Autoimmune and Airway Disorders That Cause Tracheal and Subglottic Stenosis

Tracheal and subglottic stenosis occur with a narrowing of your windpipe, which is the most critical conduit of air travel from your mouth to your lungs. The subglottic space above the trachea is where 100 percent of your ventilation takes place, yet it is quite narrow, so it is important to keep this area as open as possible. Airflow is what causes your respiratory currency; the more active you are, the more energy you are burning and the more support you need for this ventilation. If your airflow tube is narrowed, you need to increase the force to push the air through. However, this requires more effort, as you are expending more metabolic energy. The problem that transpires is that eventually many individuals with difficulties breathing will cut down on their metabolic expenditures, and ultimately, their fitness (i.e. instead of running, they opt for walking).

Dr. Robert Lebovics, Site Chair of Otolaryngology and Co-Director of the Airway Center at Mount Sinai West, as well Medical Advisory Board member of the Vasculitis Foundation and Sjogren’s Syndrome Foundation, specializes in helping people breathe. Typically patients are first diagnosed with an autoimmune or airway disorder by a rheumatologist or immunologist. They are then referred to an otolaryngologist with expertise in these conditions. Their symptoms can include: coughing, difficulty breathing, peculiar blood test results or trouble hearing. The diagnostic test is typically a tissue or blood test. In many cases the cause is unknown, which is referred to as ‘idiopathic subglottic stenosis.’

“On a simplistic level, you need to breathe, and your physicians need to help you breathe,” Dr. Lebovics notes. “These conditions can be multisystem disorders; it is critical for physicians to work together and create long-term monitoring relationships with their patients.” Hence, Dr. Lebovics employs a multidisciplinary approach when treating each patient. This means he collaborates with other experts, including rheumatologists, pulmonologists, immunologists, allergists, oncologists, radiation oncologists, thoracic surgeons, laryngologists and speech language pathologists. Together, they bridge their expertise to ensure the best monitoring and treatment path for each patient.

Autoimmune and Inflammatory Diseases Causing Tracheal and Subglottic Stenosis

Autoimmune and inflammatory diseases are indicators that the immune system is overactive (hyper-immunity). They can target certain immune cells called ‘T-cells’ that can cause a cascade of responses. This includes a misfiring of the immune system, which can cause dysregulated T-lymphocytes to transform other immune cells (B cells) into scavengers that attack healthy tissue. The following are examples of these disorders:

  • Granulomatosis with polyangitis (GPA)/Wegener’s granulomatosis: this uncommon disorder is a form of vasculitis that causes inflammation of the blood vessels; this typically affects the sinuses, lungs and kidneys, and can impact other tissues and organs. The majority of patients with GPA will develop a manifestation in the ear, nose, trachea or bronchi.
  • Eosinophilic granulomatosis with polyangitis/Churg-Strauss syndrome: also characterized by inflammation of the blood vessels, this rare disease most often affects the sinuses, heart, lungs, intestines and nerves. It could also affect the kidneys, muscles or joints. It occurs in patients with a history of allergies or asthma.
  • Sarcoidosis: this inflammatory disease can affect several organs in the body, but primarily the lungs and lymph glands. More women and men are diagnosed with it, and it is 10-17 times more common among African-Americans than Caucasians.
  • Sjogren’s syndrome: characterized by dryness of the mouth and eyes, this autoimmune disease affects the salivary and tear glands, which produce saliva and tears. Inflammation of the glands can cause decreased saliva production and dry mouth, eyes and lips.

Infectious Diseases and Cancer Treatment

Sometimes tracheal stenosis can transpire as a result of a viral infection or treatment for cancer, such as radiation or extended use of a breathing tube. In these instances, this effect is hypo-immunity, meaning the immune system is underactive. Cancer treatment and diseases such as tuberculosis or glanders can cause throat swelling and block the space in your trachea. Similar to how a drain in a bathtub can become backed up, these are factors contributing to the prevention of air moving through your windpipe.

In most cases, patients should consult an otolaryngologist (ear, nose and throat doctor) to establish a baseline. The majority of patients need to be monitored by a physician who specializes in these disorders to detect activity - often before it appears in bloodwork. These disorders can cause problems with breathing and hearing or may even be life-threatening. The optimal ways to address these disorders are to surveille and treat them.

Treatments for Tracheal and Subglottic Stenosis

It can be a difficult problem to treat that may require multiple interventions and different treatments.

  • Endoscopic dilation: widening the narrowed area of the trachea through the mouth; this can be achieved with a scope, laser, balloon or stent.
  • Tracheal resection and reconstruction: removing a portion of the trachea and reconnecting the upper and lower sections of it.
  • Airway stent: inserting a hollow, metal or silicone tube into the airway to maintain an open trachea.
  • Laryngeal/tracheal/bronchial cryotherapy: sculpting and remodeling the trachea via spray cryotherapy.
  • Laser bronchoscopy: utilizing a laser to destroy tissue causing the airway stenosis.
  • Steroid injections: stabilizing or prolonging the effects of dilation with injections.
  • Combination therapy: employing multiple treatment modalities to maximize the airway.
  • Tracheotomy: creating an opening through the neck via an incision into the trachea and placing a tube through the opening to provide an airway to assist with breathing.

Autoimmune and Airway Disorder Follow-up Care

Patients are rarely cured of autoimmune or airway diseases, which makes monitoring and follow-up care critical to their overall wellbeing. For example, those suffering from Wegener’s granulomatosis have a high incidence of disease relapse. If untreated, it can be life-threatening. Patients are recommended to follow-up every 3-6 months while in remission with their rheumatologist or immunologist. Together, Dr. Lebovics and his team at Mount Sinai West collaborate with all physicians involved in each patient’s plan to provide the highest quality medical care. The goal is to help patients breathe as best as possible and ultimately prevent future complications.