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
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.
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.