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Relapsing Polychondritis: A Chameleon Among Orphan Diseases

Relapsing polychondritis (RPC) is a rare disease with recurrent episodes of inflammation of cartilage tissue leading to fibrosis and organ damage. Despite unknown etiology, there is some evidence of a genetic predisposition. The clinical presentation is heterogeneous and an association with other autoimmune disorders such as rheumatoid arthritis or different forms of vasculitis has been described. All organ systems containing cartilage can be affected, such as ear, nose, joints, trachea, aorta, and coronary arteries. Given the broad spectrum of potential manifestations, a variety of medical specialists may be involved in the management of RPC patients. As establishing the diagnosis of RPC may be difficult, an interdisciplinary approach may be preferable. Treatment options include glucocorticoids, dapsone, disease-modifying antirheumatic drugs, and biologics. Prognosis is as heterogeneous as the clinical picture, depending on the severityof organ damage. In this paper we give an overview of the current knowledge with regard to pathogenesis, clinical picture, diagnosis, and therapy of RPC.

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Comments(2)

  1. REPLY
    Marta Bencomo Villar says

    I was diagnosed in 2014. After being treated for fibromyalgia ,cartilage in my feet Broken toes ,swollen joints ,stressed at work sitting for long periods of time and tightness in my whole body. Headaches itchy ears ,hoarse voice,hair falling out.fingers hurting nose,ears swelling up,and very weirded rashes that came and went.a lot of infections of skin and lose of hearing in one of my hears.they started me on rytuxin infused
    Shin 1000 mg and autoimmune suppressor so. Predisone.then got diagnosed with left breast cancer.Had a mestectomy.still having a lot of tiredness and stiffness all over.my body feels like it going to crack..break outs of rash on my arms with blisters that open up
    Who can help me.i have been diagnosed dystopia,stenosis of the spine.

    • REPLY
      Carol Giordano says

      Hello Marta,
      My name is Carol, I’ve been a Relapsing Polychondritis patient for nearly 20 years. I have been moderator of two support groups for 15 years and on the Foundation board since it’s inception 4 years ago.

      I’m really sorry to hear of your difficulties through the years. Your symptoms sound like many that I’ve had over the years. I am also receiving Rituxan infusions, along with Cytoxan infusions as well.

      There are people who can help you that can be found on this website. Under the Patient Support section is a list of all of our support groups. Talking with others who have similar problems is very helpful. And there are some who have or have had cancer as well.

      The other person to contact is the doctor who wrote this article, Dr. Marcela Ferrada. She is our Medical Liason with the National Institutes of Health. You may have read on this website about the clinical study she is currently running. She would be glad to talk with you via email. Her address is marcela.ferrada@nih.gov

      Please don’t hesitate to contact me directly if you’d like to talk further.

      I wish you all the very best.

      Sincerely,

      Carol Giordano
      cgiordano@polychondritis.org

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