Subglottic Stenosis: positive P ANCA screen, Wegeners / GPA Diagnotic indicators, crusty nose, cyclophosphamide.

[Chappell8]

I've had 4 surgeries in 18 months for subglottic stenosis, a narrowing of the airway caused by chronic inflammation, though no definite cause has been identified. Lots of tests are negative for Vasculitis, apart from the P ANCA screen which has been positive several times. The more specific test for vasculitis is normal and negative for PR3/MPO. Doctors opinions differ, 3 previously said the positive P ANCA screen is atypical and not a definite diagnostic for Vasculitis. However, the Vasculitis Consultant and now my ENT say the subglottic stenosis has responded well to high dose IV steroids given during dilation surgery, and they believe it should be treated as Wegeners GPA., as I've also had nasal symptoms of blocked/crusty nose, mucous, chronic cough etc, and the stenosis has recurred aggressively within 3 to 4 months of dilation. I have no systemic symptoms, bronchoscopy, gastroscopy, biopsy, kidneys, CT scan and all other bloods are negative for Vasculitis or infection, and I generally feel well and healthy. Results show the stenosis site is " a short, isolated area of mildly fibrous tissue showing chronic reactive inflammation, no granuloma's." I am concerned about starting aggressive treatment with steroids and immunosuppresents as I don't have any definite positive positive diagnostics for Vasculitis, more that it's prresumed as my symptoms are 'n keeping with Wegeners / GPA' and there is no other identified cause for the chronic inflammation causing the stenosis.

I'd be glad of any feedback to help me understand the diagnostic decision. What relevance is an atypical positive P ANCA screen, if the more specific MPO/ PR3 vasculitis test is negative? Does a good response to IV steroids strongly indicate an autoimmune cause, or could inflammation of idiopathic or unknown cause also respond well to IV steroids? This sounds gross but what exactly do doctors mean by a custy nose', how bad does it have to be to be considered a medical problem? I work in a dry environment with central heating and lots of healthy colleagues say they have a 'blocked/dry crusty nose, and also have to clear their nose when they wake in the morning, and mine is no worse than anyone else - I'd hate to be diagnosed with Vasculitis because of that! Finally, if I have to consider treatment with steroids and cyclophosphamide, I'd be glad of any feedback if you can share your experience of living with the side effects and reduced immunity

I've seen 2 ENT's, a Respiratory and a Vasculitis Consultant, so lots of experts but they differ n their opinions and there is a lack of definite diagnostics, so real life experience from this group would be a lifeline for me. I'm aware I may be in denial hoping it won't be Vasculitis, but I also feel cautious about accepting this diagnosis and aggressive treatment in the absence of much positive, objective evidence. Differential diagnosis could be Idiopathic, ie unknown cause. Thanks, Linda.

[SusanCh]

Hi Linda you seem to be seeing a Vasculitis Expert and ENT and they would point you in the right direction re treatment for GPA but the situation is not clear cut. I am not an expert but can only tell you about my experience of WG(GPA) and sub glottis stenosis.

I was diagnosed with WG 10 years ago via a biopsy of a polyp in my nose and CANCA positive( not everyone is positive). My WG is aggressive and fast acting and within a short space of time, I lost my hearing, my voice, terrible pains in my sinuses, lung lesions and saddle nose. After cyclophosphamide and steroids, things eased but I was rushed in due to sub glottis stenosis and had to have a tracheostomy. I then had dilations every 6 months for 2 years. The effects of the dilations didn't last long and I flared again and the stenosis was unstable. I don't know where you live. I live in North west of UK. I found out about Mr Guri Sandhu ENT at Charing Cross Hospital in London and managed to get referred to him. He is brilliant. I had 4 dilations, granulomas tissue was found. He deals specifically with patients with trachea problems through whatever reason. It could be trauma, idiopathic or auto immune. Eventually I had reconstruction surgery and trachey removed. Keeping my illness under control is a priority.

Contact me if you want to discuss further, I will help if I can.

Best wishes Sue

[Chappell8 in reply to SusanCh]

Thank you Sue, that's so helpful. I joined the facebook group Living with Ideopathic Subglottic Stenosis and have learnt such a lot from their discussions. Several people mentioned the pioneering work done by Guri Sandhu so I asked to be referred, and have my first appointment in May. (I'm from Liverpool). I had hoped that I may be a candidate for reconstruction and get a longterm fix, rather than repeated dilation, but my current ENT said that resection or major surgery was not recommended if the cause is vasculitis, as aggressive tracheal surgery can result in more aggressive return of symptoms. I presumed that a diagnosis of WG/GPA would mean I'm not a candidate for reconstruction. That's why it feels so important to me to get an accurate diagnosis as it will determine the pathway for treatment of the stenosis. I would fully accept its WG/GPA if my clinical symptoms, biopsy, CT scan, bloods etc confirmed something definite and positively diagnositic, but most of my results are negative for vasculitis, and the subglottic stenosis could be idiopathic, so I still have some doubts as to whether or not it is vasculitis. I've will type a history of my symptoms, and a summary of all test results, and trust that Mr Sandhu's team will repeat their own tests and make their own diagnosis of the underlying cause. I would like to email you Sue, to ask a little more about your experience and how you've been since reconstruction. I do hope that Mr Sandhu has transformed your breathing and your quality of life has improved without the trachy. Thank you so much for replying. Kind regards, Linda. email [e-mail]

[SusanCh in reply to Chappell8]

Hi Linda will email you Sue

[John_Mills Volunteer]

Just revisiting these posts 2 months later. Anything further to report?

BW John