Deciphering the alternatively activated (M2) phenotype of macrophages in scleroderma

Scleroderma refers to a heterogeneous group of autoimmune fibrosing disorders which includes systemic sclerosis (SSc) and localized scleroderma, also known as morphea (1). SSc is characterized by widespread vascular injury, autoimmunity and progressive fibrosis of the skin and multiple internal organs, resulting in multifaceted and severe clinical manifestations (2). Hence, SSc has the highest disease-specific mortality of all autoimmune connective tissue diseases.