Barnett A J
Med J Aust. 1978 Aug 12;2(4):129-34. doi: 10.5694/j.1326-5377.1978.tb131413.x.
A follow-up study has been made of a personal series of 118 cases of patients with scleroderma (progressive systemic sclerosis) followed for periods up to 25 years. These were classified according to the early distribution of skin changes as Type 1 (skin changes in fingers only), 48 cases; Type 2 (skin changes beyond the fingers but mainly in extremities), 47 cases; and Type 3 (diffuse), 19 cases, and atypical, four cases. Eighty per cent of the total series have survived five years, and 68% have survived 10 years. Survival is related to the type of scleroderma, being much better for Types 1 and 2 (acrosclerotic) than for Type 3 (diffuse). Actual compared with expected survival rate was better for younger than for older patients and for females than for males. Although the disease type gives an indication of survival in general, the caution must be used in the individual cases, as the course of the disease is very variable even in patients of the same type, and is subject to remission and relapse.
对一组118例硬皮病（进行性系统性硬化症）患者进行了随访研究，随访时间长达25年。根据皮肤改变的早期分布情况，将这些患者分为：1型（仅手指皮肤改变），48例；2型（手指以外但主要在四肢的皮肤改变），47例；3型（弥漫性），19例，以及非典型性，4例。整个队列中有80%的患者存活了5年，68%的患者存活了10年。生存率与硬皮病的类型有关，1型和2型（肢端硬化型）的生存率远高于3型（弥漫型）。实际生存率与预期生存率相比，年轻患者优于老年患者，女性优于男性。尽管疾病类型总体上能表明生存率，但在个别病例中必须谨慎，因为即使是同一类型的患者，疾病进程也非常多变，且会出现缓解和复发。
