Complications with PACNS/ Cerebral Vasculitis

[MelBeck]

Was diagnosed with PACNS in 2015, biopsy proven, after several years of severe headaches, memory loss and fatigue, advised now in remission. Originally had Chemotherapy, steroid and Methotrexate, now only on the latter, but still experience symptoms. Would be interested to hear from anybody with Cerebral vasculitis who can advise and comment on the following.1, Dry, sore and watery eyes, very sensitive to sunlight.2, Having surgery involving a general anaesthetic whilst suffering from vasculitis. 3, Having dental treatment involving extraction /implants having been on Alendronic acid for a period of time. Any advice or comments would be very much appreciated. MelBeck.

[vivdunstan Volunteer]

I've lived with cerebral vasculitis for 25 years. My story is online at

vasculitis.org.uk/living-wi...

To answer your specific queries:

1. I was very photophobic, but not dry, sensitive eyes. I would just get a headache from too strong a light, including fluorescent lighting. This was especially bad before I was treated. It improved with immunosuppression treatment. It can be a sign of a disease under treated. But I'd suspect dry eyes is less likely to be related to brain vasculitis directly.

2. If you are still on immunosuppressive treatment any surgery is more dangerous, with a higher risk of infection. It is vital that your surgical team know about your immunosuppressive status. It is even better if they can liaise with your vasculitis team. Often non-essential surgery is postponed until someone is not immunosuppressed, or on a lower dose. Cerebral vasculitis would not normally be an increased risk for surgery, but the treatment suppressing your immune system (i.e. Methotrexate now) is.

3. I've been on Alendronic Acid too for some years, after being diagnosed with full osteoporosis. It is a risk factor for any surgery involving the jaw. The danger is bone death afterwards, to do with the blood supply being reduced to the bone. There is also an increased risk of infection. It is vital that your dentist knows about your Alendronic Acid. Many local dentists won't do this surgery for you, or tooth implants - anything involving the jaw. Many prefer to refer you to the local dental hospital to be seen there, under more hygienic conditions. Some may also like you to have hyperbaric chamber treatment afterwards, to try to reduce the risk of bone complications. But you need to talk to your dental team.

Good luck!

[MelBeck in reply to vivdunstan]

Hi Viv, thank you for your reply, we have previously spoken through this forum, reference the eye issue and I have looked at your story online. I'm sure your condition is worse than mine,but we are similar in the fact, I think initially it was thought that MS might be the problem, in my case, went sometime undiagnosed, but when PACNS was found to be the case put on cyclophosphamide straight away,where as I don't think you were. With regard to the dry eyes, I attend the hospital periodically, but it has never been confirmed that vascuitis is the reason. I wear good quality sunglasses and use Hylo-forte drops. I also use Vita pos ointment when the eyes are particularly very sore and dry. With regard to surgery and a possible general anaesthetic, I note what you say about methotrexate, but I'm under the impression, they do not always stop it, prior to having a procedure. Again like you, I have been on Alendronic acid, however, I came off it myself about 18 months ag, having been on it for 2 to 3 years. Reason being a follow-up DEXA Scan showed since I stopped taking it ,my bone density had improved and also my blood sugars returned to normal, even though I had developed diabetes, prednisolone injuced ,whilst on it. Just shows,extremely important drug to help save life, but if taken that long ,can cause many issues. Of course there still remains the issue of dental treatment as the Alendronic acid apparently stays in the body for up to 15 years and we know the potential complications with osteonecrosis. I have found difficulty with dentists agreeing to do invasive treatment and Ihave subsequently written to my neurologist requesting advice. On a final note, I think we agree, that PACNS, Cerebral vasculitis remains potentially serious and complex with still no cure. Take care MelBeck.

[AmyS1]

You should see an ophthalmologist with experience in Vasculitis.

Hopefully you are being seen at a specialist centre. Your consultant who oversees your vasculitis MUST liaise with the consultant who is planning your surgery and general anaesthetic.

Dental treatment ensure that your consultant and dentist consult each other you may need to increase steroids etc to cover the dental procedures etc.

[AmyS1 in reply to AmyS1]

Nb Dry eye is a term used when the Dr is not sure of what is going.on !!!!!

