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CREST Syndrome

QUESTION:

Thank you so much for your time. It is so greatly appreciated and respected.
I am newly diagnosed by my local Rheumatologist with Limited Scleroderma/Crest Syndrome - although I have no skin thickening/hardening at this time. I have had a mild Raynauds for 10 years - although not bad.
I was having extreme unrelenting fatigue and could not bounce back after I did
the breast cancer 3day walk which is what brough me to the PCP, who in turn ran
a plethora of labs including Valley Fever (neg) and ANA (positive) - the
positive ANA led me to the Rheumy who did the full panel which revealed elevated
Centromere Antibodies - all other ANA panel was negative, CMP normal, Vit D low at 10 and B12 was low. I also always seem to have a low grade fever of 99.?

I was put through the ringer with testing and was found to have; bladder wall
thickening, mild hepatosplenomegaly, delayed gastric emptying, erosive
gastropathy, and esophageal dysmotility (along with non-related benign lung
nodule & thyroid nodule)
I was placed on Vit D supplements and weekly B12 injections. GI doc did labs to
rule out enzyme deficiency and wants to schedule Colo.
The Rheumy had started me on Plaquenil due to joint pain, fatigue, shoulder and
lower body weakness (CPK is normal) interrmittant finger tip pain & hand pain. I
also have bouts of swelling in my hands and feet in the morning that dissipates,
and on two occasions had my index finger swell so badly it was sausaged and I
could not bend it. One of my toes did this as well. (No psoriasis) - My index
finger is now kinda shot and is in a constant state of very mild swelling and
feels like it just doesnt "work" any more. On the same hand my wrist feels that
way too with an "Odd" dip or nodule at the joint.

I went for a second opinion and that Rheumy said I did not have Limited
Scleroderma because I had no skin involvement and wanted to just call it "Some
kind of autoimmune disorder" and advised no meds. no need for immunosuppressants and to come back when things got worse.
I went back to my first MD after the finger swelling episodes and he advised a
switch to Methotrextae as he felt that was an anvancement of symptoms.

At this point I am so confused about what to do and what I really even have. All
of my Labs from Lab Corp come back positive ANA and high Cen B at 5.7 or higher
(approx 6-7 times) But when I went for that second opinion his lab came back ANA negative but with the continued elevated Cen B antibodies.

I guess my question is... should I use the Metho as a prophylaxis to help halt
the degree and severity of disease progression regardless of what we decide to
call it.
I also have dry mouth and dry eye issues but the Sjo labs come back WNL. I have
bouts where the sx are more severe, but when its bad - Its bad to the bone!!
My other question I just cannot seem to find an answer for no matter how much
research is: What does a flare feel like?
I have bouts of no energy, my muscles feel trembly after even mild use almost
shaky as if I worked out to long/hard, my dryness is worse, my fatigue is almost
tangible, my arms feel so heavy, I feel kinda flu-like, I get kind of weepy (I
think since I am use to being so active and when you feel that tired you almost
feel it in your soul)
And lastly (I am so sorry) I am wondering if the new pain and loss of grip I
feel in my hands and at my thumbs is the beginning of hand changes. It is hard
to articulate what I feel exactly, but intermittant loss of grip, wrist
weakness, cramping, a stretching-ish feeling and pain at the bones down my
fingers - along with my usual joint ache
I do not need narcotic pain meds yet, but there are times I need to take a hot
bath to ease the joint issues and at times with the dull ache becomes real pain
that I WISH I had narcotic pain meds (too afraid to take it) - Did have EMG and
NCV with no CTS and normal muscle function but I did have abnormal nerve
conduction study of my legs twice. The Neuro thinks neuropathy.
The only other weird tid bit was an elevated Alpha-2 globulin but it was mild
elevation being called MGUS with yearly monitor.
So - If I havent ranted or bored you too much, what are your thoughts with
regard to diagnosis, prophylaxis medication use, Methotrexate, Flare Ups and
begining hand change symptoms for the Big S.

ANSWER:

This information fits with Limited Scleroderma, also known as CREST syndrome. A positive ANA in a centromere pattern is highly unusually to have, means something, and almost always associated with CREST. There is a subset of patients with Sjogrens that can have a Centromere antibody and CREST like symptoms. People do not always fit into a "classic" disease class.

If you live near one of the 3 sites in the US for the SICCA Registry (Philadelphia, Johns Hopkins, UCSF-San Francisco), you may find being evaluated there useful. If you elected to have the minor salivary gland biopsy and your focus score (clusters of lymphocytes) were high-you may be more of a Sjogrens first and CREST second. It helps in understanding what is happening to you and what may happen-but remember autoimmunity can be very variable and can change over time.

The GI motility problem goes along with CREST or Sjs. Neuropathy, and MUGUS can be seen with both. I assume you had PFTs (Pulmonary Function Test) with DLCO (diffusing capacity) to rule out interstial lung disease-which is more commonly symptomatic in CREST than Sjogrens. CREST also is associated with pulmonary hypertension, and less so with Sjs.

Wonder if you have had thyroid peroxidase antibodies (tenosynovitis is common with autoimmune thyroid), or parietal/gastric antibodies (autoimmune gastropathy) with the low B12.

There is a lot going on to do nothing...Plaquenil is first line, and some doctors will be comfortable increasing it to 600 mg/d depending on your weight, others will not. If Plaquenil is not enough, a DMARD (disease modifying antirheumatic agent) is used. This can include Methotrexate, CellCept, or Imuran. It seems reasonable to be more aggressive with therapy to see if  your symptoms improve. You can decide with your doctor later if you need to keep taking them or can taper off.

