New diagnosis.

[hugs84]

Hi everyone. I'm new here. I apologise if my query is already answered in other groups. I had a look through and couldn't find specifically what I'm looking for.

This is going to be a bit long winded so please bare with me.

I became pregnant with my second child in late January last year, and by 8 weeks had developed an eczema-ish rash over my entire face.

The intensity of it came and went,with specific flushing of my chest,cheeks and the bridge of my nose. I was told it was the mask of pregnancy and pregnancy "plaques".

At around 30 weeks the rash came back with a vengeance,was thick and purple over my entire face,neck and chest. I had nocturnal diarrhoea and severe breathlessness. I was told it was a mixture of hormones and anxiety. (These symptoms continued for the whole of pregnancy)

I had reoccurent unshiftable UTI'S that developed into a kidney infection.

I then developed flushing,swelling and tracking down my left arm and across my chest and lymph nodes in my arm pits,left breast and around my collar bone. Chest xray revealed nothing. Scans for pulmonary embolism all clear xray for sarcoidosis clear.

I had my baby and 1 week post partum developed a massive swelling of the big toe joint on my right foot and high uric acid. Was told I had gout.

Was sent to immunology who ruled out carcoidosis and lupus but said blood tests showed raised esr and raised IgA.

Developed severe joint pain in my wrists and thumb joints, muscle pain and upperbody weakness and severe fatigue. Began getting numbness and tingling down both arms and loss of fine movements in my fingers. I already have raynauds, and developed painful blisters on the ends of all my toes which I was told were chilblains.

Sent to rheumatology who said gout is extremely unusual in pre menapausal women and that he didn't feel I was symptomatic of a rheumatic condition and that I should take a "holistic approach"with diet and exercise.

Went to neurologist who is sending me for scans of my head and neck re: numbness. I have swam every night but the pain and weakness in my upper arms is getting worse.

Which brings us up to this week.

I received a letter that I was copied into from the rheumatologist to my g.p.

The letter states that I have raised IGA, ESR "and with the presence of anti C1q antibody urticarial vasculitis is extremely likely".

I know nothing of this disease and my g.p has admitted she isn't particularly knowledgeable but has said there are 3 different categories depending on the levels of complement proteins. Which do I have? Is it nuv, huv or huvs?

I'd really appreciate if someone could decode it all for me as I'm not sure which "type" I have. An overview of my y blood tests are as follows:

Anti c1q antibody (high)

C3 within normal range

C4 within normal range

Iga raised

Esr raised (14)

Uric acid now normal

Aswell as this I have underacrive thyroid wirh hashimotos but my levels are stable. I have a high TPO antibody.

I have raynauds,and reoccur enter severe coccyx pain with no known cause.

I have reoccurent stomach problems and was diagnosed with non coeliac gluten intolerance.

I had childhood alergies and asthma. I have a family history of R.A and SLE.

Also,the rheumatologists suggested treatment was antihistamines. Is this a long term solution? My next appointment isn't until May so any answers I can get before then would be really helpful. I've tried to Google it but can't find any info really. Is it rare? Thanks in advance. Xxx

[PMRpro]

I can't comment on your results but I have one important thing to say: find a new rheumatologist at the very least.

"Gout is a condition characterized by the deposition of monosodium urate crystals in the joints or soft tissue. The four phases of gout include asymptomatic hyperuricemia, acute gouty arthritis, intercritical gout and chronic tophaceous gout. The peak incidence occurs in patients 30 to 50 years old, and the condition is much more common in men than in women. Patients with asymptomatic hyperuricemia do not require treatment, but efforts should be made to lower their urate levels by encouraging them to make changes in diet or lifestyle. Acute gout most commonly affects the first metatarsal joint of the foot, but other joints are also commonly involved. Definitive diagnosis requires joint aspiration with demonstration of birefringent crystals in the synovial fluid under a polarized light microscope. Treatment includes nonsteroidal anti-inflammatory drugs (NSAIDs), colchicine, corticosteroids and analgesics. In patients without complications, NSAID therapy is preferred."

If the peak incidence is in patients 30-50 years old - for the majority of women these days that is pre-menopausal.