[MelBeck in reply to AmyS1]

Thank you so much for your reply. With regard to the dry eye situation, I go to the local hospital periodically and see the ophthalmologists. Not sure they are experienced in Vasculitis and I've never been told it's related. I was put on a trial survey with different eye drops in addition to the ones I already take Hylo-forte, unfortunately I did not react well to them and stopped it. I try and keep out of direct sunlight and fluorescent lighting and fortunately my eyesight is good. I still though have to contend with the uncomfortable,dry,sore and watery eyes. Interesting point you make ,the term dry eyes ,Dr; not sure what is going on !!!. Could well be right. I have also experienced problems associated with Alendronic acid and found it difficult to find dentists prepared to undertake invasive treatment. I have therefore written to my neurologist for advice. With regard to having surgery involving a general anaesthetic,obviously you have a pre; operative assessment ,but I'm not sure you are taken off medication such as methotrexate,although clearly you would advise of your condition and other medications taken. It's clear that Vasculitis is a rare and potentially serious condition. The particular type I have PACNS even more so with approximately only one in a million people having it. I do not know how much the Medical Profession actually know about it, or how much doctors and consultants liaise with each other. Everybody always seems so busy and there is very often inevitable changes in personnel.

I'm not being disrespectful but clearly there is a lack of knowledge with Vasculitis generally. I lucky 2 have got a particularly good neurologist and are very grateful to have him, he has helped me through it and although I still experience a number of issues, I am in remission. Take care and thank you for your imput. MelBeck.

[Christophene47 in reply to MelBeck]

Hi Mel,

Usually with pending surgery, you may be advised to slightly increase your immunosuppressive medication to avoid an inflammatory reaction from the stress to your system from the surgery. I had to have a lip biopsy done, and the oral surgeon asked me to take my highest Prednisone dose prescribed the night before and the morning of b/c any surgical procedure, even minor, is a stress on the body. You are lucky to have a good neurosurgeon you can ask. But how right you are, very few doctors know anything about vasculitic diseases. I had no problem from the biopsy, except it was a squamous cell carcinoma, removed later by a Moh's surgeon without any disfigurement.

Good luck.

[MelBeck in reply to Christophene47]

Hi Chris and thank you so much for your replies, details of which I note. With regard to proposed surgery, I had a recent pre-op assessment, but surgery is being held back at present. (Separate issue with arthritic shoulder). However, although they make a note of the medications you are currently taking, there was no requirement to stop Methotrexate for example ,which I continue to take. Certainly no mention was made to increase other meds; such as steroids, which I stopped about 18 months ago. The dry eye situation is an interesting one. I go to the local hospital eye department periodically and had a blood test for Sjogren's Syndrome ,but it proved negative. I have heard of the other test you mention with the small strip of paper under the eyelid, but I've never actually had it myself. Interesting point you make over the 5% saline hypertonicity drop, never heard of it, but will research it with the local pharmacist and online, particularly as you say it has helped your eyes. I note your comments about taking medications such as steroids etc intravenously, rather than orally to lessen the risk of increased weight gain, In all honesty, I really do not know the answer, I took prednisolone orally and no doubt they are a lifesaver, but was very happy to eventually come off them, due to the associated side effects and the well documented osteoporosis and diabeetus related aspect. Thank you again for your imput and I wish you all the very best. MelBeck.

[Christophene47 in reply to AmyS1]

I have dealt with dry eyes my whole adult life; I am also light and noise sensitive. But what I wanted to say is that there is a simple test ophthalmologists do to objectively test for dry eye by inserting a type of strip under the lids; ; and after removal, measure the degree of dryness or moisture . It's not painful. I had it done a number of times before eye surgeries, most recently cataract surgery; I also have very dry skin and hair at times and am quite hypothyroid. Dryness is often is a symptom of hypothyroidism. I use a 5% saline hypertonicity drop for moisture because it does improve my vision. It stings like the devil for a moment. I can only find it in my small town pharmacy, not anywhere else, although I imagine it can be found online. I suspect they do the test more often because there now there is a prescription pill drug marketed in the US for dry eye. So there is always a profit motive.

