Abstract

Skin autoimmune conditions belong to a larger group of connective tissue diseases and primarily affect the skin, but might also involve underlying tissues, such as fat tissue, muscle, and bone. Autoimmune antibodies (autoantibodies) play a role in autoimmune skin diseases, such as localized scleroderma also termed morphea, and systemic scleroderma, also called systemic sclerosis (SSc). The detailed studies on the biological role of autoantibodies in autoimmune skin diseases are limited. This results in a few available tools for effective diagnosis and management of autoimmune skin diseases. This review aims to provide an update on the detection and most recent research on autoantibodies in morphea. Several recent studies have indicated the association of autoantibody profiles with disease subtypes, damage extent, and relapse potential, opening up exciting new possibilities for personalized disease management. We discuss the role of existing autoantibody tests in morphea management and the most recent studies on morphea pathogenesis. We also provide an update on novel autoantibody biomarkers for the diagnosis and study of morphea.

Highlights

  • Being a part of an abnormal immune response, autoimmune antibodies are valuable biomarkers in autoimmunity

  • Morphea is a complex disease with a diverse profile of clinical manifestations and still unresolved issues with serological diagnosis, clear knowledge on pathogenesis, and missing of effective management routes

  • We selected studies based on the number of enrolled patients with autoantibody testing (≥50 patients), relevance to studies of morphea pathogenesis, and reported emerging animal models and biomarkers that could lead to improved personalized morphea management

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Summary

Introduction

Being a part of an abnormal immune response, autoimmune antibodies (autoantibodies) are valuable biomarkers in autoimmunity. The causality of autoantibodies in autoimmune diseases is still controversial and requires more fundamental research [1]. Clinical assays detecting autoantibodies are commonly used to diagnose and categorize autoimmune diseases [2]. Recent studies have revealed distinct autoantibody profiles among patients with autoimmune diseases, opening up new avenues for better diagnostics and personalized disease management [3]. The prototypical rheumatologic autoimmune disease of the skin, systemic sclerosis (SSc), is often defined and subcategorized by an auto-antibody profile, in addition to the extent of skin involvement [4]. The true pathogenicity of these autoantibodies in SSc disease propagation remains to be elucidated

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