Jump to content
This site uses cookies. Continued use is acceptance of our Terms of Use and Privacy Policy. More Info... ×
  • Welcome to Celiac.com!

    You have found your celiac tribe! Join us and ask questions in our forum, share your story, and connect with others.




  • Celiac.com Sponsor (A1):



    Celiac.com Sponsor (A1-M):


  • Get Celiac.com Updates:
    Support Our Content
    eNewsletter
    Donate
  • Jefferson Adams
    Jefferson Adams

    Active Scleroderma Common in Celiac Disease

    Reviewed and edited by a celiac disease expert.
    Active Scleroderma Common in Celiac Disease - New study on scleroderma and celiac disease.
    Caption: New study on scleroderma and celiac disease.

    Celiac.com 10/01/2010 - Scleroderma is a chronic, systemic autoimmune disease characterized by tissue fibrosis, or hardening, vascular changes, and autoantibodies.

    There are two forms of scleroderma. The first is called limited systemic sclerosis/scleroderma cutaneous, and manifests mainly affect the hands, arms and face. This form of scleroderma was earlier known as CREST syndrome, because symptoms included Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasias. This form of scleroderma triggers pulmonary arterial hypertension in up to one third of patients, which is the most serious problem.

    Celiac.com Sponsor (A12):
    The second, less common form of scleroderma is called diffuse systemic sclerosis/scleroderma. This form progresses quickly and affects large areas of skin and one or more internal organs, generally the kidneys, esophagus, heart and lungs.

    A recent letter by doctors R. Thonhofer M. Trummer, and C. Siegel noted that capillaroscopy shows an active pattern of scleroderma in celiac disease. They are affiliated with the Department of Internal Medicine at the State Hospital Muerzzuschlag, and with the Department of Rheumatology at the Medical University of Graz in Austria.

    Their letter notes that previous studies have shown celiac disease to be associated with higher rates of Raynaud’s phenomenon, sclerodactyly, arthritis, polymyositis, pericarditis, vasculitis, and scleroderma.

    The doctors describe the case of a 41-year-old, non-smoking woman, who presented with Raynaud's phenomenon and progressive weight loss. For nearly twenty years, the woman had suffered symptoms of Raynaud's phenomenon, with classical tricolore phenomenon and symmetrical bilateral involvement of hands, with lesser effect on her feet. The woman's weight loss had persisted for two years, accompanied by unspecific abdominal symptoms including meteorism and episodic diarrhea. The attacks of Raynaud's phenomenon seemed to be triggered by exposure to cold or by emotional stress.

    The doctors ruled out digital ulcers, vibration injury, and ingestion of toxic agents as possible causes.  A color Doppler ultrasound examination of the arteries of the upper and lower extremities showed nothing remarkable. Radiographs of the hands, feet, clavicles, cervical, and lumbar spine were also not remarkable.

    Nailfold capillaroscopy of digits two to five bilaterally showed capillary loss in some areas, mild, focally moderate to severe disorganization of the vascular architecture, frequent giant capillaries, and hemorrhages adjacent to enlarged, nearly normal, and giant capillaries.

    The patient showed negative test results for anti-nuclear antibodies, complement factors, anti-DNA antibodies, anti-neutrophil cytoplasmic antibodies, anti-cardiolipin antibodies,
    cryoglobulinaemia, and rheumatoid factor. Erythrocyte sedimentation rate and C-reactive protein were in the normal range.

    The patient showed positive screens for anti-endomysial antibodies and anti-tissue transglutaminase antibodies. Endoscopy and several biopsies of the large bowel and terminal ileum showed nothing remarkable. Gastroduodenoscopy showed normal mucosa in the upper gastrointestinal tract.

    Looking at biopsy results from different parts of the duodenum, the team found an increase in intraepithelial lymphocytes, with about 60 to 70 per 100 enterocytes, along with shortening of the epithelial cells, and a reduction of globlet cells.

    As a result of antibody testing, anamnesis, and histology, the team was able to make a diagnosis of celiac disease. After the patient began treatment with a gluten-free diet, the abdominal symptoms and weight loss subsided, and the Raynaud's phenomenon attacks became less frequent.

