Polyarteritis Nodosa - awaiting diagnosis

[Tc3844]

Hi folks,

I am new to this site/forum but have found it excellent.

My detailed story so far.....

January 2016 - I started to have bladder control problems. I had been exercising at the gym and I kept getting off the treadmill, needing to wee every 5 - 10 mins. A few weeks later, I wet myself as I was driving home on two occasions. The feeling of needing to go just came in seconds and by this time, I was already wetting myself.

March 2016 - I had a nasty fever which wiped me out and I was in bed for a few days, terrible night sweats, started to lose a little weight. These symptoms eased but came back a week later, even more extreme and I began to lose weight further. Ended up in hospital and my bloods were fine apart from my eosinophils were either nonexistent or very low. However, they were not concerned about this. My spleen was enlarged and they tried to culture any bacteria/virus in my blood but nothing was there. They were perplexed as to what was going on. An oncologist appeared at the end of my bed and I eventually had a bone marrow aspiration and a sample of bone taken. Results were all clear...no cancer or abnormal blood conditions.

April/early May 2016 - It took my 3 weeks to get my energy back and feel relatively normal. In this time, I had awful lower back pain which subsided after 2 weeks. On leaving hospital, they said that it could have been a virus which my body eventually fought off.

June - August 2016 - I then had intermittent extreme stomach pains which took me to hospital on three occasions. It was as if I was being stabbed. Yet again...full blood count was normal. I was CT scanned to death looking for kidney stones and a number of other things but all was clear. I was discharged on each occasion but there was no appetite to investigate the problem. It was at this point I knew there was something more deep routed going on. I also had a colonoscopy and endoscopy. All clear.

October 2016 - I was still having stomach problems and I decided to pay privately as the wait for an urgent appointment was 8 months!!!!! I made the appointment on Friday and saw the consultant gastrologist/Heptologist on Saturday. An amazing man - Dr Bohan (Royal Stoke Hospital). He said immediately that after reviewing my symptoms, that it was not a gastro related illness but a sistemic problem. Something is causing these plethora of symptoms in your body and we need to find it. He requested a number of tests - a number of antibody related test, 2 x Lyme disease, ANCA, Thyroid test and a 5-H11A urine test.

I ended up back in hospital at the end of October in Cheshire (Leighton Hospital) and they completed the tests requested by the Dr Bohan.

The results were that I had an under active Thyroid as my peroxidase antibodies were showing 420 and not between the normal range (0 - 9). The ANCA was negative and everything else was normal. I started Levothyroxine to replace the missing thyroxine.

Hooray....a diagnosis. I began to feel better....however...I still noticed that since September 2016, if I exercised I had a burning feeling in my legs and there was an area of my left leg which didn't feel right. I immediately had to stop. If I carried something heavy, I had a very strange cold sweat appear immediately and I just felt weak and very odd. If I did no exercise, I felt better. Odd..I thought!!!

November 2016 - I then developed a very painful left testicle which presented as a deep pain which would not go away. My linguine nodes either side of my groin were enlarged and painful. I went to the doctors after a few days. He examined both testicles, no lumps or bumps. At this stage, my spleen was still enlarged. I was prescribed Ciprofloxcin as the doctor thought I had an infection in the testicle and the nodes went down almost within 8 hours. Excellent...I thought!! However, my testicle was not as painful but just felt like it was detached from my body???? As if I had lost a degree of sensitivity.

December 2016 - (Bombshell!!!!)

I saw Dr Bohan and I explained my new symptoms I.e burning legs, cold sweat on exercising and testicle problem. He then stated that he believed I showed symptoms of Polyarteritis Nodosa. He asked me not to Google it and explained it was treatable but he needed to refer me to a Rheumatologist urgently. I also started to get deep rooted, localised pain on the right side of my head. Not like a normal headache and a pain in my left eye.

