Black C M
Department of Rheumatology, Royal Free Hospital, London, UK.
Clin Exp Rheumatol. 1994 Sep-Oct;12 Suppl 10:S75-81.
Scleroderma is a spectrum of disorders of which juvenile scleroderma forms a part. Fortunately, most children develop one of the localised forms and systemic disease is late. The prognosis of any individual type of scleroderma is dependent upon the subset of the disease, its extent, and internal organ involvement. In childhood, the problem is usually not one of survival, but of morbidity due to atrophy of skin, muscle and bone, with the potential for eventual growth defects and deformity. Drug treatment, when required, is usually directed towards suppressing inflammation and collagen deposition; vascular therapy does not play a major role, but physical therapy is crucial in certain forms of the disease.
硬皮病是一系列疾病,青少年硬皮病是其中一部分。幸运的是,大多数儿童患的是局限性硬皮病的一种类型,系统性疾病较为少见。任何一种硬皮病的预后取决于疾病的亚型、范围以及内脏器官受累情况。在儿童时期,问题通常不在于生存,而在于因皮肤、肌肉和骨骼萎缩导致的发病率,最终可能出现生长缺陷和畸形。如需药物治疗,通常旨在抑制炎症和胶原蛋白沉积;血管治疗作用不大,但物理治疗对某些类型的疾病至关重要。