A raised ESR is pretty meaningless - all it shows is that there is some sort of inflammation somewhere and it is raised in pregnancy - although if someone told me their ESR was 14 I would not classify it as raised, the normal range is usually quoted as up to 20.

You don't say where you are - and I know that that is something that is relevant to advice given you so knowing country and region would be useful.

[hugs84 in reply to PMRpro]

Thanks for replying

Does anyone else have any idea about my results?

[hugs84 in reply to PMRpro]

I am in Cambridge, UK.

[Hidden]

Hi,

If you are in Cambridge then I would ask for a referral to the Vasculitis and Lupus clinic at Addenbrookes Hospital headed by Dr David Jayne.

Vasculitis is a rare and difficult disease to diagnose and HUVS is particularly rare. You need to see someone experienced who can put together your results and symptoms to come up with a definite diagnosis.

You can also e mail or phone the VUK helpline to discuss this further. We can't give you a diagnosis but can help with questions to ask.

Best wishes

Keyes

[Hidden]

vasculitis.org.uk/helpline

Helpline details.

[hugs84]

Thank you Keyes.

I am currently at Addenbrookes and my Rheumatologist specialises in vasculitis so I'm happy I'm being taken care of. Just so frustrating having to wait so.long til my next appointment!

Can I ask though, in the research I could find online,everyone with huv seems to have this anti c1q antibody that I have. Does this antibody with diagnosed vasculitis automatically mean huv or could it still be nuv? I am clueless!!

[Hidden in reply to hugs84]

Hi,

You can get false positives with all blood tests which is why they shouldn't be taken in isolation but interpreted with your other symptoms by an experienced Dr.

Quite frankly I am concerned that you are having to wait so long to be seen again especially with some of your symptoms. Raised IGA can go hand in hand with another type of Vasculitis called HSP. I am not aware of any research that supports a " Holistic approach " as an effective, stand alone treatment for Vasculitis. Anti histamines do help Urticarial Vasculitis but won't prevent end organ damage from occurring if you do have HUVS ( which is very rare and can have serious consequences ).

All you have to go on so far is a mention in a letter from your Consultant but it sounds to me that you are still in the dark as to your diagnosis and what everything means. I don't think that is being taken care of!

[jenstarblondie in reply to hugs84]

Hi there, the very same thing has happened to me!! I'm under Addenbrooks too and by chance I had the blood test and it says I've tested as C1Q positive, I'm really worried about it! In between waiting for blood test results I'd gone privately as so many other issues going on. I have just been diagnosed with Ehlers-Danlos Sundrome and it can all be connected apparently. My Nan has Lupus and I'm wondering if I'm also heading that way!! Addenbrooks have started me on Triple Therapy (Ranitidine,Citrezine and Motelukast) they said my next appointment is September and it seems so far away, they have offered no explanation like you've experienced?! My private consultant is going to write to them and wants me to have bladder biopsies etc in regards to the Ehlers-Danlos (I've been suffering from chronic bladder infections) and he said triple therapy is a good place to start for Uticarial Vasculitis but nobody knows how I've got it??? To cut a long story short (visit my page on Facebook to see more and connect with me,The Grumpy Bumpy Foundationas I'm raising awareness and supporting lots of Mums.) I had a child in 2007 and a child in 2012 both I experienced excruciating pelvic pain and with second, spent last three months of pregnancy in hospital. Not been well since, joint pain and swelling, fatigue, low mood, neurological problems. I was in and out of hospital and then last year spent 4 months in Addenbrooks, I had front of pelvis removed, bone grafts and plates. I've not recovered well and gone so down hill!! They couldn't get to bottom of it, rheumatologist was so dismissive until this blood result came back as C1Q positive. I'd already taken it into my own hands and gone private and now am full blown Ehlers-Damlos which explains the pelvis damage and lots of other issues but it's so much to take on on top of the Uticarial Vasculitis. Like you, I think it means we have HUVS but I don't know and am just as confused and worried as you! Please let me know how you get on xx

[hugs84]

Thanks for your concern but i only received the letter on Friday. If you re read my o.p the holistic approach the rheumatologist mentioned was in relation to the muscle and joint pain and fatigue- before he had run any further bloods and new about the anti c1q antibody and vasculitis - so he probably wants to rule out lack of exercise as a cause. Everyone believed the symptoms I had/am having are pregnancy and hormone related. It is fair to say though that everyone had failed to take me seriously.

