View clinical trials related to Bullous Pemphigoid.
Filter by:Bullous pemphigoid affects mainly elderly patients. It is often associated with neurologic disorders, which represent a major risk factor of the disease
Bullous pemphigoid (BP) is a chronic, inflammatory, subepidermal, autoimmune blistering disease which mainly develops in the elderly, with onset usually in the late 70s and a substantial increase in incidence in people older than 80 years. If untreated, it can persist for months or years, with periods of spontaneous remissions and exacerbations. It has been found that blisters and sera of BP patients contain abnormally high levels of pro-inflammatory cytokines such as interleukin-6 (IL-6) and IL-8. Recently, it also has been demonstrated that NLRP3 (NACHT, LRR and PYD domains-containing protein 3) inflammasome components (the NLRP3-caspase-1-IL-18 axis) were significantly up-regulated in peripheral blood mononuclear cells from BP patients and positively correlated with disease activity. AC-203 is a topical formulation of an oral modulator of inflammasome and IL-1beta pathways. In vitro studies have demonstrated that AC-203 significantly reduced secretion of IL-6 and moderately reduced IL-8 secretion in HaCaT cells treated with specific anti-BP180 IgG. This study is designed to test the safety, tolerability, efficacy, and pharmacokinetics of AC-203 ointment (vs. a topical steroid comparator representing standard of care) ointment in subjects with BP.
Whereas pruritus is a major symptom in bullous pemphigoid, its characteristics and its impact on quality of life have been little studied. The objective of this study is to learn more about pruritus characteristics of patients with bullous pemphigoid, and in consequence to better understand the pathophysiology of pruritus, and then to better treat pruritus in this condition.
Recently, Interleukin (IL)-17 has been identified as a key driver of chronic inflammation in Bullous Pemphigoid (BP). Ixekizumab is a recombinant high-affinity fully human monoclonal antibody that targets IL-17A Immunoglobulin gamma-1 (IgG1)/kappa-class. The purpose of this study is to determine the effect of Ixekizumab on BP patients.
Bullous pemphigoid is the most common subepidermal autoimmune blistering disease of the skin in European countries, including France. Immunologically, BP is characterized by the production of autoantibodies directed against two major components of the hemidesmosome, BP180 and BP230. The anti-BP180 autoantibody is detected in 79-93% of cases of bullous pemphigoid and its serum level at diagnosis have been correlated with disease activity. The anti-BP230 autoantibody is detected in 57%-63% of bullous pemphigoid cases and its score at diagnosis did not correlate with disease activity. Up to now, no clinical or immunologic factors have been identified to predict outcome of patients with good or poor prognosis bullous pemphigoid as defined by long complete remission off therapy and recurrent disease requiring maintenance therapy for years. The usefulness of BP180 or BP230 ELISA scores for monitoring BP patients during treatment also remains unclear.
Autoimmune bullous dermatoses include pemphigus, bullous pemphigoid, pemphigoid gestationis, linear IgA dermatosis, mucous membrane pemphigoid, lichen planus pemphigoid, anti-p200 pemphigoid, epidermolysis bullosa acquisita and dermatitis herpetiformis. Autoimmune bullous dermatoses are rare and have an incidence of 20-60 new cases per 1 million person- year in Europe. The incidence of the individual entities is slight significantly different within Europe, but strongly also in comparison to other countries such as Kuwait, Singapore, USA and South America. The most common of these disorders is the bullous pemphigoid. A considerable progress has been made in the last years to elucidate the pathogenic role of autoantibodies in these diseases. To this end, various in vitro and animal experiments have been used to understand some basic pathophysiological mechanisms in these diseases. Further studies are currently being carried out to explain a precise elucidation of the disease process and to be able to treat the patients targeted later. At present, however, no data are available to explain why certain individuals develop the autoimmune disease and others do not. Epidemiological studies showed some triggers to the development of autoimmune dysregulation, e.g. drugs. Furthermore, it has been shown that genetic factors play a role in the pathogenesis of the disease. A clear association with certain HLA regions have been shown in patients with pemphigus, e.g. about 95% of pemphigus patients from the group of Ashkenazi Jews have the HLA-DRB1*0402 haplotype. Recently, the first non-HLA gene associated with pemphigus was described. For other conditions such as bullous pemphigoid, pemphigoid gestationis or linear IgA dermatosis the association with HLA antigens is less pronounced. Another indication of the importance of the genetic background in these diseases can be elucidated from the observation of autoantibodies at a low concentration in healthy relatives of pemphigus patients.
The study is an observational, multi-center, prospective, non-interventional and open-label data collection study assessing outcomes, treatment patterns, adverse events and costs in patients diagnosed with bullous pemphigoid. The patient enrollment period will be 1 year with a follow-up (observation period) of 1 year for each patient. Four dermatology centres in France will participate. The hypothesis to be answered by the study is that superpotent topical corticosteroid therapy is properly used to treat bullous pemphigoid in real-world life as recommended by French guidelines and whether this treatment influences the medical costs by comparison with systemic therapies (e.g. methotrexate or prednisone).
Pemphigus and bullous pemphigoid (BP) are severe autoimmune blistering diseases (AIBD) that pose a critical need for new therapeutic approaches. Clinical trials in pemphigus and BP will require the availability of validated disease severity measures that can be used to define primary outcomes.
Clinical observation frequently shows a paradoxical effect of topical corticosteroids in charge of a sudden melting of edema in the first days of treatment, which could be due to mobilization of extracellular. No study has shown the value of this measure in patients treated with topical steroids. This uncertainty, coupled with the observation of the paradoxical effects of topical steroids on edema are some patients that despite the systemic absorption of clobetasol propionate, a salt-free diet is not currently recommended practice.
This controlled multi-center randomized clinical trial, with direct individual benefit, will compare efficacy and safety of two strategies in non-localized BP care: a combined regimen using initial superpotent topical steroids associated with methotrexate for 4 weeks followed by methotrexate alone for 8 months and superpotent topical steroids alone maintained 9 months (current standard of care). The expected result is an equivalence between the two compared strategies in terms of safety and efficacy, MTX monotherapy during the maintenance phase being easier to manage and therefore associated with better compliance than topical steroids with less cutaneous side effects and most cost-effective.