    Clinical and laboratory evaluation over the last four years have not shown any connective tissue disease in the patient. The team used capillaroscopy every 6 months to show that the pattern of damage remained unchanged, and was not improved by the gluten-free diet within the first year.

    After 18 months, the vascular architecture showed some improvement, with no further bleeding or enlarged capillaries. Repeated examinations after that point showed similar, near-normal findings.

    There have been previous reports of several rheumatological disorders, including Raynaud's phenomenon in people with celiac disease, but this seems to be the first report of extensive
    microvascular damage, similar to capillary changes in scelroderma, documented by nailfold capillaroscopy in a patient with celiac disease.

    Because they excluded any underlying connective tissue disease or other secondary causes of Raynaud's phenomenon, the team's findings support a causal relationship between celiac disease and Raynaud's phenomenon, especially in regards to the described capillary pattern. Normalization of the patient's vascular architecture after a year and a half on a gluten-free diet further bolster their findings.

    The team admits the possibility of evolving scelroderma in the patient, but points out that the negative laboratory testing, organ screening, and inconspicuous clinical examination would seem to make that scenario highly unlikely.

    It will be interesting to see what doctors can learn through additional nailfold capillaroscopy investigations into the special capillary pattern in celiac disease patients.

    Source:


    User Feedback

    Recommended Comments

    Guest Hallie Davis

    Posted

    Very, very interesting! About 3 years ago I went to Dr. Fasano to investigate the possibility of celiac disease. All of the celiac antibodies, as well as the endoscopy biopsies were negative. But he found I have the DQ8 gene. Because I was having so many autoimmune symptoms I decided to go gluten-free despite not being technically celiac.

     

    About 4 weeks ago I was finally definitely diagnosed by one of the nation's top scleroderma specialists as having definite limited systemic scleroderma. I have mild nailfold capillary changes, extremely high anticentromere B antibodies, high sedimentation rate, high CRP, high Complement 4, high ANA (up to 2560), gastric reflux, slow swallowing action, a narrowing of the back of the throat which has caused severe obstructive sleep apnea, constipation alternating with diarrhea, hiatal hernia, loss of hair on my limbs, mild sensorineural hearing loss with ringing in the ears, foot Raynaud's, neuropathic pain, tendonitis, rotator cuff inflammation, swollen fingers, mottled pigmentation on my forearms, a stiffening of the heart muscle (left ventricular diastolic dysfunction, grade 2), telangiectasias, and other autoimmune things such as thyroiditis, and probable Sjogren's syndrome. Despite all these things, I still have no skin hardening, and ironically look perfectly healthy to the average observer.

     

    I do believe the DQ8 HLA type is at least partly responsible for this association between celiac disease and scleroderma

    Link to comment
    Share on other sites
    Guest Marget

    Posted

    Wow. Really interesting. I wish I could convince my friend with Reynaud's to get tested for celiac. Thank you.

    Link to comment
    Share on other sites
    Guest IAN GEARY

    Posted

    This is almost impossible to comprehend for the non-medical reader!!!

    Link to comment
    Share on other sites
    Guest norma brannan

    Posted

    I have RA, OA discoid lupus which is on my skin, Raynauad's, dermatitis herpitaformas (celiac of the skin), sjogrens, ect. I am affraid of what will show up next.

    I stated a gluten free diet a few months ago.

    Link to comment
    Share on other sites
    Guest Jefferson Adams

    Posted

    This is almost impossible to comprehend for the non-medical reader!!!

    Not all of our articles are for lay people. Some of these studies are quite technical, and largely intended for clinicians and medical professionals treating and diagnosing and advising celiac patients. I suppose if, like Helen, you had been diagnosed with scleroderma and had some basic reference points, this article might mean more to you.

    Link to comment
    Share on other sites
    Guest gwen vickery

    Posted

    excellent

    Link to comment
    Share on other sites
    Guest Paula

    This is one article, I need to see more data. My daughter has been diagnosed with celiac for over a year. What comes first, the celiac disease or the scleroderma?

