Efficacy and Safety of Rituximab Combined With Omalizumab in Patients With Bullous Pemphigoid
Bullous PemphigoidTo evaluate the efficacy of rituximab combined with omalizumab in achieving sustained complete remission, evaluated by Bullous Pemphigoid Disease Area Index (BPDAI) in patients with bullous pemphigoid (BP) at Week 24 in patients with active moderate-to-severe BP refractory to rituximab therapy alone.
Efficacy and Safety of Dupilumab in Patients With Bullous Pemphigoid
Bullous PemphigoidThis study was designed to be a retrospective, multicentre, observational study to evaluate the efficacy and safety of dupilumab in the treatment of bullous pemphigoid and to find predictors of efficacy.
Human Leukocyte Antigen Class II (DRB1 and DQB1) Alleles and Haplotypes Frequencies in Patients...
PemphigusBullous Pemphigoid2 morePemphigus is a rare autoimmune life-threatening blistering condition affecting skin and mucous membranes. Pemphigus belongs to a family of polygenic disorders. Several different genes encoding molecules regulate pemphigus autoimmunity. Many trials focused on HLA investigation. Increased levels of certain HLA class II alleles frequencies in pemphigus have been reported in various populations. However, they were not investigated in the Russian population. The aim of our study is to investigate HLA class II alleles and haplotypes in Russian patients with pemphigus. Methods Patients and controls We are recruiting 120 patients with pemphigus. The diagnosis was based on clinical and histopathological findings and confirmed by immunofluorescent techniques (direct and indirect immunofluorescent tests). Before sampling, written consent was obtained from each subject. A single blood sample for HLA typing was obtained from all subjects. This study has been approved by the Ethics Committee of Sechenov University, Russia. Phenotypic and allelic frequencies were compared with healthy blood donors (n=100) registered in Sechenov University blood center.
Proteomic and Metabolomic Lacrimal Fingerprint in Diverse Pathologies of the Ocular Surface
Dry Eye SyndromeInfectious Keratoconjunctivitis2 moreThis study aims to obtain the lacrimal fingerprint for frequent pathologies of the ocular surface and establish a normative base for each of them.
FARD (RaDiCo Cohort) (RaDiCo-FARD)
Inherited Epidermolysis BullosaIchthyosis7 moreThe goal of this observational study is to conduct a prospective assessment of the individual Burden of 9 rare skin diseases to assess disability in the broadest sense of the term (psychological, social, economic and physical) for patients and/or families. Two types of indicators will be used to reach this objective : an individual burden score calculated based on a burden questionnaire created specifically, approved and designed to understand the tendency to changes in care and lifestyles. The burden questionnaire should be used by patients and/or their family themselves in self-assessment. a descriptive analysis of all resources (medical and non-medical) used by the family unit to manage the disease.
Autoimmune Blistering Diseases Study
Pemphigus VulgarisPemphigus Foliaceus1 morePemphigus 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.
Pilot Efficacy and Safety Study of Oral DF2156A in Patients With Active Bullous Pemphigoid
Bullous PemphigoidThe objective of this clinical trial was to evaluate whether DF2156A has a potential in improving the clinical outcome in patients with active blistering bullous pemphigoid (BP) to warrant its further development. The safety of DF2156A in the specific clinical setting was also evaluated.
A Proof-of-Concept Study of Topical AC-203 in Patients With Bullous Pemphigoid
Bullous PemphigoidBullous 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.
A Study to Assess the Therapeutic Effect and Safety of Adjunctive AKST4290 in Subjects With Bullous...
PemphigoidBullousThis study will evaluate the therapeutic effect and safety of adjunctive AKST4290 in subjects with bullous pemphigoid (BP).
Safety, Efficacy and PK/PD of QGE031 vs. Placebo in Patients With Active Bullous Pemphigoid Despite...
Bullous PemphigoidTo evaluate the safety and efficacy of QGE031 versus placebo in patients with bullous pemphigoid. Efficacy will be assessed as a reduction of disease activity. How QGE031 is broken down by the body and the impact it has on different blood and tissue markers will also be explored.