Jump to content
Sclero Forums
MAT54

Hello - a few questions I need answers for and some support

Recommended Posts

Hi. This is the 1st post here from me. I will add it to the UK forum as I'm based in Scotland.

I'm posting mainly to ask about hard skin on fingertips with pitting and lines - a new development for me.

I have not yet been diagnosed with Scleroderma but am to have a nailfold capilliary test in 7 weeks time. So far my main diagnosis is primary Sjögren's - diagnosed by lip biopsy last year which was 100% positive. Previously I was diagnosed with seronegative RA and treated quite aggressively for this disease six years ago. I am also Hypothyroid and suffer from Hypertension.

Raynauds only started for me five years ago and I'm 54 now. It did affect my hands by turning my fingers white a few times - I was on Methotrexate and Plaquenil at the time. After this I just felt the cold a lot and my extremities would turn patchy red and white - no blues or purples ever. Mostly my Raynauds has only affected my toes and soles during the winter months.

My journey has been long to get to where I am now - and I've moved around a lot which hasn't helped. But last year, once off all drugs and steroids, my ANA swung from equivocal to 1:320 with a nucleolar pattern. My ENA panel including Scl70 were all negative. I have very high sed rate and raised CRP - presumed now to relate to the hyperviscocity of Sjögren's. Because I was under neurology already for presumed small fibre neuropathy - the rheumatologist suggested I might have Sjögren's - hence the very positive lip biopsy which confirmed.

Meanwhile Raynauds somehow dropped off the list and my rheumatologist retired. I was started, somewhat reluctantly because of the increase Lymphoma risk with Sjögren's, on Cellcept/ MMF. This was 3 months ago now.

Since then the Raynauds attacks have increased in the way I have described - just white and red. My finger pads have been increasingly wrinkly and prune like and now my finger tips have turned shiny, hard and pitted and my general practitioner is a bit baffled - he can see and touch these changes for himself. He prescribed Betnovate - which I already use occasionally for the Pompholyx eczema on my fingers. He did not think this new symptom corresponded with Raynauds/ Scleroderma but nor did he think it Pompholyx or fungal related.

Meanwhile I am finding this sensation very uncomfortable for typing (tapping sounds and prickling sensation as I type). I can't get under my nails because of the hard skin and pitting. Does this ring any bells? Should I continue using the betnovate as instructed or is this a waste of time - it hasn't helped yet at all?

Share this post


Link to post
Share on other sites

PS I forgot to add that I will be having a nailfold capiliary test in 7 weeks during a rheumatology consultation. I do not yet have any organ involvement but have GERD and gastritis with dry cough and swallowing problems, telangiecstasias on hands and face and Raynauds affecting my hands and feet as well as hypertension plus severe tinnitus.

Share this post


Link to post
Share on other sites

Hi Mat54,

 

Welcome to these forums!

 

I'm sorry to hear that you have been experiencing unpleasant Raynaud's and Sjogren's symptoms and I've included a link to our medical pages on both diseases to give you some more information.

 

Unfortunately, scleroderma is a notoriously difficult disease to diagnose, as it presents itself in many different ways and can also be an overlap with other diseases, such as Sjogren's and hypothyroid problems. The symptoms you describe can relate to other health conditions as well as scleroderma and blood tests for the disease are not always conclusive.

 

I understand from your other posts that you're awaiting an appointment with your rheumatologist. Please note I have no medical training, but would suggest that perhaps you contact your rheumatologist/doctor regarding the continuation of the betnovate treatment, pending the result of your nailfold capillaroscopy, when it's possible that the capillaroscopy may help towards a diagnosis.

 

We do recommend that our members, if possible, consult a listed scleroderma expert, as this complex disease is not straight forward and does require specialist knowledge and expertise in order to deal with all it's little idiosyncrasies.

 

KInd regards,


Jo Frowde

ISN Board Member

ISN Secretary of the Board

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

International Scleroderma Network (ISN)

Share this post


Link to post
Share on other sites

Thanks very much. I'm seeing a specialist in vascular medicine who conducted nailfold capilliary tests in UK's second biggest Scleroderma centre in Dundee, Scotland. I did some research this morning and phoned the connective tissue disease clinic where I'm to be seen. The secretary made them aware of what is happening to my hands and the doctor said that this is why I'm now on their clinic list but no room to be seen sooner. And anyway he pointed out that I'm already on Mycophenolate, the main treatment, so nothing more they could do if it is Scleroderma.

 

Please can you tell me if eczema/ Pompholyx is quite common alongside the skin manifestations of Scleroderma?

 

Thanks for your advice and sorry to be a nuisance with too many posts at once. For some reason my iPad isn't coping well with the software so I'm unable to read the text I'm writing or edit it?

 

Finally - should I be getting email alerts for your replies because I've not had any yet?

 

Kind regards,

Mat54

Share this post


Link to post
Share on other sites

The key here with your positive ANA results and symptoms is to get detailed antibody testing.  There are 8 antibodies for scleroderma that can readily be tested for.  Here is a list from the Antibodies section of the "Scleroderma FAQ" on my Scleroderma Education Project website (sclerodermainfo.org): http://sclerodermainfo.org/faq/scleroderma-antibodies/.