January 2017 - On 2nd January, the headaches got worse and I felt pressure behind both eyes, but mainly my left. Headache moved to my left side. At 10am, I lost sight in my left eye for 2 minutes and it came back. At 8:30pm, headache got worse and I had pins and needles down the left side of my face. Off to A & E. CT scan in head was showing no damage or bleed but they treated it as a TIA. I thought.. "Bloody hell...I am only 39 years old". I was then referred to TIA clinic. Stoke doctor said that he was happy I had not had a TIA and would treat me for migraines. However, as Polyarteritis Nodosa was being considered,an MRI scan would be required to examine the 'Circle of Willis' in particular and other parts of the brain.

I had an ultrasound on both testicles and all that was picked up was a groin hernia which my GP wanted to make a routine appointment to discuss.

Telephone call - Rheumatologist appointment made 23rd January:

I was at my wits end....if I have PAN, has it gone to my optical nerves and brain. I know that this is not a good area for the disease to present. I then had a call yesterday (20th January) from a dedicated, Rheumatological hospital in Stoke on Trent (Burslem). Dr Bohan had called the head of Rheumatology personally as well as sending my referral as a 'proper urgent appointment'. Instead of waiting 14 weeks (current waiting time for urgent appointments), I had waited 8 days for the appointment to be made.

I know it is a detailed post but can I ask the following from people:

1) Would other PAN suffers please contact me and let me know if my symptoms have presented in their lives? It would also be good to message fellow people going through the mill!!

2) Is an ultrasound definitive in ruling out PAN symptoms in the testicles?

3) If these symptoms have linked to other vascular disease, please message me.

In my experience so far, I have learned that getting a good diagnostic lead has been down to luckily finding a good doctor (Dr Bohan).

Good luck to everyone else who is suffering.

Regards,

Tony

[Suzym2u Moderator Vasculitis UK]

I do know another guy with PAN I will see if I can contact him. PAN is extremely rare incidence of 1/million per year. So it is important you are seen by a doctor who has experience and knowledge of vasculitis. Only a very small percentage of patients have testicular involvement. I will get back to you.

[Tc3844 in reply to Suzym2u]

Sue,

Thanks for your reply. I do understand the implications if this is diagnosed, especially in the cerebral area. Whatever it is, it appears to not to want to go into remission and continue to 'attack'. I do hope it isn't PAN, even though Dr Bohan has stated it is treatable.

I will be seeing Dr Khan on Monday, head consultant of Rheumatology at the specialist hospital in Stoke.

[tetrapana]

Hi Tony, good luck tomorrow. I can't help with your questions but we share the same local hospital. Since being diagnosed with vasculitis in 2012 I've been looked after by the renal team at Stoke, my consultant is brilliant.

I don't have PAN, I have MPA, ANCA+I've.

Keep us posted.

All The Best

Geraldine

[Tc3844 in reply to tetrapana]

Geraldine,

Thank you for your kind reply. What should I have expect from the first appointment? Do they do much? Should I write down a summary of my symptoms?

I am lucky to have had MRI and CT scans of my head sent across from Leighton to Stoke. My doctor has also sent all test results, including testicular ultrasound etc. Therefore, the consultant at Stoke will have something to review.

[tetrapana]

Hi Tony,

I guess they will want details of all your symptoms and will want to do blood tests in addition to possibly other tests. My circumstances were different, I was admitted to Stoke as an emergency after worsening renal function.

As PAN is very rare it might be worth asking the Rheumatologist at Stoke what experience he has in this respect.

[Prudencepig]

I was diagnosed with PAN 4 years ago after suffering fatigue and dizziness for 8 weeks which resulted in me only lying down as kept vomiting. Finally had a bed and then whilst in hospital for three months had a stroke in my left eye. That's when they were able to diagnose me. I u derwent chemo and huge doses of steroids which are now vastly reduced. I function really well back at work full time as a social worker and have very little symptoms. I too was fortunate to have a fantastic dr who monitors me closely.

Hope this helps, no problems with testicles as female.

[Tc3844 in reply to Prudencepig]

Prudencepig,

Thanks for the message. I am very intrigued...if you have read my post, did anything I had written match with your symptoms? Please let me know. I have my first rheumatologist appointment tomorrow.

I also did not mention that I have felt nauseous for 3 - 4 months. Of late, I have found it harder to remember things and think clearly. Still getting bouts of headaches at the back of my head and pains down my neck.

