Possible vasculitis diagnosis looming...

[nicole92]

Hi guys!

I'm new here, and I just wanted to ask a few questions about diagnosis of vasculitis. After being ill since May, I was finally diagnosed with autoimmune hepatitis around September 1st and started on 9mg of oral budesonide per day. Just over a week ago the few little red dots I noticed on my feet started to spread, and last Monday I came up in a bright red rash all over my lower legs and the tops of my feet, which started to spread up my thighs and onto my arms a little bit. I got an appointment with an out of hours GP as they were worried about some sort of severe infection due to the budesonide (which I had been taking for about 9 days at that point). GP admits me, and on the Tuesday I had an ECG, chest X-Ray, ultrasound to check up on my liver, loads of bloods and a skin biopsy. I was told that it was a vasculitic rash, and they were testing my blood for ANCA, plus a load of other autoimmune markers. I had had no infections recently and no evidence of anything was seen in my throat, ears etc. That Monday night there was protein in my urine, but none was found when this was repeated twice on the Tuesday. I was given Dermovate, and discharged on Thursday as the rash was clearing up well (and is now nearly gone).

I've had allergic rhinitis (stuffy/runny nose and a cough caused by post nasal drip) for a couple of years now, with no clear trigger and only minimal response to antihistamines. I also feel very tired a lot, but this could easily be due to the hepatitis as the fatigue is a long standing problem. Furthermore, I've had a lot of bad stomach pain recently, often but not always after meals. There's no clear correlation with food, and this morning I was in a lot of pain almost as soon as I woke up, but tonight after tea there was no pain. I've had right upper quadrant pain/discomfort and nausea for quite some time because of the liver problems, but I think this pain is a relatively new thing. Yesterday morning I realised that my left calf, which was where the rash started and was more affected, has felt like I've overworked the muscles a bit through exercising even though I know I haven't. It's a bit better today but driving in traffic yesterday was becoming very uncomfortable, and if I press the muscle it's sore.

In my infinite wisdom, I decided to get out my textbooks (I'm a medical student) and try and learn a bit before I get the results of my skin biopsy and bloods on Thursday morning. I read about all kinds of scary looking autoimmune vasculitis conditions, but I don't really understand a huge amount about diagnosis. I know that the ANCA result will guide them a bit, but will a skin biopsy be able to confirm a particular disease (i.e. Wegner's, Churg-Strauss etc.), or will it simply tell them that it was some kind of vasculitis? By the way, I'm 24 and prior to the AIH diagnosis I was fit and well. I could see the AIH diagnosis coming as soon as my GP said hepatitis back in early July, so although I worry about the effect it might have on my life a bit I at least had some time to process things. I think I'm just a bit scared of being told that there's something else wrong with me, and that it's going to have a big impact on my life.

If anyone has any advice, either about the process of diagnosis or just in general it would be greatly appreciated!

Thanks for reading!

[Hidden]

Hi Nicole,

Vasculitis is a clinical diagnosis made on the presence of symptoms, bloods, biopsies and imaging scans etc. As its a rare, complex condition your best chance of getting a diagnosis is to see a Consultant who has experience in diagnosing and treating it.

There are over 18 variants, only 3 are associated with ANCA but you can have an ANCA associated Vasculitis ( AAV ) but be ANCA negative. You can also be ANCA positive but not have AAV. Some patients never mount an inflamatory response so ESR and CRP can be normal as well. Biopsy can be very useful in diagnosis but much depends on how deep they go, the experience of the person doing the biopsy and a pathologist who recognises the appearance under the microscope. The Granulomatous lesions etc that can be characteristic of Vasculitis aren't equally spread along the blood vessels so it's possible to get a false negative result.

As the treatment for Vasculitis carries risk ( immunosupression ) Dr's tend to have to be very sure before being prepared to make the diagnosis. Of course now you are wondering how any patients manage to get a diagnosis!

You are welcome to phone or e mail the VUK helpline for advice and we can point you in the direction of the nearest experienced Consultant.

[nannysaurus]

Hello, I also got diagnosed with auto immune hepatitis ( caused by an antibiotic) I also had the rash which they did a biopsy on it came back as Vasculitis and my blood tests showed anca positive. I developed rheumatoid arthritis and the pain and fatigue is sometimes unbearable. Unfortunately my rheumy is infatuated with fibromyalgia and will not accept anything else even though I have sinus problems, kidney problems and lung issues!!!! Good luck with getting a diagnosis. There is a brilliant closed page on Facebook called Vasculitis UK. The amount of info they have is more than all the rheumys in the world, as everyone has a form of vasculitis. All the best xx

[Hidden in reply to nannysaurus]

That sounds awful, have you gone for a second opinion?