 

The three most common antibodies in systemic scleroderma are Scl-70, centromere, and RNA Polymerase III.  With a standard ENA panel you were probably only tested for Scl-70 antibodies but some do include centromere, depending on the testing method.  If those are negative, then go on the other five antibodies listed in the antibody table referenced above.


Ed Harris

Scleroderma Education Project

A member of the ISN's Scleroderma Webmasters Association (SWA)

(Only SWA members may post links to their own site in forums posts)

Share this post


Link to post
Share on other sites

Hey there MAT54

 

Welcome and you can't post too much but you can ask questions we can't answer, don't worry, though, when that happens we ratchet up the support.

 

I have found the approach to bloodwork very different here in the UK to that in the US and ultimately it's not the most important issue. I'm a patient at the Royal Free, under Prof Denton's team and they're the experts for sure but my ANA SCL-70 status has never been discussed and they have been the only hospital to test my antibodies. I only know what it is because I have asked. I was diagnosed in 2007, my ANA only became positive around 2014/15 but my SCL-70 has always been negative. Always. I was diagnosed August 2007 with scleroderma and when I saw Prof Denton that September my diagnosis was confirmed as atypical diffuse cutaneous systemic sclerosis (diffuse systemic scleroderma) with my nailfold capillaroscopy showing that I also had Raynaud's.

 

I know that antibodies can point you in the direction of potential symptoms and can clarify what type of connective tissue disease you may have but unless you proactively treat a symptom (do they even do that) there's not much more that you can currently do. You are already suppressing the immune system with the mycophenolate and as symptoms arise, they'll be treated too.

 

I do understand why people want to know about antibodies just bear in mind that the information doesn't always hold any keys. I was told, by the Royal Free that my negative ANA and SCL-70 meant that I was unlikely to ever develop internal organ involvement. Imagine my shock at being in serious heart failure 3 years later having developed myocardial fibrosis!

 

I hope this is of help and interest and take care.


Amanda Thorpe

ISN Sclero Forums Senior Support Specialist

ISN Video Presentations Manager

ISN Blogger

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Email Support Specialist

International Scleroderma Network (ISN)

Share this post


Link to post
Share on other sites

Amanda, there are three different antibodies associated with diffuse systemic scleroderma: Scl-70, RNA Polymerase III, and U3-RNP.  U3-RNP is very rare but the incidence of RNA Poly III and Scl-70 is about the same, around 20% of of the total SSc population.  RNA Polymerase III has a higher risk of early kidney involvement than Scl-70 and as a result prednisone can be very dangerous with this antibody as it can trigger scleroderma renal crisis (the risk is dose dependent).  It is also risky with Scl-70 antibodies.  I am not sure if the research has been done with U3-RNP since this is a very rare diffuse antibody.

 

Knowing your antibody is very important because of specific risks like this.

 

Also, if you ANY variant of systemic scleroderma, you have internal organ involvement.  I wrote this paper last year and it was reviewed and edited by one of the top scleroderma researchers in the US: http://sclerodermainfo.org/yes-you-do-have-internal-organ-involvement-but/.  As the paper notes, the internal organ involvement may not lead to any functional impairment, but it is definitely there.


Ed Harris

Scleroderma Education Project

A member of the ISN's Scleroderma Webmasters Association (SWA)

(Only SWA members may post links to their own site in forums posts)

Share this post


Link to post
Share on other sites

Whereas I am by no means denigrating the importance of antibodies in the diagnosis of scleroderma, I feel that some of our members do become overly worried and concerned about positive antibody blood test results, which is why we emphasise that they are only a part of the picture and not the whole story.

 

Quote

I do understand why people want to know about antibodies just bear in mind that the information doesn't always hold any keys. I was told, by the Royal Free that my negative ANA and SCL-70 meant that I was unlikely to ever develop internal organ involvement. Imagine my shock at being in serious heart failure 3 years later having developed myocardial fibrosis!

 

I have the Anti-PM/Scl antibody, but thankfully no signs (so far!) of polymyositis (and I'm hoping that pleasure will be indefinitely postponed. :wink:)


Jo Frowde

ISN Board Member

ISN Secretary of the Board

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

International Scleroderma Network (ISN)

Share this post


Link to post
Share on other sites

Joelf, that is a good point to emphasize.  There are many people who have positive ANA and antibodies that never develop functional disease.  Also, there are the 5% to 10% of people who are ANA negative by IFA who clearly meet diagnostic criteria for systemic sclerosis.

 

I do, however, always suggest trying to figure out what specific antibody you have if possible as it gives you important information on risk profile and issues like whether or not certain medications are safe to use.


Ed Harris

Scleroderma Education Project

A member of the ISN's Scleroderma Webmasters Association (SWA)

(Only SWA members may post links to their own site in forums posts)

Share this post


Link to post
Share on other sites

Join the conversation

You can post now and register later. If you have an account, sign in now to post with your account.
Note: Your post will require moderator approval before it will be visible.

Guest
Reply to this topic...

×   Pasted as rich text.   Paste as plain text instead

  Only 75 emoji are allowed.

×   Your link has been automatically embedded.   Display as a link instead

×   Your previous content has been restored.   Clear editor

×   You cannot paste images directly. Upload or insert images from URL.


×
×
  • Create New...