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[硬皮病的预后与治疗]

[Prognosis and treatment of scleroderma].

作者信息

Crickx B, Belaich S

出版信息

Ann Med Interne (Paris). 1984;135(8):624-9.

PMID:6529058
Abstract

The prognosis and treatment of scleroderma depend on the clinicopathological form of the disease: superficial, localised scleroderma is only a minor cosmetic problem; circumscribed, deep forms of scleroderma may cause troublesome symptoms which justify steroid therapy. Circumscribed and diffuse scleroderma are similar to systemic scleroderma for which prognostic criteria have been identified. There is a poor prognosis in men, in extensive skin involvement and in intestinal, cardiac or renal involvement. Limited skin disease and anti-centromere antibodies are associated with a good prognosis. The absence of an animal model and the uncertainty as to the underlying cause, explain the therapeutic problems. The authors discuss the respective value of a number of "specific" drugs, including D-penicillamine which seems to be the most hopeful line of treatment. Continuous follow-up of this chronic condition remains essential for the diagnosis and symptomatic management of visceral complications.

摘要

硬皮病的预后和治疗取决于疾病的临床病理类型

浅表性、局限性硬皮病只是一个较小的美容问题;局限性、深部硬皮病可能会引起令人烦恼的症状,这使得类固醇治疗成为合理选择。局限性和弥漫性硬皮病与系统性硬皮病相似,系统性硬皮病的预后标准已经明确。男性、广泛的皮肤受累以及肠道、心脏或肾脏受累时预后较差。皮肤病变局限且存在抗着丝点抗体提示预后良好。缺乏动物模型以及潜在病因的不确定性解释了治疗方面的问题。作者讨论了多种“特效”药物各自的价值,其中包括似乎是最有希望的治疗方法的D-青霉胺。对于这种慢性病持续进行随访对于内脏并发症的诊断和症状处理仍然至关重要。

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