Chandran G, Smith M, Ahern M J, Roberts-Thomson P J
Rheumatology Unit, Flinders Medical Centre, Adelaide, SA.
Aust N Z J Med. 1995 Dec;25(6):688-94. doi: 10.1111/j.1445-5994.1995.tb02854.x.
Scleroderma is a systemic rheumatic disorder seen in a wide range of clinical specialties.
To establish the prevalence and mortality rates of scleroderma in South Australia (SA), to determine the relative frequency and characteristics of the three principal subsets (diffuse, limited and overlap), and to examine the role of nailfold capillaroscopy in subset identification and implied prognosis.
Outpatient and discharge diagnostic indexes from five major teaching hospitals in SA were reviewed between February 1987 and November 1993. A total of 215 patients with scleroderma were identified. Case notes of 115 of these patients were reviewed in order to validate scleroderma diagnosis, and subset characteristics such as sex, mean age at diagnosis, extent of skin involvement, internal organ involvement and serology were analysed. Fifty-two of these patients were then examined prospectively to confirm positive discharge diagnosis, and nailfold capillaroscopy was performed on these patients.
The point prevalence of scleroderma in SA for 1993 was estimated to be 208/10 This figure is a conservative estimate and is higher than most other reported series. The female to male ratio was 4:1. The majority of patients had limited disease with a ratio of 6:1:1.6 limited vs diffuse vs overlap. Systemic involvement excluding the oesophageal components limited disease was found predominantly in the diffuse group. Autoimmune serology was positive in 90% of patients, with Scl-70 being more common in diffuse scleroderma, anti-centromere antibody (ACA) in the limited form and anti-ribonucleoprotein (RNP) in the overlap form. Nailfold capillaroscopy was useful in predicting disease-subtype as capillary dilatation was observed predominantly in limited disease, capillary dropout in diffuse disease.
Scleroderma is more common in SA than previously recognised. Limited disease is more common than diffuse or overlap disease, carries a better prognosis and in associated with ACA. Nailfold capillaroscopy is a useful tool in disease assessment and may provide useful diagnostic and prognostic information.
硬皮病是一种在广泛临床专科中可见的系统性风湿性疾病。
确定南澳大利亚州（SA）硬皮病的患病率和死亡率，确定三个主要亚型（弥漫型、局限型和重叠型）的相对频率及特征，并研究甲襞毛细血管镜检查在亚型识别及隐含预后方面的作用。
回顾了1987年2月至1993年11月期间SA五家主要教学医院的门诊及出院诊断指标。共识别出215例硬皮病患者。对其中115例患者的病历进行回顾以验证硬皮病诊断，并分析亚型特征，如性别、诊断时的平均年龄、皮肤受累范围、内脏器官受累情况及血清学。然后对其中52例患者进行前瞻性检查以确认出院诊断，并对这些患者进行甲襞毛细血管镜检查。
1993年SA硬皮病的点患病率估计为208/10万。该数字为保守估计，高于大多数其他报道系列。男女比例为4:1。大多数患者为局限型疾病，局限型、弥漫型和重叠型的比例为6:1:1.6。排除食管成分的系统性受累在局限型疾病中主要见于弥漫型组。90%的患者自身免疫血清学呈阳性，Scl - 70在弥漫性硬皮病中更常见，抗着丝点抗体（ACA）在局限型中常见，抗核糖核蛋白（RNP）在重叠型中常见。甲襞毛细血管镜检查有助于预测疾病亚型，因为毛细血管扩张主要见于局限型疾病，毛细血管缺失见于弥漫型疾病。
硬皮病在SA比之前认为的更常见。局限型疾病比弥漫型或重叠型疾病更常见，预后更好且与ACA相关。甲襞毛细血管镜检查是疾病评估的有用工具，可能提供有用的诊断和预后信息。
