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What is idiopathic subglottic stenosis?

So, you have just been diagnosed and it’s all very overwhelming. Suddenly doctors are reeling off words you, your friends and family have never heard of, and when you search on the internet there is no or very little insight to be found. Well done for finding this document and hopefully our Facebook support group. We understand where you are coming from and this document will help.

Back to the question in hand. What is idiopathic subglottic stenosis? Getting down to basics;

  • Idiopathic – is a fancy way of saying ‘no known cause’

  • Subglottic – is the part of the larynx below the vocal cords (glottis). It connects to your trachea (‘windpipe’). You may have been told you have tracheal stenosis – this describes a stenosis is located lower down, within the trachea.

  • Stenosis – another fancy word for ‘narrowing’. 

diagram of the trachea

There are four types of stenosis, all with the same symptoms but different causes. It is important that your doctor rules out the other three types of stenosis before you are sure you are idiopathic. The other types of stenosis may result in different treatment paths. This document will help you regardless of your stenosis location, and of course, even if your stenosis is not idiopathic, you will still learn a lot here.

As a patient with this disease, you are going to have to get used to being in control of your treatment – make sure you are aware of the treatment options available, the tests your doctor should be doing and be your own advocate. If you are uncomfortable with your doctor’s level of experience, please request a referral to another doctor. Your airway is not to be experimented with. Unfortunately, not all ENT and Thoracic doctors are experts or fully familiar with the best way to treat subglottic stenosis. 

Question mark

Idiopathic - Doctors suggest your stenosis is idiopathic if you have no history of any of the following:

  • Significant laryngotracheal injury

  • Endotracheal intubation or tracheotomy within 2 years of your first symptoms (see below – iatrogenic stenosis)

  • Thyroid or major anterior neck surgery

  • Exposure to radiation on your neck

  • Caustic (e.g. chemical burn) or thermal (heat) injuries to the airway

  • Vasculitis (a condition that involves inflammation of the blood vessels)

  • Angiotensin converting enzyme (ACE) and antinuclear cytoplasmic antibody (ANCA) 

symbol of autoimmune diseases

Autoimmune - Patients with documented diagnosis of Wegener’s (GPA), Relapsing Polychondritis (RPC), Systemic Lupus Erythematous (SLE), Rheumatoid Arthritis (RA), Epidermolysis Bullosa (EB), Sarcoidosis, Amyloidosis or Mucous Membrane pemphigoid (MMP). Doctors may also treat you as autoimmune if your blood tests positive for ANCAs (Antineutrophil cytoplasmic antibodies) or if your stenosis ‘behaves’ like an autoimmune stenosis (high levels of inflammation and/or returning rapidly post surgery)

leaves in a circle

Polytrauma - Patients with airway stenosis following documented traumatic injuries – particularly involving the trachea – such as breathing in chemicals or hot or burning air.

symbol of suffering

Iatrogen -  Patients that develop subglottic or tracheal stenosis following prolonged orotracheal tube ventilation (intubation in intensive care) or a tracheostomy – either immediately or within 2 years of intubation (although if you have EVER been intubated, it is certainly worth getting hold of your hospital notes to see whether there were any difficulties – there is a chance your stenosis was caused by the intubation).

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