Publications by authors named "Pascal Joly"
Background: Pemphigus is an autoimmune bullous disease (AIBD) and has two main subtypes, pemphigus vulgaris (PV) and pemphigus foliaceus (PF). For adequate interpretation and comparison of clinical studies in pemphigus, it is essential to have outcomes and outcome measurement instruments (OMIs) that are well-defined, uniform and relevant.
Objectives: To provide a comprehensive overview of previously reported outcomes and OMIs in pemphigus clinical research over the past two decades.
Author Correction: Bullous pemphigoid.
Nat Rev Dis Primers
March 2025
Background: Desmocollin-3 (DSC3) is a calcium-dependent desmosomal cadherin that plays an essential role in cell-cell adhesion. IgG antibodies (Abs) directed against the extracellular (EC) domain of DSC3 have occasionally been detected in rare types of pemphigus. Investigations into the prevalence of anti-EC-DSC3 IgG Abs and those targeting the intracellular (IC) domain of DSC3 in pemphigus vulgaris and pemphigus foliaceus sera, and their potential pathogenic activity, have yielded conflicting results.
View Article and Find Full Text PDFBullous pemphigoid.
Nat Rev Dis Primers
February 2025
Bullous pemphigoid is a chronic, subepidermal autoimmune blistering disease characterized by tense blisters on erythematous or normal skin that predominantly affects the older population. The disease arises from autoantibodies targeting hemidesmosomal proteins BP180 and BP230, which are crucial for dermal-epidermal adhesion. The incidence of bullous pemphigoid is increasing, attributed to an ageing population and improved diagnostic recognition.
View Article and Find Full Text PDFImportance: Rituximab is approved for the treatment of moderate to severe pemphigus. However, 20% of patients in the RITUX 3 trial relapsed within the first year of treatment.
Objective: To assess the outcome of an additional rituximab infusion at month 6 in patients with pemphigus who were in complete remission (CR) after rituximab regimen but had 1 or more predictors of relapse at month 3.
The Lübeck Institute of Experimental Dermatology celebrated its 10 Anniversary Symposium on Inflammatory Skin Diseases at the University of Lübeck, Germany, on October 17-18, 2024. This event brought together international key opinion leaders, faculty members, researchers, and clinicians to foster insightful discussions on the diagnosis, pathomechanisms, and treatment of autoimmune skin diseases, with a particular focus on pemphigus, pemphigoid diseases, and systemic sclerosis.
View Article and Find Full Text PDFThe need to prevent corticosteroid-induced myopathy in patients with autoimmune bullous skin diseases.
J Eur Acad Dermatol Venereol
February 2025
Article Synopsis
- Bullous pemphigoid (BP) is an autoimmune blistering disease mainly seen in older adults, significantly affecting their quality of life.
- The disease involves autoantibodies against specific proteins and displays characteristics of type 2 inflammation, including high levels of IgE and eosinophils, along with increased type 2 cytokines in skin lesions.
- This review highlights the pathophysiology of BP, the impact of biologics targeting type 2 immune mediators, and the potential for future targeted therapies to improve treatment options.
Article Synopsis
- Avelumab is a treatment approved for metastatic Merkel cell carcinoma (mMCC) and this study focused on its effectiveness in patients in France who received it as a second-line or later treatment.
- The study analyzed data from 180 patients, revealing a median overall survival of 14.6 months after starting avelumab and a 40.5% survival rate at 24 months.
- The results showed that real-world outcomes for avelumab align with previous clinical trial findings, reinforcing its recommendation as a standard treatment for mMCC.
Article Synopsis
- Sézary syndrome is a rare and deadly skin cancer, and this study looked into the survival rates and factors affecting outcomes, particularly focusing on the treatment mogamulizumab.
- *The research analyzed data from 339 patients diagnosed between 2000 and 2020 across Europe, highlighting that age over 80 and large-cell transformation worsened survival rates.
- *Results indicated that patients treated with mogamulizumab had significantly lower mortality rates, suggesting it is an effective treatment option for Sézary syndrome.
Efficacy of virtual reality on pain and anxiety management in dermatological procedures.
J Eur Acad Dermatol Venereol
March 2025
The emergence of circulating activated autoreactive desmoglein 3-specific follicular regulatory T cells is associated with long-term efficacy of rituximab in patients with pemphigus vulgaris.
Br J Dermatol
September 2024
Article Synopsis
- Pemphigus vulgaris (PV) is an autoimmune disease marked by autoantibodies targeting adhesion proteins and is associated with the HLA-DRB1*0402 gene, with treatment involving rituximab and corticosteroids leading to disease control.
- The study aimed to assess how rituximab affects specific T lymphocyte subpopulations that regulate B-cell responses, particularly follicular helper T (Tfh) and follicular regulatory T (Tfr) cells, in patients with PV.
- Findings revealed a decrease in autoreactive Dsg3-specific Tfh cells post-rituximab treatment, alongside the emergence of a notable Tfr subpopulation, indicating that these Tfr cells may play a role
Efficacy and Safety of Rilzabrutinib in Pemphigus: PEGASUS Phase 3 Randomized Study.
J Invest Dermatol
August 2024
Trial Design: Pemphigus is a rare but life-threatening autoimmune disease requiring long-term treatment that minimizes corticosteroid (CS) exposure while providing consistent disease control. The phase 2 pemphigus study of oral, reversible, covalent Bruton tyrosine kinase inhibitor rilzabrutinib demonstrated rapid and sustained efficacy with well-tolerated safety.
Methods: Adults (aged 18-80 years) were randomized 1:1 to 400 mg rilzabrutinib (n = 65) or placebo (n = 66) twice daily (with CS ≤ 0.
Article Synopsis
- Bullous pemphigoid (BP) is a rare autoimmune skin disease causing significant suffering and reduced quality of life, currently managed with corticosteroids and immunosuppressants that have unwanted side effects.
- A clinical trial named LIBERTY-BP ADEPT is underway to investigate the effectiveness and safety of dupilumab, a drug that targets type 2 inflammation, in treating adults with moderate-to-severe BP.
- The trial includes a 52-week treatment period for 98 adults, focusing on achieving steroid-free remission and other key outcomes such as itch reduction and disease severity over time.
Article Synopsis
- Linear IgA Dermatosis (LAD) is a rare autoimmune skin disease characterized by IgA deposits at the skin's basement membrane, affecting both children and adults with diverse clinical features.
- The European Academy of Dermatology and Venereology (EADV) developed consensus guidelines by collaborating with 29 experts across multiple countries to ensure a comprehensive approach to diagnosis and treatment.
- The resulting guidelines provide a combination of evidence-based and expert-based recommendations to aid dermatologists in effectively diagnosing and managing LAD.
Article Synopsis
- The Ritux 3 trial showed that rituximab is effective and safe as a first-line treatment for pemphigus, although long-term follow-up data was previously lacking.!* -
- This 7-year follow-up study evaluated the long-term outcomes for patients treated with rituximab alongside prednisone versus those treated with prednisone alone, focusing on disease-free survival and relapse rates.!* -
- Results indicated that 93% of patients in the rituximab group achieved complete remission without corticosteroids, showing significantly higher disease-free survival and lower relapse rates compared to the prednisone-only group.!*