Mucous Membrane Pemphigoid, also known as Cicatricial Pemphigoid, is a serious autoimmune blistering disorder that can result in blindness and other complications as the result of scarring of the mucous membranes. To our knowledge, there have been 2 reported uses of Etanercept for MMP in the literature.
Rituximab (RTX) is anti-CD20 chimeric antibody that selectively targets B cells. CD20 is a molecule that functions as an antigen for it. CD20 is expressed on mature antibody producing B cells, but not on plasma cells.(1) The FDA has approved the use of Rituximab for the treatment of B cell lymphomas.(2) Since the pathogenic and clinical manifestations of PV appear to be antibody related, it could be hypothesized that eliminating the pathogenic antibody and the cells that produce it, might be more effective than general nonspecific immune suppression. This is the rationale for using Rituximab in treating pemphigus vulgaris. Indeed, success with the Rituximab has been observed in many autoimmune diseases which are mediated by antibodies such as systemic lupus erythematosus and immune thrombocytopenic purpura.(3)
By Michelle Greer, RN, MBA
Michael T. Rigas, PharmD
Crescent Healthcare, Inc.
IVIG stands for intravenous immunoglobulin. Immunoglobulins are also known as antibodies, which are produced by the body to activate the immune system to attack virus and bacteria. IVIG is a medication that is a blood product. It is made from the plasma of thousands of carefully screened donors. 90 – 98% of IVIG is made up of Immunoglobulin G (IgG.) IgG is a Y-shaped protein that is the main antibody found in blood plasma.
The International Pemphigus & Pemphigoid Foundation and Dr. Sarah Brenner recently conducted a survey on some aspects of pemphigus, in particular the gender distribution of the patients, and the relationship between the disease and the use of sex hormones.
Herbal supplements are used widely, and some of these supplements may stimulate the immune system in ways that could be harmful for people who have or are prone to autoimmune diseases.
There are a number of herbal medications that have been studied and have immunostimulatory effects. Among these are Echinacea, the algae Spirulina platensis and Aphanizomenon flos-aquae. We recently reported three patients with autoimmune disease, two of whom had pemphigus vulgaris, whose disease exacerbated shortly after beginning one of these herbal medications (Lee and Werth, Arch Dermatol 140:723, 2004). In one case of dermatomyositis, the patient re-flared several months later after rechallenging herself with the same herbal product.
These herbs seem to affect the immune system by increasing pro-inflammatory proteins produced by inflammatory cells, and their effects have been studied in a number of experimental models. Studying the potential mechanisms and effects of these herbs in humans has been done in only a few studies, and much more work will be needed in order to document their potential toxicity in patients with autoimmune diseases. Until these studies are performed, it would be prudent for patients with pemphigus vulgaris to avoid potentially immunostimulatory herbal medications.
The function of the immune system was critical to survival of our species. Prior to the 20th century man’s greatest killers were, in fact, infectious diseases. It is important to note that not everyone’s immune system functions in the same way because genetic diversity determines how one individual will react to a given infection. Diversity in the immune response has protected us from devastating events. For example, not everyone who got the bubonic plaque died. Some individual had a genetically programmed immune response that was more effective than others and they were able to survive. There are many examples of that throughout history of our species.
Rituximab is a laboratory manufactured antibody that targets the cells in our bodies that produce antibodies. This was genetically engineered and is the first monoclonal antibody to be approved by the FDA for the treatment of cancer. The current indication for its use is in the treatment of non-Hodgkin’s lymphoma. It has become an important tool for patients with that disease.
by Sarah Brenner, MD, Jacob Mashiah, MD, Einat Tamir, MD, Ilan Goldberg, MD and Yonit Wohl, MD, Department of Dermatology, Tel Aviv Sourasky Medical Center, and Sackler Faculty of Medicine, Tel Aviv University, Israel
Pemphigus is generally considered to stem from a genetic predisposition to the disease triggered and/or aggravated by one or more external factors. An acronym has been suggested from the name of the disease, PEMPHIGUS, to encompass those factors:
This Plan takes us a giant step closer to getting additional funding for autoimmune disease research. Congressional approval is now needed to implement and fund the Plan. The Foundation is asking everyone to advocate for this Plan by contacting their Senators and Representatives and urging them to support it. This effort is being coordinated by the American Autoimmune Related Diseases Association (AARDA), which worked closely with NIH on the development of the Plan. Virginia Ladd, Executive Director of AARDA, said: “It is important for all patients with any autoimmune disease to fight for funding for this plan. Supporting autoimmune disease research will help all patients, regardless of their particular illness.”
by Edward Tenner, M.D.
The autoimmune bullous skin diseases, pemphigus (with major subsets pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus) and the more common bullous pemphigoid (with variant disease phenotypes of cicatricial pemphigoid and gestational pemphigoid) each may have ocular manifestations.