What Is Idiopathic Subglottic Stenosis? The Rare Airway Disease That Changed My Life By Miriam Rees
If you’ve followed my story for any length of time, you’ve probably heard me mention Idiopathic Subglottic Stenosis (iSGS).
It’s a mouthful to say.
It’s difficult to spell.
And unfortunately, it’s a condition that many healthcare providers may only see a handful of times during their entire careers.
Yet for those of us living with it, it can completely change our lives.
One of the reasons I wanted to write this article is because awareness matters.
Too many people spend years searching for answers before finally receiving a diagnosis.
I know because I was one of them.
What Is Idiopathic Subglottic Stenosis?
Idiopathic Subglottic Stenosis (iSGS) is a rare condition that causes narrowing of the airway just below the vocal cords and above the trachea (windpipe). The word “stenosis” simply means narrowing.
Over time, scar tissue forms inside the airway.
As the scar tissue builds up, the airway becomes smaller and smaller, making it increasingly difficult to breathe.
Imagine trying to breathe through a straw.
Now imagine that straw slowly becoming narrower over months or years.
That is what many patients experience.
The challenging part is that the narrowing often develops gradually, so symptoms can be mistaken for other conditions.
What Does “Idiopathic” Mean?
The word that frustrates many patients the most is “idiopathic.”
Idiopathic simply means:
“We don’t know the cause.”
Unlike other forms of airway stenosis that can develop after prolonged intubation, trauma, infection, or autoimmune disease, idiopathic subglottic stenosis develops without a clearly identifiable reason.
Researchers have spent years studying the condition, but there is still no definitive answer as to why it occurs.
Who Gets Idiopathic Subglottic Stenosis?
One of the most fascinating aspects of this disease is how specific the patient population tends to be.
Research has consistently shown that the overwhelming majority of patients diagnosed with iSGS are women.
Some studies report that 95–99% of patients are female, and most are diagnosed between the ages of 30 and 60.
Researchers believe hormones may play a role because of the strong female predominance, but the exact connection remains unclear.
As a 48-year-old woman myself, I fit squarely within the demographic most commonly affected.
Why Is It Often Misdiagnosed?
One reason iSGS can go undiagnosed for years is because the symptoms mimic much more common conditions.
Patients are frequently told they have:
- Asthma
- Allergies
- Anxiety
- Vocal cord dysfunction
- Chronic bronchitis
- Acid reflux-related breathing issues
The most common symptoms include:
- Shortness of breath
- Difficulty exercising
- Wheezing
- Noisy breathing (stridor)
- Chronic cough
- Fatigue
- Difficulty taking a deep breath
Many patients are treated for asthma for months or even years before someone finally looks at the airway itself. Studies have reported delays in diagnosis ranging from nearly two years to four years.
That was one of the reasons I wanted to share my own story.
The earlier someone receives the correct diagnosis, the sooner they can receive appropriate treatment.
How Rare Is It?
Idiopathic subglottic stenosis is considered extremely rare.
Some estimates place the incidence around 1 in 400,000 people. Other population studies have reported annual incidence rates between approximately 0.2 and 0.7 cases per 100,000 people.
Because it is so rare, many people have never heard of it until they are diagnosed.
That lack of awareness can make the journey feel incredibly lonely.
What Causes the Scar Tissue?
The honest answer is:
We don’t know.
Researchers have proposed several theories, including:
- Hormonal influences
- Chronic inflammation
- Autoimmune mechanisms
- Genetic predisposition
- Abnormal wound-healing responses
- Environmental triggers
But no single theory has fully explained the disease.
That uncertainty is one of the hardest parts of living with iSGS.
When you don’t know why something happened, it’s difficult not to wonder if there’s something you could have done differently.
Current Research Gives Hope
The good news is that awareness and research have increased significantly over the last decade.
Organizations such as the North American Airway Collaborative have helped researchers gather information from hundreds of patients around the world. Large multicenter studies are comparing treatment approaches, recurrence rates, quality of life, voice outcomes, and long-term airway health.
Researchers are also studying:
- Tissue samples from patients
- Genetic factors
- Hormonal influences
- Immune system activity
- Inflammatory pathways
- Why the disease recurs after treatment
Mayo Clinic and other leading airway centers continue to conduct studies and maintain patient registries to better understand the disease and improve treatment outcomes.
While we still don’t know the cause, researchers know far more today than they did twenty years ago.
That gives me hope.
Living With the Unknown
One of the hardest realities of iSGS is that treatment often manages the disease rather than cures it.
Many patients require multiple procedures over time because the scar tissue can return. Recurrence remains one of the greatest challenges in managing this condition.
I know this firsthand.
After my airway was repaired, I experienced the joy of breathing normally again.
Then my stenosis returned.
That was difficult.
Not just physically.
Emotionally.
Mentally.
Spiritually.
But it also reminded me how important it is to stay proactive, pay attention to my body, and appreciate every breath I can take.
Why Awareness Matters
If there is one thing I hope people take away from this article, it’s this:
If you are experiencing unexplained breathing problems and asthma treatments aren’t helping, don’t stop searching for answers.
Ask questions.
Advocate for yourself.
Seek specialists when necessary.
And if you know someone living with Idiopathic Subglottic Stenosis, understand that while the disease may be rare, its impact is very real.
Every breath matters.
And for those of us living with iSGS, awareness could help someone else receive answers years sooner than we did.
Sources & Research
- Aravena C, Almeida FA, Mukhopadhyay S, et al. Idiopathic Subglottic Stenosis: A Review. Journal of Thoracic Disease, 2020.
- Chan RK, et al. Prevalence and Incidence of Idiopathic Subglottic Stenosis in Southern and Central Alberta. Canadian Journal of Respiratory Therapy, 2021.
- Gelbard A, et al. Comparative Treatment Outcomes for Patients with Idiopathic Subglottic Stenosis. JAMA Otolaryngology, 2020.
- Mayo Clinic Research – Ongoing Clinical Trials and Registries for Idiopathic Subglottic Stenosis.
- Cleveland Clinic. Idiopathic Subglottic Stenosis: Misdiagnosis with Asthma Is Common.