My initial follow up appointment was for 6 months, the end of July, so it now being brought forward to the begging of may, nearly 12 weeks, is pretty good I think.

He has also adviced the g.p to prescribe me ceterizine until our appointment, which I see is a common treatment, so it's not like he's ignoring my diagnosis.

Im not looking for anyone here to diagnose me. My original query was to know about the different types of urticarial vasculitis and how they are distinguished in relation to the differing levels of complement proteins. And how common it is in the uk and world as a whole.

It's frustrating that there appears to be no information out there and even g.p's are so poorly informed.

[Hidden in reply to hugs84]

You might find this helpful

vasculitis.org.uk/about-vas...

The problem is that there are more than 7000 rare diseases which is why information is so scarce and GP's have a poor understanding. Many never come across a case in their whole career.

My understanding is that anti c1q antibodies are unique to HUVS but don't know definitively if it's possible to get false positives.

I get the feeling that I have upset you, that was never my intention. I think there are only about 100 cases of HUVS worldwide which is why there appears to be a lack of ready information and it's important to see a real specialist in Vasculitis. I was just questioning whether a few lines in a letter and making you wait another 12 weeks was appropriate. Of course it's just an opinion which I am happy for you to disregard.

[hugs84]

No you didn't upset me! Thank you, that is very helpful! Sorry if it came across as me being a bit sharp, I was trying to give an accurate account of my situation

What you say about only huvs patients having the c1q antibody is exactly what appeared to be the case when I researched. What I don't understand though is that I am nowhere as sick as is described by the other sufferers; I no longer have that rash, which appears to be characteristic of the condition. I'm not aware of any organ damage although could it explain why I'm getting gout? Are the excess purines in my blood from my own muscle/tissue breakdown? I'm massively speculating here.... my worst symptoms now are extreme fatigue, really painful wrists and upper body muscle pain and weakness, raynauds and blisters on my toes. Plus I don't know when I last did a solid b.m (tmi, sorry). Could this really be huvs? I guess you're right, that we really don't know until I see a specialist again. 12 weeks doesn't seem that long a wait to me, but then I've never had a rare condition before and maybe I'm a bit naive about how dangerous this is?

Thanks anyway. I'll let you know how it pans out. Xx

[Hidden in reply to hugs84]

I know you said in your post that you are seeing Neuro and are going for scans, are they going to do nerve conduction tests as well as the loss of fine movements in your peripheries could point towards peripheral neuropathy. I would keep a close eye on the blisters as sometimes it's not apparent what tissue damage is going on underneath.

Inflammatory bowel disease can go hand in hand with Vasculitis, has anyone ever checked a faecal calprotectin as that's very sensitive for information in the bowel ( your GP can request it )?

Part of the problem is that I can only speak in generalities and a lot of Vasculitis damage can be insidious, especially in younger patients as the body has a wonderful ability to compensate that disappears as we get older. Pregnancy can often be a trigger for Vasculitis. Your specialist holds the key to the information, I suppose I am surprised that you have tested positive for cq1 but aren't being reviewed again for 3 months. I don't wish to alarm you but feel that the worst case scenario should always be ruled out.

Please let me know how things go and I am sorry for being too sensitive!

[hugs84]

No problem. I'll let you know how it goes. X

[Lupylass]

Hi there

I've come to this late and unfortunately can't answer your specific question about your positive antibodies and which type of UV you may have.

What I wanted to say is that I have UV with low complement and mine is associated with lupus (it took my doctors a while to get to the lupus). I don't have any organ damage but have had most of the symptoms you describe - and I have coeliac disease with that diagnosis coming later.

I started on antihistamines before requiring more aggressive treatment.

These days I'm really well.