[Tc3844 in reply to Prudencepig]

Prudencepig,

Thanks for the message. I am very intrigued...if you have read my post, did anything I had written match with your symptoms? Please let me know. I have my first rheumatologist appointment tomorrow.

I also did not mention that I have felt nauseous for 3 - 4 months. Of late, I have found it harder to remember things and think clearly. Still getting bouts of headaches at the back of my head and pains down my neck.

[Prudencepig in reply to Tc3844]

I lost three stone ....good diet. I had no symptoms prior to becoming ill....my diagnosis was due to the stroke in the eye which was six weeks into the hospital stay. The bleed was in the part of the brain which makes you feel sick in the nape of my neck...

I returned to work six months after it started whilst still on chemo, thinking about it I am a tough cookie lol

My life is restored but my memory is Norway as good as it was but I function well.

Often tired sleeping nine hours a night but manage ok.

Let me know how you get on.

Good luck

[Hidden]

Hi Tony, sorry to hear that you have been having such a hard time with PAN. I was diagnosed with systemic PAN in June 2007 and I hope next month will see me being taken off mycophenolate at long last but my symptoms are very different to your own and also being female I wont have some of your problems. It does tend to affect people in different ways, when I get a flare up I get nerve damage along with dangerously high blood pressure and heart rate which has put me at high risk of stroke, heart attack or both but fortunately I made sure I got to hospital at first severe signs and was given high dose intravenous steroids. You are right when you mention the most important thing with PAN is a good consultant that has experience with it.

Good luck and I hope you get good news in the future

[Tc3844 in reply to Hidden]

Interesting....About 3 weeks ago, I started to get very very red gums with a white edge. It isn't painful but it does bleed when brushing my teeth.

I went to the dentist this afternoon to see what it might be. She said, "It looks like lichen planus." I am lost as to where I am going with any potential diagnosis. Did you have any gum/mouth involvement?

[Hidden in reply to Tc3844]

I haven't had gum problems and I haven't heard of anyone else as a result of PAN but it could be that if you immune system is compromised you are going to be prone to these kind of things. Just keep going to Dr's and get things checked out, don't delay.

[Tc3844]

1st appointment with rheumatologist-

(23rd January)

Had first appointment with rheumatologist regarding suspected Polyarteritis Nodosa at Rheumatological Hospital in Stoke on Trent.

Was a positive appointment and Dr Vermaak made very detailled notes. There was no rush to get me out the door and it was reassuring to know that she had treated PAN previously. She went through my full list of symptoms.

She gave me a physical examination and I made sure that my head MRI and CT scans were sent across from my local hospital, together with testicular ultrasound images.

She seemed to think that I was not presenting with symptoms of PAN. If fact she was perplexed as to any direction of a diagnosis. However, she wanted to rule out PAN.

She stated that I would need a CT angiogram, nerve tests, urine dip test and a referral to the infectious medical department for more detailled blood tests (as she wanted to test for rare virus/bacterial infections). She informed me that my MRI scan was fine, no lesions or abnormalities.

My testicular ultrasound highlighted a cyst on one of the tubes of my left testicle, a sac of fluid on my left testicle (called something with 'V' ) and a stone in my right testicle.

If things were discovered from the tests, then further investigations would be completed. Out of all the areas, she was more interested in temperature spikes/fevers and when they occurred. If I had another bout of fever, she was willing to admit me on to one of their wards for observations.

My only concern is...if the tests are inconclusive, is rheumatology just going to let me go???

Time will tell.

[Hidden in reply to Tc3844]

I am glad to hear that the Dr you saw was very thorough.

The problem is PAN is very rare and there isn't a consensus as far as diagnostic criteria although most Dr's would want an angiogram or biopsy that showed PAN.

The problem is that Dr's need to be very sure before giving a diagnosis of Vasculitis as the immunosuppressive treatment carries risk. I don't know if you will find this useful

bestpractice.bmj.com/best-p...

As for where do you go from here if they determine its not a Rheumatological problem then ask them to signpost you to the next specialist. I would also be aware, just because a Dr has seen PAN in the past doesn't mean that they are experienced in it. You may need to attend one of the large multi disciplinary Vasculitis clinics ( eg Birmingham, Cambridge or London ) to get a definitive answer or ask your Consultant if she would be willing to ask them for advice.

