In systemic mastocytosis (SM), mast cells develop abnormally and infiltrate the connective tissue and internal organs, including the skin, liver, spleen, brain, gastrointestinal tract and, in rare cases, the lungs.
The mast cells accumulate, and when an allergic reaction is triggered, they release histamines and other chemicals into the bloodstream, causing a range of debilitating symptoms.
While the lungs are not commonly affected, if this excessive mast cell activity occurs in the lungs, it can damage the lung tissue and cause long-term respiratory complications.
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.
How SM affects the lungs
When mast cells accumulate in the lungs, they cause inflammation and damage to the lung tissue. As the lung tissue becomes scarred and less elastic, lung function and capacity is affected. Respiratory issues can resemble symptoms of asthma and include tightness in the chest, coughing, wheezing and breathlessness.
Read more about SM signs and symptoms
The effect on quality of life can be severe, with physical activity limited because of reduced lung capacity and breathing difficulties. You may also find that your daily life can be affected by the need to avoid common triggers of lung-related symptom episodes, including smoke, pollens and strong perfumes.
SM symptom management
The best approach to SM symptoms is to recognize your triggers and make permanent changes to your lifestyle and diet to avoid activating them. It may take some time to identify them, but tracking symptoms and how and when they occur is key to taking more control of your symptom burden.
The severity, duration and triggers of symptoms vary from person to person, as do the organs affected. As SM can affect different parts of the body, it can mean having several different symptoms occurring simultaneously.
You may experience some or all of the following symptoms.
- Fatigue.
- Itchy skin, flushing, hives.
- Joint and bone pain.
- Brain fog, low mood, poor concentration.
- Digestive complaints, abdominal pain, nausea, diarrhea, vomiting.
- Anaphylaxis.
The effect and level of discomfort may be mild, moderate or severe.
Lifestyle changes will improve your quality of life by avoiding the following triggers: certain high-histamine foods; some medicines; sudden changes from hot to cold, or cold to hot; emotional or physical stress; pollens, strong perfumes or allergens; smoke; skin friction; insect stings; and alcohol.
How to support your lung health in SM
Medication to alleviate SM symptoms include antihistamines, mast cell stabilizers and leukotriene receptor antagonists. You can also take the pressure off your lungs by using a bronchodilator to make breathing easier.
Regular chest X-rays and CT scans will monitor the damage to the lung tissue, and lung function tests will assess changes in lung capacity.
