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砂拉越的多发性肌炎和皮肌炎:砂拉越总医院治疗患者的特征。

Polymyositis and dermatomyositis in Sarawak: a profile of patients treated in the Sarawak General Hospital.

机构信息

Unit of Rheumatology, Department of Medicine, Sarawak General Hospital, Jalan Hospital, 93450 Kuching, Sarawak, East Malaysia.

出版信息

Rheumatol Int. 2012 Jan;32(1):265-8. doi: 10.1007/s00296-010-1745-2. Epub 2011 Jan 18.

Abstract

We performed a cross-sectional study of the demography, clinical and laboratory features of patients with polymyositis and dermatomyositis followed up in our centre from 2006 to 2009. There were 12 cases, with the majority of them (58.3%) being woman. They have a mean age of 57.8 years and mean disease duration of 11.83 (SD 9.92) months. Our patients comprised of multi-ethnic groups with predominantly Chinese (83.3%), Sarawak natives (8.3%) and Malays (8.3%). They have a mean lag time to diagnosis of 3.67 (SD 4.27) months. Nine (75%) patients had dermatomyositis and 3(25%) had polymyositis. The common clinical manifestations found in our patients were proximal myopathy (100%), neck weakness (33.3%), dysphagia (33.3%) and interstitial lung disease (33.3%). For the nine patients with dermatomyositis, the most common dermatological manifestations were shawl sign (88.9%) and V sign (88.9%). Muscle enzymes were raised in 91.7% of patients. Electromyographies were carried out in four patients, and only one of our patients had muscle biopsy. Only 41.7% of our patients have positive ANA. The majority received prednisolone (100%) and hydroxychloroquine (58.3%). Malignancy occurred in five (three nasopharyngeal carcinomas, one sigmoid colon cancer and one lung cancer) out of the nine dermatomyositis patients but none in the polymyositis group. The mortality rate in our group was 4(33.3%) over the 4-year period. This study demonstrated the rarity of PM/DM in our centre with considerable lag time to diagnosis in our patients. Despite lack of muscle biopsy in our centre, our centre achieved appropriate diagnosis and management of PM/DM.

摘要

我们对 2006 年至 2009 年在我中心随访的多发性肌炎和皮肌炎患者的人口统计学、临床和实验室特征进行了横断面研究。共有 12 例,其中大多数(58.3%)为女性。他们的平均年龄为 57.8 岁,平均病程为 11.83(SD 9.92)个月。我们的患者来自多个种族群体,其中以华人(83.3%)、砂拉越本地人(8.3%)和马来人(8.3%)为主。他们的平均诊断延迟时间为 3.67(SD 4.27)个月。9 例(75%)患者为皮肌炎,3 例(25%)为多发性肌炎。我们的患者常见的临床表现为肌病(100%)、颈部无力(33.3%)、吞咽困难(33.3%)和间质性肺病(33.3%)。在 9 例皮肌炎患者中,最常见的皮肤表现是披肩征(88.9%)和 V 征(88.9%)。91.7%的患者肌肉酶升高。对 4 例患者进行了肌电图检查,只有 1 例患者进行了肌肉活检。只有 41.7%的患者抗核抗体阳性。大多数患者接受泼尼松(100%)和羟氯喹(58.3%)治疗。9 例皮肌炎患者中有 5 例(3 例鼻咽癌、1 例乙状结肠癌和 1 例肺癌)发生恶性肿瘤,但在多发性肌炎组中没有。在 4 年期间,我们组的死亡率为 4(33.3%)。本研究表明,我中心 PM/DM 较为罕见,患者诊断延迟时间较长。尽管我中心缺乏肌肉活检,但仍能对 PM/DM 做出适当的诊断和治疗。

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