“…The drugs in the dark red boxes (cyclophosphamide, mycophenolate, azathioprine and methotrexate) are all myelosuppressives: combining these results in unacceptable toxicity. Some authors have described the use of CD 20 inhibitors (such as Rituximab) with existing myelosuppressive therapy for unresponsive ocular MMP 119 but this may be unsafe and we follow the recommendations of restricting adjunctive drugs to sulfas 120 and stop the myelosuppressives immediately before the first CD20 monoclonal infusion. Prednisolone can generally be given for a short term effect with any of the other drugs listed but is usually needed in high doses, with its associated side effects.…”
This review is in two sections. The first section summarises 35 conditions, both common and infrequent, causing cicatrising conjunctivitis. Guidelines for making a diagnosis are given together with the use of diagnostic tests, including direct and indirect immunofluorescence, and their interpretation. The second section evaluates our knowledge of ocular mucous membrane pemphigoid, which is the commonest cause of cicatrizing conjunctivitis in most developed countries. The clinical characteristics, demographics, and clinical signs of the disease are described. This is followed by a review and re-evaluation of the pathogenesis of conjunctival inflammation in mucous membrane pemphigoid (MMP), resulting in a revised hypothesis of the autoimmune mechanisms causing inflammation in ocular MMP. The relationship between inflammation and scarring in MMP conjunctiva is described. Recent research, describing the role of aldehyde dehydrogenase (ALDH) and retinoic acid (RA) in both the initiation and perpetuation of profibrotic activity in MMP conjunctival fibroblasts is summarised and the potential for antifibrotic therapy, using ALDH inhibition, is discussed. The importance of the management of the ocular surface in MMP is briefly summarised. This is followed with the rationale for the use of systemic immunomodulatory therapy, currently the standard of care for patients with active ocular MMP. The evidence for the use of these drugs is summarised and guidelines given for their use. Finally, the areas for research and innovation in the next decade are reviewed including the need for better diagnostics, markers of disease activity, and the potential for biological and topical therapies for both inflammation and scarring.
“…The drugs in the dark red boxes (cyclophosphamide, mycophenolate, azathioprine and methotrexate) are all myelosuppressives: combining these results in unacceptable toxicity. Some authors have described the use of CD 20 inhibitors (such as Rituximab) with existing myelosuppressive therapy for unresponsive ocular MMP 119 but this may be unsafe and we follow the recommendations of restricting adjunctive drugs to sulfas 120 and stop the myelosuppressives immediately before the first CD20 monoclonal infusion. Prednisolone can generally be given for a short term effect with any of the other drugs listed but is usually needed in high doses, with its associated side effects.…”
This review is in two sections. The first section summarises 35 conditions, both common and infrequent, causing cicatrising conjunctivitis. Guidelines for making a diagnosis are given together with the use of diagnostic tests, including direct and indirect immunofluorescence, and their interpretation. The second section evaluates our knowledge of ocular mucous membrane pemphigoid, which is the commonest cause of cicatrizing conjunctivitis in most developed countries. The clinical characteristics, demographics, and clinical signs of the disease are described. This is followed by a review and re-evaluation of the pathogenesis of conjunctival inflammation in mucous membrane pemphigoid (MMP), resulting in a revised hypothesis of the autoimmune mechanisms causing inflammation in ocular MMP. The relationship between inflammation and scarring in MMP conjunctiva is described. Recent research, describing the role of aldehyde dehydrogenase (ALDH) and retinoic acid (RA) in both the initiation and perpetuation of profibrotic activity in MMP conjunctival fibroblasts is summarised and the potential for antifibrotic therapy, using ALDH inhibition, is discussed. The importance of the management of the ocular surface in MMP is briefly summarised. This is followed with the rationale for the use of systemic immunomodulatory therapy, currently the standard of care for patients with active ocular MMP. The evidence for the use of these drugs is summarised and guidelines given for their use. Finally, the areas for research and innovation in the next decade are reviewed including the need for better diagnostics, markers of disease activity, and the potential for biological and topical therapies for both inflammation and scarring.
“…Es existieren mehrere Fallberichte, die ein gutes Ansprechen bei Therapieversagen zeigten [44,45]. B) Interleukin-2-Rezeptor-Antagonisten Daclizumab ist nicht in der Europäischen Union zugelassen.…”
The ocular cicatricial pemphigoid (OCP) belongs to a family of chronically progressive autoimmune disorders, predominantly affecting mucous membranes (mucous membrane pemphigoids). It is an immunopathologically heterogeneous group of disorders with variable phenotypes that share the unique feature of a subepidermal blistering, through disruption of the adhesion between epidermis and dermis. A key feature is the chronically active inflammation with consecutive fibrosis, leading to a partial or complete loss of function of the affected organ. The ocular disease as a chronic cicatrising conjunctivitis is a common manifestation of the mucous membrane pemphigoid. The identification of the subtle pathology and the prompt initiation of an appropriate therapy are of pivotal importance. One purpose is to prevent further vision loss due to extensive corneal scarring and life-threatening systemic complications, such as the formation of oesophageal or tracheal strictures. So far there are no prospective, randomised studies, regarding the therapy guidelines with an evidence level more than III. The autoimmune nature of the disease implies that systemic immunosuppression is the only effective treatment option, most notably in extended stages. The aim of our study is to give a guideline for a stage adjusted therapy with conventional immunosuppressants and to give a perspective for alternative therapies, especially for recalcitrant disease.
“…Aucune étude prospective n'est à ce jour disponible, néanmoins les données illustrées par plusieurs cas rapportés laissent entrevoir des perspectives prometteuses [51][52][53]. La réponse inflammatoire à la synthèse des auto-anticorps de la PC est responsable d'une expression accrue de cytokines parmi lesquelles le TNFa, trouvé à des concentrations importantes dans le sérum des patients atteints [54].…”
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