Upcoming treatments for morphea

Abstract

Morphea (localized scleroderma) is a rare autoimmune connective tissue disease with variable clinical presentations, with an annual incidence of 0.4–2.7 cases per 100,000. Morphea occurs most frequently in children aged 2–14 years, and the disease exhibits a female predominance. Insights into morphea pathogenesis are often extrapolated from studies of systemic sclerosis due to their similar skin histopathologic features; however, clinically they are two distinct diseases as evidenced by different demographics, clinical features, disease course and prognosis. An interplay between genetic factors, epigenetic modifications, immune and vascular dysfunction, along with environmental hits are considered as the main contributors to morphea pathogenesis. In this review, we describe potential new therapies for morphea based on both preclinical evidence and ongoing clinical trials. We focus on different classes of therapeutics, including antifibrotic, anti‐inflammatory, cellular and gene therapy, and antisenolytic approaches, and how these target different aspects of disease pathogenesis.

Intramuscular Polydeoxyribonucleotides in Fibrotic and Atrophic Localized Scleroderma: An Explorative Prospective Cohort Study

“…At present, the purpose of therapy during the inactive stage of the localized scleroderma can only be to act on the connective tissue dystrophy expressing as an imbalance between deposition and degradation of collagen and leading to atrophic or sclerotic outcomes. This challenging endeavor has yet to be conclusive [ 1 , 8 , 10 ].…”

“…The literature acknowledges methotrexate, UV-A1 (340–400 nm), and psoralen UVA as the currently most effective therapies [ 1 , 28 , 29 ]. For instance, in subjects exposed to low-dose and medium-dose radiation, clinical recovery, described as fair to good response, increased from 46.2% to 72.7%, reaching 70% of considerable improvement in patients treated with high-dose UV-A1, although with a side effect burden (erythema, pruritus, burning sensations) of 15% [ 28 ].…”

“…Should it always be called morphea, dismissing all references to systemic sclerosis and acknowledging it is a different disease? The many similar histologic features between systemic and localized sclerosis suggest a similar pathogenesis [ 1 ]. However, the lack of fingertip ulcerations, sclerodactyly, Raynaud’s phenomenon, and autoantibodies specific to systemic sclerosis differentiate morphea from systemic scleroderma [ 2 , 3 ].…”

“…Current treatment recommendations include topical and systemic corticosteroids, topical calcineurin inhibitors, and systemic immunosuppressants like methotrexate, cyclosporine, or mycophenolate mofetil, all with a grievous side effect burden and all acting on the inflammatory phase. Therapeutic options targeted to the non-inflammatory fibrotic and atrophic morphea stages are currently scarce and inconclusive [ 1 , 10 ].…”

Удосконалення Комплексного Лікування Хворих На Локалізовану Склеродермію, Поєднану З Лайм-Бореліозом

“…Локалізована склеродермія (ЛС, морфеа) -це автоімунне захворювання, при якому уражається переважно шкіра та підшкірна клітковина, що призводить до їх склерозу і фіброзу [1]. Спочатку виникає запалення (рання фаза), після якого настають пізні склеротичні та/ або атрофічні зміни [2,3].…”