What is idiopathic tracheal stenosis?
Description
Idiopathic progressive subglottic stenosis (IPSS, also known internationally as iSGS) is a slowly progressive narrowing of the trachea with no known cause.
There is a constant accumulation of scar tissue that narrows the trachea directly under the vocal cords. 98% of patients with this disease are female. The disease is very rare, with an estimated incidence of 1:400,000 per year.
It is often misdiagnosed as asthma or psychosomatic.
Idiopathic - ancient Greek for "without known cause"
Progressive or progrendient - means progressive
Subglottic - is the part of the larynx below the vocal cords (glottis). It is connected to the windpipe (trachea).
Stenosis - ancient Greek for “narrowing”.
Caused
There are several possible causes of tracheal stenosis, including damage from previous intubation (e.g., from a previous surgery no more than 2 years ago), damage from inhaling chemicals or heat, an autoimmune disease (e.g., GPA or lupus), or an unknown cause.
Only when all known causes and types of stenosis can be ruled out is it considered idiopathic stenosis. Idiopathic literally means "cause unknown". Your doctor should clarify all possibilities, as treatment varies slightly depending on the level of knowledge.
In idiopathic tracheal stenosis, a connection with the metabolism of female sex hormones is suspected. The studies on this have so far been contradictory, but there have recently been indications that an imbalance between different types of estrogen and progesterone receptors could be causally significant.
Diagnosis
The stenosis is usually clearly visible in a CT or MRI scan, but an experienced ENT doctor or pulmonologist can also detect it with a laryngoscope or bronchoscopy.
After the diagnosis has been made, usually by an ENT doctor or pulmonologist, you should visit a specialized clinic with as much experience as possible.
Treatment
Because it is a rare disease, most patients already have severe breathing difficulties when they receive their diagnosis, so initial treatment often needs to be given very quickly, limiting the ability to explore different options.
Treatment is only carried out in specialised clinics; various options are listed under Treatment. Treatment appointments should always be made in good time. It is often also possible to contact the treating clinic doctors directly.
As a rule, high-pressure balloon dilation, i.e. dilatation of the constriction, is recommended as the initial treatment.
And then?
After dilation, most of our members describe a significant improvement in their shortness of breath, some even think "why not sooner?" One of the main problems with this chronic disease, however, is that the narrowing usually returns. How quickly the shortness of breath returns is very individual.
Even though it is often annoying when the stenosis keeps coming back, we can learn to deal with it and lead a good life. And if one of us is feeling down, our group is there for just that; you can always find someone to talk to and someone who will listen. We also regularly exchange tips and tricks that can make it easier for us to deal with the stenosis and its symptoms in everyday life. And we are in close contact with doctors and clinics, pass on information and promote professional exchange.
We are affected by the very rare idiopathic progressive subglottic tracheal stenosis. We have formed a support group and unfortunately learned from our discussions that most of us had to suffer from increasing shortness of breath and its consequences for months and years before our illness could be diagnosed and treated. That is why we are committed to making this illness and its treatment methods more widely known so that those affected can receive the correct diagnosis and help more quickly in the future.