Gupta Rajiv, Gow Peter J
Department of Rheumatology, Middlemore Hospital, Private Bag 93311, Otahuhu, South Auckland, New Zealand.
N Z Med J. 2013 Mar 1;126(1370):89-95.
To estimate incidence, review clinical characteristics and management of newly diagnosed patients with idiopathic inflammatory myositis in Counties Manukau and to compare the findings with other reported series in New Zealand and overseas.
A case note study of computer generated data of patients having a diagnosis of inflammatory myopathy from January 2004 to December 2008 were included in this retrospective review.
Twelve patients were newly diagnosed in the 5-year period. Polymyositis (PM) was diagnosed in 58%, dermatomyositis (DM) in 33% and inclusion body myositis (IBM) in 8%. Amyopathic dermatomyositis (ADM) and malignancy associated dermatomyositis were diagnosed in one patient each. There was slight preponderance of men in dermatomyositis and women in polymyositis. Muscle biopsy was performed in 75% and electromyography was reported in 58%. High-dose prednisone was administered to 83%, and 50 % required other immunosuppressives, such as methotrexate (33%) and azathioprine (16%). Overall therapeutic response was good (75%) with 2 deaths (17%), one each in dermatomyositis and polymyositis. Regular follow-up was maintained in 92 %. Nasopharyngeal carcinoma was diagnosed in a patient with polymositis during follow up.
Idiopathic inflammatory myositis is a challenging group of heterogenous disorders. This study highlights the need to review current criteria to include subcategories, such as amyopathic dermatomyositis, and the importance of long term surveillance to detect occult malignancy.
评估曼努考郡新诊断的特发性炎性肌病患者的发病率，回顾其临床特征及治疗情况，并将结果与新西兰及海外其他报道系列进行比较。
本回顾性研究纳入了2004年1月至2008年12月诊断为炎性肌病患者的计算机生成数据的病例记录。
5年期间有12例新诊断患者。其中58%诊断为多发性肌炎（PM），33%为皮肌炎（DM），8%为包涵体肌炎（IBM）。各有1例患者诊断为无肌病性皮肌炎（ADM）和恶性肿瘤相关性皮肌炎。皮肌炎患者中男性略占优势，多发性肌炎患者中女性略占优势。75%的患者进行了肌肉活检，58%的患者有肌电图报告。83%的患者使用了大剂量泼尼松，50%的患者需要其他免疫抑制剂，如甲氨蝶呤（33%）和硫唑嘌呤（16%）。总体治疗反应良好（75%），2例死亡（17%），皮肌炎和多发性肌炎各1例。92%的患者进行了定期随访。随访期间1例多发性肌炎患者诊断为鼻咽癌。
特发性炎性肌病是一组具有挑战性的异质性疾病。本研究强调需要审视当前标准以纳入无肌病性皮肌炎等亚类，以及长期监测以发现隐匿性恶性肿瘤的重要性。
