title: Systemic sclerosis - a systematic overview: part 1 - disease characteristics and classification, pathophysiologic concepts, and recommendations for diagnosis and surveillance.
abstract: Due to its high association with Raynaud's phenomenon systemic sclerosis (SSc) is probably the most common connective tissue disease seen by vascular specialists. In part 1 of our systematic overview we summarize classification concepts of scleroderma disorders, the epidemiologic and genetic burden, the complex pathophysiologic background, and the clinical features and the stage-dependent capillary microscopic features of SSc. Furthermore, we address the diagnostic recommendations propagated by the German Network for Systemic Sclerosis and the Task Force for Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology, the European Respiratory Society, and the International Society of Heart and Lung Transplantation.
authors.affiliation: Klinik für Innere Medizin, Schwerpunkt Angiologie und kardiovaskuläre Frührehabilitation, DRK Kliniken Berlin, Germany. p.klein-weigel@drk-kliniken-berlin.de
authors.forename: P
authors.initials: P
authors.lastname: Klein-Weigel
authors.forename: C
authors.initials: C
authors.lastname: Opitz
authors.forename: G
authors.initials: G
authors.lastname: Riemekasten
mesh_terms: D002196:Capillaries / Q000473:pathology / Q000503:physiopathology; D005060:Europe; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D017410:Practice Guidelines as Topic; D011237:Predictive Value of Tests; D011928:Raynaud Disease / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000145:classification* / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology; D012720:Severity of Illness Index; D012955:Societies, Medical; D009626:Terminology as Topic; D014456:Ulcer / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology