wheezing are all respiratory symptoms that can be a sign of systemic mastocytosis (SM). Although the exact rate of respiratory involvement in SM remains unclear, a sizable proportion of patients with the disease are believed to experience such symptoms. Thankfully, the right care can help ease them.
How mast cells impact the lungs
Lung involvement in SM is related to the biochemical processes that give rise to the disease.
Mast cells are naturally present in the lung tissue in all individuals, playing a role in supporting the immune system by defending against pathogens. In patients with SM, however, abnormal and overactive mast cells accumulate in many different areas of the body, including the lungs.
In their active form, mast cells release various chemicals including histamine and tryptase into the affected tissue, contributing to chronic inflammation and potential organ dysfunction.
Read more about SM signs and symptoms
Patients with SM are also at a significantly higher risk of anaphylaxis than the general population. Anaphylaxis, a severe, life-threatening reaction caused by a massive release of inflammatory chemicals, causes the airways to constrict and swell. This leads to breathing difficulties and chest tightness, requiring immediate treatment with epinephrine.
Symptoms of SM that may exacerbate respiratory involvement
Individuals with SM may also develop symptoms beyond the lungs that can mimic or even exacerbate existing respiratory symptoms. For example, many individuals with SM experience a stuffy nose, which makes breathing difficult. SM can also cause a rapid or irregular heartbeat, which can cause you to feel lightheaded and short of breath.
Furthermore, having trouble breathing can be very frightening and may contribute to heightened anxiety levels. Not only can anxiety on its own cause chest pain and shortness of breath, but emotional stress is also a trigger of SM in some patients.
Treating lung symptoms of SM
In general, treating the respiratory symptoms of SM isn’t much different from treating the disease in general, as the goal is to address the underlying cause of the symptoms. Management options may include:
- Avoiding triggers: Though people with SM may have unknown triggers, avoiding any known triggers will help lower the risk of acute reactions.
- Antihistamines: Because histamines are a major cause of airway tightening in SM, antihistamines can address symptoms. Depending on your condition, you may either take antihistamines on-demand for quick relief or on a more regular basis.
- Mast cell stabilizers: Drugs such as cromolyn sodium or ketotifen may reduce the release of mast cell chemicals in the lungs.
- Glucocorticoids: Glucocorticoids, a class of steroid, help to reduce inflammation and swelling.
- Epinephrine: Treatment with epinephrine is reserved for anaphylaxis, where it is used to quickly reverse airway closure.
By closely following your prescribed treatment regimen and being prepared for emergencies, your respiratory symptoms should become more manageable with time.
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