Differentiating between SJS and possible Wegener's Granulomatosis? Imuran?

[rnathans]

Irish is a much better resource than I but I can comment on a few things. First though antibody negative my lip biopsy was positive and my teeth are fine. I have a mildly dry mouth, dry eyes but my systemic neuro symptoms are the worst-including gastroparesis. So yes Sjogrens causes GI problems. I was on immuran at one point for my Sjogrens so why not consider it for your RA and probable Sjogrens. I did have to stop it eventually due to low white counts but others tolerate it just fine.

If your skin biopsy shows sf neuropathy then IVIG may be a possibility for that.

[MAT51]

Thanks. Yes I am about to try Imuran providing my chest x-ray is clear and my white blood cells are okay next week. I'm actually quite excited about getting back onto an immune suppressant again sad as that may sound!

Because I depend on the NHS I am unlikely to be offered IVIg as it's very expensive and they mostly only offer it to people with demyelinating forms of neuropathy such as MMN, CIDP and Guillaine Barre. If my RA comes back in classic form then I'm told I will be offered one of the anti-tnf drugs - but it hasn't returned in this form for over a year now. Thanks for letting me know that, like myself your mouth and eyes aren't as badly affected as your systemic neuro symptoms. I have given up expecting anything from tests now apart from my high inflammatory markers which I'm told confirm my autoimmunity but that is all. It seems to me that Sjogren's gets a raw end of the deal where the medical profession is concerned - certainly in the UK.

[Jasper]

I found this to be a very interesting discussion (and not boring at all).

I just want to mention a few things which may be helpful to you. First, I do have a positive SS-A. I also have a positive lip biopsy. (One thing of interest is that my SS-A was negative in 2012, but was positive in 2013.)

My eyes are very dry and, before I started using Restasis drops, they felt like they had sand in them all of the time. However, I have never had conjunctivitis nor have I ever had any corneal ulcerations.

My nasal passages and sinuses have given me problems for decades. The sinus drainage was thick for decades (it is now thin since I have been using N Acetyl L Cysteine).  Plus, I had two sinus surgeries to remove sinus polyps and to widen the nasal passages for better drainage. In addition, I developed a nasal septal perforation from the constant dryness and inflammation.

My mouth is dry, but not nearly as dry as some people on this forum. My teeth are in excellent condition (so far). So, even though many with Sjogren's have very very poor teeth and gums, not everyone with Sjogren's has rotten teeth. I am just pointing out that, just because your teeth are still okay, it does not mean you do not have Sjogren's. In addition, if there is a change in the salivary glands and the the saliva production decreases more markedly, the teeth and gums will deteriorate rapidly too.

I do have some Gastro-Intestinal problems as well with the Sjogren's.

I also have Peripheral Neuropathy (Sensory Polyneuropathy/Ganglionopathy). This mainly affects my feet, ankles, hands, and wrists.

I was not diagnosed with Sjogren's until 2013. However, I have had symptoms of Sjogren's for decades.

In 1993-94 I had Systemic Vasculitis. My current Rheyumatologist says it was almost certainly due to Sjogren's, even though I was not yet diagnosed with Sjogren's. I was extremely ill when I had Systemic Vasculitis.  I had severe, overwhelming fatigue, severe chest and jaw pain, shortness of breath. I was off work for 7 months.

The reason I had those symptoms was because the vasculitis caused systemic inflammation of the entire vascular system which caused me to have pericarditis with pericardial effusions (inflammation of the sac around the heart with fluid in the sac around the heart), pleuritis and pleural effusions (inflammation of the sacs around the lungs with fluid in the sacs around the lungs), and hemolytic anemia (anemia due to the red blood cells being killed off).

My Rheumatologist at the time at first thought it MAY be Wegeners but he later decided it was not Wegeners. He tested me for various Auto Immune Diseases. My ANA was elevated as was my sed rate, but no specific tests for specific AI diseases were positive. I was treated with high dose steroids (70 mg a day) for months. Gradually, the symptoms abated and I improved.

I never had a rash with Systemic Vasculitis. Not everyone with vasculitis has a rash.

I have had no recurrence of Systemic Vasculitis since 1993-94.

I hope you can find out what is causing all of your symptoms and get appropriate treatment.

[Deb 27]

My antibodies for SJS were negative. They did a lip biopsy of the saliva glands and that was positive. It took years for someone to recommend a lip biopsy.... Also my x rays just turned up early RA. It seems like these darn diseases travel together.

I hope you can get some relief and help for your symptoms and condition. Sometimes you gotta get a little pushy with the Drs................. arm yourself with knowledge and ask questions!!!!!

[MAT51]

Thanks Deb 27 - sorry you have a similar mix of connective tissue diseases to me it seems. If Imuran doesn't help my painful teeth and jaw or my small fiber neuropathy then I will ask to have a lip biopsy and also ask for my autoantibodies to be rechecked. I think once you have a diagnosed  rheumatic disease the doctors stop trying to find the causes of things and just focus on treating the primary disease. I do worry that they put so much focus on joint damage and not very much on nerve damage but I guess, more than anything, I just want some mojo back so I can be an active member of my family and of society again. Rather than a pain ridden, sickly, bed ridden blob! X