[nannysaurus in reply to Hidden]

I have asked my GP to refer me but he has ignored me and now retired!!! I was walking around with a broken leg last year for 3 months I saw nurse practitioner,GP and rheumy, they all said fibromyalgia I had to burst into tears in rheumys office and she begrudgingly sent me for a non urgent scan of ankle,leg after which I got urgent phone call to get my leg put in plaster!!!! Still is agony but it's fibromyalgia again ( no scan)

I am at the Norfolk and Norwich hospital. Initial diagnosis by vasculitis specialist but he retired too

I am too tired to keep fighting and being looked at like a hypochondriac.

Sorry for the moan!!!

[Hidden in reply to nannysaurus]

No need to apologise. I had a very torturous route to diagnosis with multiple second opinions and been labelled as having a " complex psychosomatic illness " along the way.

I never gave up hope though and now am much better on Infliximab and Methotrexate.

Who do you see at Norfolk and Norwich?

[nannysaurus in reply to Hidden]

I see Dr Marshal. Who is extremely scatty, always running around and , who I think, gets paid a tenner for every fibromyalgia diagnosis she makes!!!!!!

[Hidden in reply to nannysaurus]

Hi,

There is a Dr Chetan Mukhtyar at Norfolk and Norwich. He is a Rheumatolgist with a special interest in and great experience of Vasculitis. Worth asking for a referral.......

nnuh.nhs.uk/people/chetan-m...

[nannysaurus in reply to Hidden]

Thanks so much, will have a good try to see him ( even if I have to let my rheumys tyres down!!!!)

[PMRpro in reply to nannysaurus]

Couldn't agree more with Keyes. Blind rheumies seem a rather common experience. I'd be looking for another - pdq.

Where are you?

[Hidden]

Nicole, I am sorry to read about your symptoms.

Maybe it helps when I tell you that for me it also started with autoimmune hepatitis 16 years ago and while this vasculitis thing is still with me, (that diagnosis arrived only 7 years ago) I have been able to have a pretty amazing life since the first diagnosis.

The hepatitis was diagnosed after a routine lab test found elevated liver enzymes after a bacterial infection (septicaemia) which was treated with penicillin. This was followed by a liver biopsy with tell tale findings. Also, ANCAs were found for the first time and these critters have been with me ever since.

Have you had a liver biopsy and/or MRI of the gall bladder and bile ducts?

I don't think your abdominal pain is liver related as the liver "knows no pain". The nausea, yes. Any itching? Yellow eyes?

It could be more to do with the gall bladder, has anybody ever mentioned autoimmune cholangitis? It often overlaps and the treatment with budesonide is the current gold standard in any case. Gall bladder pain can be quite awful but thankfully it comes AND goes.

My liver recovered within 18 months and I have had a few small flare ups but nothing dramatic. Make sure to have your liver enzymes and inflammation markers tested regularly and if possible by the same lab for comparison.

I recommend a bland-ish diet for starters, just so you can determine what your bile ducts and your liver can handle without too much trouble. In my case, digesting fat, red meat and large portions in general are problematic. I also cut out all alcohol. Yes, I know but still . . .

I started out with the hepatitis, this was followed by ulcerative colitis (which is currently in remission but raises its ugly head from time to time), keratitis and since 2009, ANCA vasculitis (or Wegeners or GPA) mainly with ENT involvement. I had this sinusitis stuff going on for years before it became pretty awful incl. vertigo and hearing loss (fully recovered thanks to cortisone).

You need a good rheumatologist/immunologist who will work in tandem with your GP with regular lab work and general monitoring. You also should look for an ENT expert who will check and monitor ENT symptoms and a dermatologist who knows their stuff. Possibly also a regular gastro check-up incl. colonoscopy. Vasculitis comes in many shapes and forms.

Don't panic, you have been diagnosed early and the treatment options have become so much better in the last 15 years.

If it helps: Since my initial diagnosis, I have travelled widely, climbed lots of real mountains, cycled long distance several summers, renovated a house, and kept my job throughout - with a couple of restful breaks in between.

All the best!

[JontyW]

Hi Nicole,

Regarding one question you asked in your original post ......

"but will a skin biopsy be able to confirm a particular disease (i.e. Wegner's, Churg-Strauss etc.), or will it simply tell them that it was some kind of vasculitis? "

I was told by my rheumy when dx with Churg-Strauss Syndrome (EGPA) in April 2013, that it only confirms it is vasculitis, and not which type.

Wishing you all the best ...... Jonty

[Hidden in reply to JontyW]

That's not entirely true Jonty. The findings on skin biopsy can point to a specific type of Vasculitis. Esinophils can be found in CSS, IGA in HSP etc.

[jackrussell]

My vasculitis. And type was diagnosed with a skin biopsy ,I have had a skin biopsy twice in 25 years and both times the same diagnosis was made, so yes, which type of vasculitis you have,can,at least in some instances be diagnosed by skin biopsy.