There a few centers around the country that have Scleroderma/Limited Scleroderma clinics-you may consider being seen in one of them. There are clinical trials using some novel agents that look promising-like Gleevac. Stanford (Lori Chung MD), U of Maryland-Johns Hopkins (Fred Whinery MD) are 2.

Dr.C.

Printed from: http://www.sjogrensforum.com/ask-the-doctor/crest-syndrome/ .
© Your Name Here 2017.

9 Comments   »

  • Jilly Reynolds says:

    All that I have read here seems so completely familiar, I am diagnosed with Sjogrens recently and being tested for Crest for which I have positive blood test. It is so difficult to communicate with those around to whom one appears to be completely normal. It is a relief to know that others are having the same experience. What I would like to know is the prognosis or outlook for ss/crest patients. Any information gratefully received.

  • Deana Marie Gallatin says:

    please join the c.r.e.s.t. syndrome group on facebook. we share a lot of tips and therapies and quacks, gennerally we give each other support when no one really understands...

  • Maggie Alberts says:

    I have the same symptoms as the first comments, except I have had skin problems for years and was actually diagnosed with Mixed Connective Tissue Disease which includes Lupus, CREST and Vasculitis. I too have difficulty with Rheumatologists. I guess they only want to help people with a full blown disease! I know this is what I have and I have actually been told I don't by a rheumy who had no tests and didn't even examine me! Now my PCP says definitely with a ANA of 1:320 with a centromere pattern. I still don't have any medication and this has been going crazy on my body for years. So many pains and symptoms!

  • Michelle Virgilio says:

    I have Crest and its horrible. I had all the symptoms mentioned above, except the doctors in Massachusetts just kept taking out my organs. First my gallbladder, then appendix, the my large intestines/colon. This was done a lot in Boston where doctors are supposed to be great.
    Anyways we moved to Florida within one month I needed a full hysterectomy. Every time they took an organ they said they were in bad condition.
    I finally got into the Cleveland Clinic. After my right side would go numb. I actually feel broke my foot and didn't even know I did it. Then then numbness would go to burning.
    I still have trouble eating but I'm on 6 different meds and sometimes they help. But with my pancreas pain I live on pain killers, anxiety meds and depression meds.
    Get your self a good doctor before you end up like me.
    I would love to know how many people have full blown CREST like I do? Everything I go to the hospital it seems the doctors have not heard of crest or treated anybody with it.

  • Wonda Gail Romano says:

    I have had the crest syndrome now for 3 years as of May 12th 2012, alot has changed in my life, 33 throth sugerys , to get food out that will, not go down, or to be stratched. I have been on medtraxet for the hole time, id love to have more info on how to do more for my self,i had 3 sugerys in one, so it can help me eat, if it keeps up i will need an feeding tube, I said no, I had an double highall hunra, 2 big ones, then the had to fix the lower part of my asogaus to try to fix the ling of my asoagus was growing into my stomch, then last dec, 19th had other sugery, Had my galbadder removed, but still haven alot of promles, could some one help me to understand it all in more deal tell? Most of the time the drs dont know what Crest is, i explan it or tell them to look on line. thanks, an 3 year suval of CREST SYNDMorm, WONDA GAIL ROMANO,

  • nancy says:

    The initial writer's symptoms are close to mine--labs say Scleroderma but limited symptoms so far--mostly migrating joint pain, some nerve pain, numbness & tingling, occasional fatigue, dry eyes to the point of getting erosions( thought it was from my Lasik 10 yrs ago, optho didnt make the connection) and calcifications on finger joints (maybe otherplaces too but didnt xray everywhere). I had been on plaquinil and methotrexate (no help) and pain was getting worse and worse--but disappeared 3-4 years ago when I went gluten-free. It is slowly starting to come back, however, so I am thinking I was just delaying the inevitable. Centromere B and RF were only elevations. Is all of this really in my future?? Anything I can do to prolong the big onset?

  • drcarteron says:

    Everyone is unique. There is not a definite progression. Autoimmune disorders can fluctuate. Nutrition, healthy lifestyle, stress management, and avoiding "triggers" of flares can be an important part of maintaining your best health. More Research is focusing on fibrosing disorders like Scleroderma, CREST, and Pulmonary Fibrosis ...so with more understanding comes better management/treatment options.

    dr c

  • pj carter says:

    I have had crest for 18yrs or more. No dr has ever been helpful. Always a negative experience. My hands are so distorted now I can hardly write. Calcium deposited on all joints. Muscle weakness in legs getting worse. Flares and feeling flu like come and go. Difficulty with throat and swallowing and choking. Back pain getting worse and dryness of eyes and mouth why is there no help or real answers for this diseasei? no dr is well informed and sends me to the internet to do my own research research. I only take 2 aspirin a day. I will be 72 next month. I don't knownhow much worse it will get.

  • drcarteron says:

    CREST is a limited form of Scleroderma. The CREST subset does co-occur in Sjogrens.

    There are only a few centers that have expertise in CREST. Having a Consultation at one of them can provide a Resource and Guideline for Management that can be helpful to you and your health care team.

    One such Center:

    The Scleroderma Foundation is also a good Resource with information to share with your team.

    Focusing on what is "not worse" usually works better ... easier said than done...

    dr c

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