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以皮肤和内脏纤维化为特征的进行性系统性硬化症(PSS)或硬皮病可能是一异质性疾病,其病因和发病学仍属不明,传统上将PSS视为结缔组织病。在各种分类法中,作者倾向于将PSS分为以下四种临床类型:1.CREST综合征,相当于肢端硬化症,临床经过较良性。2.肢端上升型,侵及臂及腿部(从远至近端)。3.弥漫型(四肢、躯干受累),预后不良。4.重叠综合征,包括混合性结缔组织病及硬化性皮肌炎。PSS发病学的现代
Progressive systemic sclerosis (PSS) or scleroderma, characterized by cutaneous and visceral fibrosis, may be a heterogeneous disease whose etiology and pathogenesis remains unknown and has traditionally been considered as connective tissue disease. Among the various taxonomies, the authors tended to classify PSS into the following four clinical types: 1. CREST syndrome, which is equivalent to extremity sclerosis and is clinically more benign. 2. Ascending type, invading the arm and leg (from as far as the proximal end). 3. Diffuse type (limbs, trunk involvement), the prognosis is poor. 4. Overlap syndrome, including mixed connective tissue disease and sclerosing dermatomyositis. PSS morbid modern