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      The role of intravenous immunoglobulin in treatment of mucous membrane pemphigoid: A review of literature

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          Abstract

          Background:

          Mucous membrane pemphigoid (MMP) is considered an autoimmune blistering disease that predominantly affects mucous membranes. Various treatments are available for controlling the diseases, but not all of them may respond.

          Materials and Methods:

          PubMed and Google Scholar were searched for all the associated studies until 2015, using the keywords such as “cicatricial pemphigoid” or “ocular pemphigoid” or “mucous membrane pemphigoid” or “MMP” and “intravenous immunoglobulin” or “IVIg” to find all the relevant studies. The last search update was for September 2, 2015. Among the searched items, only English studies were included in the review.

          Results:

          After excluding nonrelevant studies, 13 studies with a total number of seventy patients with MMP who were under treatment with IVIg were analyzed. The 65 patients responded completely, one did not respond, two had partially responded, and the remaining two patients stopped IVIg therapy, which resulted in ocular cicatricial pemphigoid progression. Majority of the studies reported mild adverse effects while two of them did not report any unwanted side effect. The most common side effect was headache, followed by nausea. Most of the patients who had a cessation of IVIg therapy before achieving clinical remission experienced the disease progression.

          Conclusion:

          Overall, it can be concluded that IVIg therapy was very helpful in treatment of MMP patients who did not respond to conventional therapy or stopped using them for various side effects. Adverse effects associated with IVIg therapy were considerably lower than conventional therapy that can lead toward treatment with this agent in patients who suffer from severe side effects.

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          Most cited references99

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          Immunomodulation of autoimmune and inflammatory diseases with intravenous immune globulin.

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            Rituximab: mechanism of action and resistance.

            Rituximab (Rituxan; Genentech, Inc, South San Francisco, CA, and IDEC Pharmaceuticals, San Diego, CA)-mediated killing of CD20-positive tumor cells is likely caused by a combination of immune-mediated effects including complement-mediated lysis and antibody-dependent cell-mediated cytotoxicity and direct effects induced by CD20 ligation. In vivo, the clearance of damaged or preapoptotic cells through specific receptors for phosphatidylserine translocated to the outer cell membrane may also be important. Direct effects, including growth inhibition and apoptosis, have been shown in vitro; however, their contribution to the clinical effect is not known. Currently, most data suggest that the predominant effector mechanism is antibody-dependent cell-mediated cytotoxicity, with a minor role of complement. With treatment, resistance to rituximab-mediated killing may emerge. Little is known regarding the molecular pathogenesis of this resistance. In rare cases, the CD20 antigen may be lost. Complement-resistance proteins may also increase, but it is not clear that this is the reason for loss of sensitivity. A better understanding of these mechanisms should allow combination therapy with agents capable of augmenting antibody-based killing. Copyright 2002 by W.B. Saunders Company.
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              Mucous membrane pemphigoid and pseudopemphigoid.

              To describe the clinical characteristics of patients with mucous membrane pemphigoid (MMP) and pseudopemphigoid. Retrospective cohort study. Two hundred eighty consecutive patients referred for the evaluation of possible ocular MMP from January 1, 1985, to December 31, 2001. Information on patients presenting for evaluation of possible MMP was entered prospectively into a database, which was supplemented by a retrospective chart review. Mucous membrane pemphigoid was diagnosed in patients with a compatible clinical picture by the linear deposition of antibodies to the basement membrane zone (BMZ) on direct immunofluorescent analysis of a mucous membrane biopsy specimen or by the presence of circulating autoantibodies to epithelial BMZ. Demographic and clinical characteristics of MMP and pseudopemphigoid; risk of ocular MMP among patients presenting with extraocular MMP without ocular disease. Among patients with ocular MMP, extraocular disease was common (82.4% of patients). The risk of ocular involvement among patients with MMP seen without ocular disease was approximately 5% per year over the first 5 years of follow-up (cumulative risk at 5 years, 22%). Although immunohistologic confirmation of the diagnosis was obtained in all patients, the initial conjunctival biopsy was positive for MMP in 80% of the patients diagnosed with ocular MMP. The most frequent presumed causes of pseudopemphigoid were topical glaucoma medications (28.3%), rosacea blepharoconjunctivitis (20.0%), atopic keratoconjunctivitis (8.3%), and conjunctival lichen planus (8.3%). Patients with ocular MMP typically have other systemic manifestations of MMP. Patients who are initially seen with extraocular MMP without ocular involvement are at risk for ocular disease developing. The clinical characteristics of ocular MMP and pseudopemphigoid are similar; therefore, immunohistologic evaluation of biopsied tissue is needed to confirm the diagnosis of MMP.
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                Author and article information

                Journal
                J Res Med Sci
                J Res Med Sci
                JRMS
                Journal of Research in Medical Sciences : The Official Journal of Isfahan University of Medical Sciences
                Medknow Publications & Media Pvt Ltd (India )
                1735-1995
                1735-7136
                2016
                14 June 2016
                : 21
                : 37
                Affiliations
                [1 ]Department of Dermatology, Faculty of Medicine, Iran University of Medical Sciences, Tehran, Iran
                [2 ]Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
                Author notes
                Address for correspondence: Dr. Soheil Tavakolpour, Infectious Diseases and Tropical Medicine Research Center, Shahid Labbafinejad Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran. E-mail: soheil.tavakolpour@ 123456gmail.com
                Article
                JRMS-21-37
                10.4103/1735-1995.183992
                5122191
                cc26f8a4-1eec-4b90-b83d-88cea9d6bfc3
                Copyright: © 2016 Journal of Research in Medical Sciences

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                History
                : 08 September 2015
                : 27 January 2016
                : 23 February 2016
                Categories
                Review Article

                Medicine
                cicatricial pemphigoid,intravenous immunoglobulin,mucous membrane pemphigoid
                Medicine
                cicatricial pemphigoid, intravenous immunoglobulin, mucous membrane pemphigoid

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