TY - CHAP M1 - Book, Section TI - Systemic Sclerosis A1 - Moinzadeh, Pia A1 - Denton, Christopher P. A1 - Black, Carol M. A1 - Krieg, Thomas A2 - Kang, Sewon A2 - Amagai, Masayuki A2 - Bruckner, Anna L. A2 - Enk, Alexander H. A2 - Margolis, David J. A2 - McMichael, Amy J. A2 - Orringer, Jeffrey S. PY - 2019 T2 - Fitzpatrick's Dermatology, 9e AB - AT-A-GLANCEScleroderma (systemic sclerosis [SSc]) is a multisystemic autoimmune disease characterized by vasculopathy, inflammation, and fibrosis of the skin and many other organs.Differential diagnosis of SSc includes severe forms of localized scleroderma, as well as many other scleroderma-like conditions.Raynaud phenomenon, circulating autoantibodies, and skin sclerosis are almost always present and are important for the early diagnosis.Patients with SSc are classified into 2 major subtypes depending on the extent of skin sclerosis (diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis).Patients with an overlap syndrome, including mixed connective tissue disease, are characterized by additional clinical features of other rheumatic diseases.Involvement of internal organs (digestive tract, lung, kidney, and heart) can lead to severe dysfunction and determines the prognosis.The heterogeneity and clinical course of SSc and SSc overlap syndromes require the urgent need of interdisciplinary collaborations and regular followup visits.Although the disease is still not curable, there have been substantial advances in developing new therapeutic approaches and treating organ-based complications based on a better understanding of the pathophysiology. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessmedicine.mhmedical.com/content.aspx?aid=1162816818 ER -