    Link to comment
    Share on other sites
    Guest G Stark

    Posted

    I was diagnosed with celiac disease in 2006 and been on a strict gluten free diet ever since. This year, I have been just diagnosed with limited scleroderma and polymyositis overlap. I have interstitial lung disease, which was confirmed with CT and PFT tests. My CPK and aldolase levels are high, and my ANA came back with positive PM-Scl. Even though I have been on gluten free diet, I am still ill with other auto immune diseases. This article is interesting, but requires a lot more research. Going on a gluten free diet did not prevent my scleroderma from occurring. There must be something more to it.

    Link to comment
    Share on other sites
    SportySue

    I too have celiac disease. For me I learned that gluten-free foods were a trigger for continued celiac disease symptoms. Try just eating from the circum of your food market; meat, non-starch green well cooked veggies. No dairy including yogurt of any kind. I know it’s tough to do but you can do it! Sue D 

    Link to comment
    Share on other sites


    Create an account or sign in to comment

    You need to be a member in order to leave a comment

    Create an account

    Sign up for a new account in our community. It's easy!

    Register a new account

    Sign in

    Already have an account? Sign in here.

    Sign In Now

  • Get Celiac.com Updates:
    Support Celiac.com:
    Donate
  • About Me

    Jefferson Adams

    Jefferson Adams is Celiac.com's senior writer and Digital Content Director. He earned his B.A. and M.F.A. at Arizona State University. His articles, essays, poems, stories and book reviews have appeared in numerous magazines, journals, and websites, including North American Project, Antioch Review, Caliban, Mississippi Review, Slate, and more. He is the author of more than 2,500 articles on celiac disease. His university coursework includes studies in science, scientific methodology, biology, anatomy, physiology, medicine, logic, and advanced research. He previously devised health and medical content for Colgate, Dove, Pfizer, Sharecare, Walgreens, and more. Jefferson has spoken about celiac disease to the media, including an appearance on the KQED radio show Forum, and is the editor of numerous books, including "Cereal Killers" by Scott Adams and Ron Hoggan, Ed.D.

    >VIEW ALL ARTICLES BY JEFFERSON ADAMS

     


  • Celiac.com Sponsor (A17):
    Celiac.com Sponsor (A17):





    Celiac.com Sponsors (A17-M):




  • Recent Activity

    1. - Jeff Platt replied to Vozzyv's topic in Celiac Disease Pre-Diagnosis, Testing & Symptoms
      1

      Weird Symptoms

    2. - cristiana replied to Kathleen JJ's topic in Celiac Disease Pre-Diagnosis, Testing & Symptoms
      10

      Options - 7 year old boy - Helicobacter pylori and serology

    3. - cristiana replied to Kathleen JJ's topic in Celiac Disease Pre-Diagnosis, Testing & Symptoms
      10

      Options - 7 year old boy - Helicobacter pylori and serology

    4. - Kathleen JJ replied to Kathleen JJ's topic in Celiac Disease Pre-Diagnosis, Testing & Symptoms
      10

      Options - 7 year old boy - Helicobacter pylori and serology

    5. - CXinjera 2 posted a topic in Gluten-Free Recipes & Cooking Tips
      0

      Injera recipe ?


  • Celiac.com Sponsor (A19):



  • Member Statistics

    • Total Members
      126,097
    • Most Online (within 30 mins)
      7,748

    Claire Singer
    Newest Member
    Claire Singer
    Joined

  • Celiac.com Sponsor (A20):


  • Forum Statistics

    • Total Topics
      120.9k
    • Total Posts
      69.2k

  • Celiac.com Sponsor (A22):





  • Celiac.com Sponsor (A21):



  • Popular Now

    • Kathleen JJ
    • Captain173
      10
    • jjiillee
      7
    • Kristina12
      7
    • StaciField
  • Popular Articles

    • Scott Adams
    • Scott Adams
    • Scott Adams
    • Scott Adams
    • Scott Adams
  • Upcoming Events

×
×
  • Create New...