Bronchocentric Glanulomatosis?

[Muddywater100]

We have literally had to fight to get a diagnosis of bronchocentric glanulomatosis for my Mum, after 5 weeks of steroids and her literally being reduced to a shell of a woman. She is currently battling with the terrible side effects of extreme depression, anxiety, insomnia and paranoia. I have been researching GPA however and I feel that she may have been misdiagnosed. Mum has had a tumour removed on the brain, had a sinus operation which I can't even remember the reason now, and part of her lung removed (they thought it was cancer but it wasn't.) She was blowing out scabs through her nose, had a continuously runny nose and a cough so bad that she could hardly breathe for months. She was eventually put on these steroids, which has cleared up the cough but she has lost the will to live. I'm at a loss as to how to help her. Has anyone else on this site heard of bronchocentric glanulomatosis or been misdiagnosed. I'm just thinking that maybe the rituximab treatment might be the answer. I don't have faith in the medical support that she has been given so far and feel that she is being pushed from pillar to post. Any suggestions or information will be greatly appreciated.

[Main1234]

I’m so sorry your mother is so ill and having such dreadful side effects from steroids.

You may find it helpful to contact the help line to discuss her treatment and diagnosis, they are extremely helpful and will offer excellent support and information.

Please let us know how your mother gets in, she is very lucky to have you in her side.

Best wishes to you both

[Muddywater100 in reply to Main1234]

Thank you so much. I will definitely get in touch with the helpline.

[Grizzly-bear]

I don't have lung involvement myself but bronchocentric glanulomatosis can be a symptom of vasculitis so it isn’t necessarily that the diagnosis is wrong and more that it’s incomplete. They should be looking at the cause of the bronchocentric glanulomatosis. At least some of the other symptoms sound very much like GPA - crusting painful nose and sinuses is pretty common amongst us with GPA (I had it for years before my diagnosis)

Rituximab should help if that’s on the cards but it can take about 6 weeks to kick in. Sometimes they initially also go with something more aggressive like cyclophosphamide alongside rituximab if they feel they need to get it under control fast (that’s what I had as I was in kidney failure at diagnosis - make sure they’re checking her kidneys too)

[Muddywater100 in reply to Grizzly-bear]

Thank you. This makes a lot of sense. I'm going to write down what you have said. Mum was admitted to hospital today and I'm going over to see if I can speak with the consultant tomorrow as he should be visiting her.

[Grizzly-bear in reply to Muddywater100]

best of luck! Hope you get some answers and start to see improvements for your mum soon

[Chipper03 in reply to Muddywater100]

Hi, I agree with Grizzly Bear, this may not be the whole diagnosis, and also consider EGPA, this type of vasculitis effects lungs especially, as well as other organs. The acronym stands for Eosonophilic Granulamotosis with Polyangiitis. If you have access to your mother’s blood results, take a look at the eosinophils, for me these have been the key marker. Keep asking questions until you’re satisfied, these are rare diseases. Good luck!

[Mooka]

Firstly your mother is so lucky to have you supporting her. Vasculitis is a rare set of diseases and it needs an expert to give a diagnosis. If her doctor doesn’t understand vasculitis she will continue to struggle. I was lucky my rheumatologist sent me to another hospital where I was diagnosed after my own doctor had ruled it out. You may need to ask for a second opinion or a referral to a specialist centre. Your first port of call should be the helpline though. Good luck. Let us know how you get on.

[yogarita1955]

definitely go for ritixamab it has changed my life don't be afraid it's the way forward it will help with her mental health it did for me I was a mess crying all the time go for it

[trijaws]

I don't think anyone on a "forum" can provide a proper medical diagnosis. However, your question about Rituxan...it's a wonder drug for those of us with GPA. She should get additional testing to ascertain if she has GPA or not. Best of luck to her.

[Johndeeregreen]

I have been dx with GPA for almost 2 years and after a very rough initial battle to get things under control, I'm been in relative remission with Retuximab infusions and Methotrexate. I did have a very recent disease flare, but Prednisone at 10mg has calmed down the lung problems and nose bleeding. I just had another Retux infusion and it will take it a while to start working and I'll taper on the prednisone.

I've not had any noticeable side effects from the Retuximab. I take oral methotrexate weekly (8 pills) and only feel a little blah from that. But it's hard to know since GPA and most vasculitis diseases make you feel blah to bad.

I would recommend Retuximab if she has a solid dx of vasculitis and especially GPA. But, biopsy is the only full proof test. I watch my CRP levels with frequent blood tests to gauge inflammation levels.

This is my first flare and first time to have noticeable breathing issues and a persistent cough that was confirmed with CT of my lungs that I had changes indicating a disease flare.

Hang tough,

[Red-head44]

how are things for your mum now - what is she getting? Having read her story I’d say definitely GPA. I’ve had it 7 years and by the sounds of things she needs immediate treatment on cyclophosphamide as it works instantly - she needs help now. They tried to send me to another hospital to have my whole right lung removed but thankfully that hospital resisted or I’d only have one lung !!! This was before my diagnosis as they couldn’t find what was wrong. It wasn’t until my lower legs came out in a huge rash that crusted over that they took a biopsy that told them what it was. But that was weeks after the stage your mums at. Demand a referral to a vasculitis centre. I too had a nose operation a year before this trying to stop the pain in my face and still no diagnosis.