NEW CLASSIFICATION CRITERIA FOR SCLERODERMA SYSTEMATICA (a lecture)

Abstract

In 1980, the American College of Rheumatology proposed the first criteria as tentative for the diagnosis of scleroderma systematica (SDS). These criteria were aimed at revealing the comprehensive picture of mainly the diffuse form of the disease. They could not timely make a diagnosis in the low manifestive forms of the disease or virtually reveal its early stages that were most therapeutically promising. The review traces the evolution of approaches to diagnosing SDS and considers the novel classification criteria elaborated to select patients for epidemiological surveys and clinical trials. They include 8 parameters, each having scores. Skin thickening on both hands above the metacarpophalangeal articulations was highest (9 scores). The clinical variants of skin thickening on the fingers as its swelling (2 scores) or sclerodactyly (4 scores) are estimated from the maximum scores, as digital ischemia (sores, 2 scores; cicatricles, 3 scores). Teleangiectasias and characteristic capillaroscopic changes, as well as leading lung injury (pulmonary hypertension and/or interstitial lung disease) are rated as 2 scores each. Three scores are added to the total amount when Raynaud's syndrome is present and SDS-specific (anti-Scl-70, anti-centromere, and anti- RNA polymerase III) autoantibodies are detected. The patients gaining a total of 9 scores or more are classified as having definite SDS. Testing the novel criteria on a validation sample of 405 people showed that their sensitivity and specificity were 91 and 92%, respectively.