I don't have it, but it's often a symptom of Sjorgens also. Of course, dry air, allergies, heating and AC , irritants, can cause it in some people.

So I'm not sure it's a catch all; there are some real conditions associated with dry eye. Just for information.

[KellyInTexas in reply to Christophene47]

It’s called a schirmers test, this paper test. It’s a good way to test for Sjögren’s syndrome. ( auto immune diseases come in gangs, as we know...)

I have to wonder if you some of you ladies are experiencing symptoms of Sjögren’s syndrome.

[Christophene47 in reply to KellyInTexas]

Thanks for the name; I knew it once, but did not remember.

[whitleybayfc]

Hi ive Giant cell arteritis vasculitis ,large vessel .Im on 20mg methotrexate self inject once weekly.I was on prednisilone for 3 years ,off now thank god,i had chemotherapy cyclophosphomide as well. I am also type 1 diabetic ,have Addison s disease ,and antiphilosphid syndrome or Hughes syndrome.I suffer from fatigue and stiffness still ,im on a lot of other meds. Its now 5 years im 56 since diagnosis of vasculitis,im not doing too badly thank god.I was ill health retired at 51 on full ill health pension,so wont starve.I realize im lucky to have pension.Many arent .I take anti sickness tablets after metho injection as feel bit sickly for a day or 2.

[Christophene47 in reply to whitleybayfc]

I just read a scholarly article that injections of Prednisone and Methotrexate , rather than oral tablets, seem to be more effective in preventing or lessening weight gain from steroids. Do you think that is true? Sorry for all your other problems, but happy you are financially OK, and not doing too badly. Curious about injection. and weight gain?.

[whitleybayfc in reply to Christophene47]

I have lost weight since coming of pred tablets ,my weight is stable at present on methotrexate injections.

[Christophene47 in reply to whitleybayfc]

I should clarify; the article I read pertains only to Methylprednisone , not Methotrexate.

[Christophene47 in reply to whitleybayfc]

And it is from the British Journal of Rheumatology , 1997. I am sure that would not be read by GPs, and certainly not in the United States where I am. It is however on Medline.

[MelBeck in reply to whitleybayfc]

Thank you so much for your reply details of which I note. Just read an interesting blog on here, saying that if you inject steroid intravenously are you less likely to increase weight than by taking them orally. I'm not sure or qualified to say that, l did take them myself orally for 2/3 years and not intravenously and my weight slowly returned to ormal after slowly coming off, so I'm not sure that it makes any difference how you take it. I must say though, it was a real relief when I did eventually stop taking it, bearing in mind the side effects and complications they can cause. My Chemotherapy was Cyclophosphamide infusions and occasionally, I did feel signs of sickness, but never really a problem. I am still on methotrexate orally and also take a host of other drugs, but not directly linked to vasculitis. I am 72 years old, have experienced severe fatigue and exhaustion, since being diagnosed over 4 years ago and previously before that. Unfortunately I'm not conversant with your other conditions and therefore not able to comment . Thank you again for your input and I hope things go well for you going forward. MelBeck.

[AmyS1]

Call the Vasculitis uk website and check out the closest specialist centre for Vasculitis. Not many neurologists are Vasculitis specialists.

[MelBeck in reply to AmyS1]

Yes, you are correct and I understand that, in fact the nearest one to me would probably be in London and I would think it would be very difficult and delayed in getting an appointment. I do have some imput from the local Rheumatology department at the main hospital, but only see them about every 9 months. They initially dealt with my Chemotherapy ,Very well ,I must say, but again they have a number of personnel changes and other than a general knowledge of vasculitis, they are not specialists. I do occasionally liaise with John Mills vasculitis UK, who does advise me. Would not be easy and cannot see me changing to a specialist vasculitis centre, particularly as I'm thought to be in remission and it would all have to be approved and dealt with by my existing neurologist. Not being negative, but to be realistic, the way things are and bearing in mind the issues referred to in my earlier reply, I just cannot see that happening unless I took a serious turn for the worse. When I first had a stroke in 2015 and admitted into Southampton neurological I was nearly transferred to Addenbrookes but that was different. For one reason or the other is just so difficult now to see anybody. Take care Mel Beck.