[Prudencepig in reply to Tc3844]

Hi

I also had a rare diseases specialist who tested me for all sorts as I had just returned a month earlier from Korea. Brought no joy for him. I also suffered with fevers and rashes on my feet which at their worst looked like I had red socks on.Hope you get sorted soon

[Tc3844 in reply to Prudencepig]

Interesting....About 3 weeks ago, I started to get very very red gums with a white edge. It isn't painful but it does bleed when brushing my teeth.

I went to the dentist this afternoon to see what it might be. She said, "It looks like lichen planus." I am lost as to where I am going with any potential diagnosis. Did you have any gum involvement?

[Tc3844]

Interesting....About 3 weeks ago, I started to get very very red gums with a white edge. It isn't painful but it does bleed when brushing my teeth.

I went to the dentist this afternoon to see what it might be. She said, "It looks like lichen planus." I am lost as to where I am going with any potential diagnosis. Has anybody had this or similar symptoms with PAN, SLE or any other disease?????

[Prudencepig]

I did have trouble with my gums and needed a lump removed and a couple of stitches but enjoyed the laughing gas lol

[Kentish_Man]

Hi Tony,

I also have PAN. Up until I read your post I only know of one other person who has got it in the UK. I am treated by Professor David D'Cruz who is quite an expert on Vasculitis and made my original diagnosis.

My pain can strike me anywhere at anytime and like you I get it in the testicles, my eyes and head. When it strikes in the head I have noticed that that area of the head seems hotter then the rest. The head and eye pain is if someone has put a red hot needle in there.

Finding a good professional who can treat you is key. I wish you good luck. I have had it now for 11 years, 9 years under the correct diagnosis.

Regards,

Peter

[Tc3844 in reply to Kentish_Man]

Peter,

Thanks for your message. I have not been diagnosed yet but do my symptoms sound familiar to what you have suffered?

[Kentish_Man]

Hi Tony,

I am afraid to say that yes they do. Let me know when they finally diagnose you.

Having an ultrasound of your testicles to see if you have PAN is a new one on me.

As I put in my original message it took two years for me to get a correct diagnosis. I was being treated privately by a "specialist" every fortnight then after a year every three weeks. The first thing he would do when I got there was ask how I was, while getting out a sheet of paper to write his invoice !! After between 18 months to two years, I was getting worse not better. So I asked for a second opinion. He did not like this (probably as I was a cash cow for him) and he sent me to the London Bridge Hospital. When I got to London Bridge I had blood and urine tests then I was sent to see Professor D'Cruz. Within 5 minutes he told me what I had. He also confirmed that the medication that I had been put on was doing me more harm then good. As well as saying the way that I was then, I had about 18 months left to live.

Believe it or not I left the hospital with a big smile on my face as someone had finally diagnosed me correctly. I was taken off those Meds and had to wait while they came out of my system. Then I had three months of Chemo. That did not work, so I had a further three months at double the dose. That did not work either, I am now on a regime of 22,500 pills a year (not just for PAN) and my condition is pretty stable. I have glares every now and then, but life expectancy is far, far better.

Regards,

Peter

[Tc3844 in reply to Kentish_Man]

Peter,

Drop me an e-mail with your contact details on. I think it would be great to talk to you further:

[e-mail]

I have had renal/stomach angiogram on Thursday. I am waiting for the results as well as appointments for nerve tests and blood tests with a rare disease specialist.

[Tc3844]

Things seem to be calmer than they have been in a while. However, it seems as if whatever it might be is creeping up my leg muscles gradually towards my groin. My rheumatologist has not really wanted to look at my leg issues I.e Burning in legs, fatigue, cold sweat when doing exercise. As I did not have any physical purpura on my legs, she completed the reflex tests and moved on.

My MRI (not angiogram) on my head was normal but I am still getting headaches. I am wondering if an angiogram should have been completed.

I am considering if I am getting the best service considering my symptoms. London Bridge is a private hospital but seems geared up for Vascular disease. I might contact them.