Systemic Sclerosis (Scleroderma)

  • Lazaros I. SakkasEmail author


Systemic sclerosis (SSc) is a rare systemic chronic disease characterized by widespread fibrosis of skin and internal organs, microvasculopathy, and autoantibodies that frequently affects internal organs and quality of life and survival. Disease-related autoantibodies, anti-topoisomerase I, anticentromere, and anti-RNA polymerase III antibodies, Raynaud’s phenomenon, and microvasculopathy, as detected by nailfold capillaroscopy, appear before skin fibrosis and help in early diagnosis. Based on the extent of skin involvement, SSc is divided into limited cutaneous SSc and diffuse cutaneous SSc. SSc-related autoantibodies are associated with manifestations and help in the management of SSc patients. In this chapter we review epidemiology, pathogenesis, clinical manifestations, specific organ involvement and assessment tools, diagnosis, differential diagnosis, and treatment.


Autoantibodies Differential diagnosis Manifestations Pulmonary arterial hypertension Systemic sclerosis Treatment 


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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health SciencesUniversity of ThessalyLarissaGreece

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