21/04/2024
تم بحمد الله تقديم بحثي الجديد ( يعتبر من اوائل الابحاث عالميا في علاج التقرحات و الفقاعات الحويصليه التي تصيب الفم و الوجه و الفكين) بعنوان:
مثبطات يانوس كيناز (JAK inhibitors ) لعلاج اضطرابات المناعة الذاتية اللزجة المناعية للجلد والأغشية المخاطية. التطورات الحديثة ومراجعة الأدبيات
(تم تقديمه في المجلة البريطانية للأمراض الجلدية، أبريل 2024)
Janus kinase (JAK) inhibitors for the treatment of Autoimmune Visiculo-immunobullous disorders of Skin and Mucous Membrane. Recent Advancements and Literature Review
( Submitted, British Journal of Dermatology, April, 2024)
Mohammed A El Maaytah
Oral & Maxillofacial Surgery and Disease Consultant
Amman
Jordan
Autoimmune Visiculo-immunobullous disorders are rare conditions characterized by erosion, blistering, and bullous lesions on the skin and mucous membranes. These diseases arise from the production of autoantibodies that target proteins responsible for maintaining cell-to-cell and cell-to-matrix adhesion. These diseases can be categorized into two groups: Intraepidermal immunobullous disease and Subepidermal immunobullous disease). Intraepidermal diseases encompass pemphigus vulgaris (PV), pemphigus foliaceus (PF), paraneoplastic pemphigus (PNP), immunoglobulin A (IgA) pemphigus, and others. Subepidermal diseases include bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA), anti-lamininγ1 pemphigoid, mucous membrane pemphigoid (MMP), dermatitis herpetiformis (DH), linear IgA bullous dermatosis, pemphigoid gestationis, lichen planus pemphigoid (LPP), and bullous systemic lupus erythematosus.
Currently, the preferred treatment for of these disorders involves the use of glucocorticoids or traditional immunosuppressants. Additionally, the utilization of biological agents such as rituximab, omalizumab, and dupilumab is on the rise. The Janus kinase/signal transducers and activators of transcription (JAK/STAT) pathway has been implicated in various inflammatory diseases. In recent years, a range of drugs known as JAK inhibitors, which target this pathway, have been developed.
Janus kinase inhibitors (JAKi) also known as JAK inhibitors or jakinibs, are a type of medication that functions by inhibiting the activity of one or more of the Janus kinase family of enzymes (JAK1, JAK2, JAK3, TYK2), thereby interfering with the JAK-STAT signaling pathway.
JAK inhibitors are a family of tyrosine kinases that mediate cytokine-receptor signaling through phosphorylation and activation of signal transducer and activator of transcription (STAT) nuclear proteins, which are critical for leukocyte activation and proliferation and play a major role in immune regulation.
Several studies have explored the efficacy and safety of JAK inhibitors for treating Autoimmune Visiculo-immunobullous disorders. Consequently, this review begins by examining the role of the JAK/STAT pathway in Autoimmune Visiculo-immunobullous disorders summarizing the application of different JAK inhibitors in these disorders treatment, and emphasizing the importance of disease management using JAK inhibitors. Furthermore, it highlights the need for a better understanding of the JAK/STAT pathway’s role in Visiculobollus diseases , as well as the effectiveness and safety of JAK inhibitors for treating this disease.
Summary:
�JAK inhibitors were effective in cases with unsuccessful responses to other therapies.��Tofacitinib and baricitinib were effective in treating cases of MMP, DH, and EB.��No severe adverse effect following the use of JAK inhibitors was reported.