title: [Forty years study of systemic scleroderma (data of Institute of Rheumatology, Russian Academy of Medical Sciences)].
abstract: The basic stages and the results of 40-year studies of systemic scleroderma (SSD) at the Institute of Rheumatology, Russia Academy of Medical Sciences, are given. The goal-oriented studies of the systemic, peripheral, and visceral manifestations of the disease, which were undertaken in the 1960s basically altered our insight into the disease. Subsequently the diagnostic signs of the disease were developed, which substantially improved the diagnosis of SSD. Long-term studies examined the evolution of SSD and identified three major types of its course: acute, subacute, and chronic, which differ in the rapidity of progression of a pathological process, the pattern of clinical and morphological manifestations and prognosis, as evidenced by survival rates. The identified types of the course (n = 3) and clinical forms (n = 5) formed the basis for the Russia classification of SSD. Studying the pathogenesis of the disease mainly in the context of the mechanisms of formation of fibrosis and impaired microcirculation, as well as its clinical heterogeneity served as the basis for developing pathogenetic therapy regimens by differentially using disease-modifying agents and other therapeutical complexes. At present, the Institute of Rheumatology has accumulated unique experience in studying and following over 1500 patients with SSD and related diseases. There is a rise in the sclerodermal group of diseases, cross-over and combined forms of SSD along with great progress in the diagnosis and treatment, which enhances the quality of life of patients with SSD and prognosis in them.
authors.forename: N G
authors.initials: NG
authors.lastname: Guseva
mesh_terms: D000047:Academies and Institutes / Q000266:history; D049673:History, 20th Century; D006801:Humans; D012106:Research / Q000266:history; D012107:Research Design; D012219:Rheumatology / Q000266:history / Q000379:methods*; D012426:Russia; D012595:Scleroderma, Systemic / Q000266:history*