DIAGNOSTIC AND PROGNOSTIC PARAMETERS OF P ROGRESSIVE SYSTEMIC SCLERODERMA

Progressive systemic scleroderma (PAA) ist characterized by fibroscler osis of the skin and numerous visceral organs. The prognosis of the di sease ist decisively determined by its tendency to spread and by the e xtent of the functional disturbances caused by the disorder. Depending on the cutaneous fibrosis pattern, various clinical forms of manifest ation are distinguished, each taking a prognostically different course . As the disease progresses protractedly, different patterns of fibros is become manifest which do not go beyond the hand or elbow region. Ho wever, if the fibrosclerosis ascends from peripheral to proximal upwar ds or if it develops at the trunk with a fibro-oedema and a tendency t o centrifugal spreading, the course is over-shadowed by functional dis turbances of visceral organs. An additional assessment of the prognosi s of progressive systemic scleroderma becomes possible by means of ant inuclear antibodies, especially anticentromeric antibodies or scl-70 a ntibodies; the prognosis is further assisted by including the genes of the HLA system in the assessment.