I'm telling you all this just to say that it may take a while to get to the bottom of it all but keep asking questions and you're doing everything right by learning about your condition for yourself. GP's are very limited in their knowledge and do defer to the specialists much of the time (I work in a GP practice)!

I hope you get some answers soon. Like you, I had to wait a while, but I have a super rheumatologist who has got me well and for that, I'm very grateful.

[dianamarie]

Hi...sorry to hear about what you're going through...acute urticaria can be associated with Hashimoto's thyroiditis, about 30% of chronic Urticaria are! and it is treated with first-generation anti histamines like benadryl, and also second generation anti histamines like claritin...fg's will make you more tired with the side effects but I think seem more effective..and yes diet does play a part of that condition, you need to avoid histamine releases like strawberries, tomatoes, preservatives and coloring agents, triggers to avoid also are alcohol and aspirin. If they think it is vasculitis variety then they should biopsy the lesions immediately, especially if you have autoimmune disease such as Hashimoto's or possible lupus. Apparently other conditions that present those symptoms as well are mastocytosis, SLE, vasculitis, hepatitis and lymphoma...angioedema is on the face, and also in the lungs and the mouth and causes shortness of breath, as you described! It can be very serious even leading to the closing of the airways! I'm surprised they weren't more diligent and quick in diagnosing you!

But you mention your rash was purple, vasculitis urticaria, it's typically a red rash that they call wheals, and sometimes can be produced simply by stroking the skin. They show typical pictures of it on websites including the one I mentioned below..

Here is a great article for you to look up if you have the Internet! The author also has some articles about postpartum manifestations... It is called..

Urticaria: Evaluation and Treatment

by American Family Physician- dated May 1, 2011, authored by Paul Schaefer, M.D.

I think it might answer a few of your questions!

Well good luck on your journey, and knowledge is power, so keep reading, keep searching until you find all your answers! ....sometimes we need to present to the doctor with our own research, because they do not know everything all the time.. Lol

[dianamarie]

Oops, forgot to mention one more article that's really good about HUV...

Hypocomplement Urticarial Vasculitis Syndrome by A Buck, 2012.....just enter it on Google it will pop right up.

...this describes a lot of the symptoms that you're having and also diagnosis is confirmed by skin biopsy revealing leukocytoclastic vasculitis, LCV...

Wondering if you had ANA testing?...seems you should have had a more aggressive work up, and biopsies to me!

[PaulaC]

Hi, I was diagnosed with HUVS in 2010. Initially my symptoms were fatigue, urticaria, swelling of the joints and mild swelling of the throat. It started in March 2010, my GP and his colleagues were totally stumped - didn't have a clue. I had private health care and was sent to several different specialist including an allergist and dermatologist - no suggestions what so ever. In the October I started with Uveitis and Episcleritis, it was the Opthalmic surgeon who suggested HUVS, which was then confirmed by the dermatologist and a rheumatologist in Oxford, who gave me more detail regarding my complement levels, antibodies etc. I'm sat here 6 years later still not 100% what it all means though!

The point I am getting round to is that for many years I functioned normally with the illness, localised medication ie anti histamine for the rash, painkillers for the joint swelling and pred forte for the eyes, I managed a very stressful full time job, ran a home and raised 2 teenagers (at the time). Like you, I did not recognise my illness as the one described (so scarily) in the online literature. Unfortunately I developed COPD which put a whole new slant on it, I won't go into detail but there have been a couple of life threatening situations in the past 15 months which has caused me to rethink the whole illness.

You appear to be in a better position than I was, you have a diagnosis quite early and I understand Dr Jaynes team at Addenbrookes are THE people to see (I am in Jersey so the vasculitis specialists in the Uk are difficult for me to have access to).

Write down lots of questions (even if they appear stupid), keep an open mind, take it seriously but don't think worst scenario (there is great variation with this disease).

Somebody earlier mentioned the Vasulitus website, lots of information and there is a help line, if you are on Facebook, the Vasulitis UK (Stuart Strange Vasculitis Trust) is a good place to ask questions (although it isn't UV specific). I recall there was a posting a few weeks ago from a lady with similar issues to you that had a lot of advice offered.

Good luck - make sure you get your answers!