典型皮肌炎
经过治疗,预后有改善,5 年生存率为 95%,10 年生存率为 84%。[180]Marie I, Hachulla E, Hatron PY, et al. Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis. J Rheumatol. 2001;28:2230-2237.http://www.ncbi.nlm.nih.gov/pubmed/11669162?tool=bestpractice.com尽管生存率有提高,但三分之一的患者会出现严重的永久性残疾。[181]Maugar YM, Berthelot JM, Abbas AA, et al. Long-term prognosis of 69 patients with dermatomyositis or polymyositis. Clin Exp Rheumatol. 1996;14:263-274.http://www.ncbi.nlm.nih.gov/pubmed/8809440?tool=bestpractice.com[182]Sultan SM, Ioannou Y, Moss K, et al. Outcome in patients with idiopathic inflammatory myositis: morbidity and mortality. Rheumatology (Oxford). 2002;41:22-26.http://rheumatology.oxfordjournals.org/cgi/content/full/41/1/22http://www.ncbi.nlm.nih.gov/pubmed/11792875?tool=bestpractice.com
尽管部分患者是急性发作,不需要长期维持治疗,但大部分患者都是包含复发-缓解过程的慢性疾病。大多数患者需要使用皮质类固醇或其他免疫抑制剂进行长期治疗,有很多会罹患与此相关的并发症。
无肌病性皮肌炎
临床无肌病性皮肌炎患者可能有肌肉病变的亚临床证据(少肌病性),或者真的没有肌病,通常早期有皮损表现。
肌炎不太严重,或对亚临床肌病进行早期诊断和治疗,反应会更好。[77]Dawkins MA, Jorizzo JL, Walker FO, et al. Dermatomyositis: a dermatology-based case series. J Am Acad Dermatol. 1998;38:397-404.http://www.ncbi.nlm.nih.gov/pubmed/9520020?tool=bestpractice.com皮肤病变通常对治疗抵抗更强,可导致严重的并发症发病率。[77]Dawkins MA, Jorizzo JL, Walker FO, et al. Dermatomyositis: a dermatology-based case series. J Am Acad Dermatol. 1998;38:397-404.http://www.ncbi.nlm.nih.gov/pubmed/9520020?tool=bestpractice.com
无肌病性皮肌炎患者需要连续随访,以筛查是否出现肌肉受累。不存在恶性肿瘤时预后良好。[183]Caproni M, Cardinali C, Parodi A, et al. Amyopathic dermatomyositis: a review by the Italian Group of Immunodermatology. Arch Dermatol. 2002;138:23-27.http://archderm.ama-assn.org/cgi/content/full/138/1/23http://www.ncbi.nlm.nih.gov/pubmed/11790163?tool=bestpractice.com[184]el-Azahary RA, Pakzad SY. Amyopathic dermatomyositis: retrospective review of 37 cases. J Am Acad Dermatol. 2002;46:560-565.http://www.ncbi.nlm.nih.gov/pubmed/11907509?tool=bestpractice.com
预后指标
结局不良与几个因素相关。其中包括:
症状发生后治疗延迟>6 个月[119]Joffe MM, Love LA, Leff RL, et al. Drug therapy of the idiopathic inflammatory myopathies: predictors of response to prednisone, azathioprine, and methotrexate and a comparison of their efficacy. Am J Med. 1993;94:379-387.http://www.ncbi.nlm.nih.gov/pubmed/8386437?tool=bestpractice.com[185]Fafalak RG, Peterson MG, Kagen LJ. Strength in polymyositis and dermatomyositis: best outcome in patients treated early. J Rheumatol. 1994;21:643-648.http://www.ncbi.nlm.nih.gov/pubmed/8035387?tool=bestpractice.com
就诊时有更大面积的肌无力[186]Carpenter JR, Bunch TW, Engel AG, et al. Survival in polymyositis: corticosteroids and risk factors. J Rheumatol. 1977;4:207-214.http://www.ncbi.nlm.nih.gov/pubmed/881699?tool=bestpractice.com
呼吸肌受累[187]Danko K, Ponyi A, Constantin T, et al. Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases. Medicine (Baltimore). 2004;83:35-42.http://www.ncbi.nlm.nih.gov/pubmed/14747766?tool=bestpractice.com
间质性肺病[181]Maugar YM, Berthelot JM, Abbas AA, et al. Long-term prognosis of 69 patients with dermatomyositis or polymyositis. Clin Exp Rheumatol. 1996;14:263-274.http://www.ncbi.nlm.nih.gov/pubmed/8809440?tool=bestpractice.com
恶性肿瘤[181]Maugar YM, Berthelot JM, Abbas AA, et al. Long-term prognosis of 69 patients with dermatomyositis or polymyositis. Clin Exp Rheumatol. 1996;14:263-274.http://www.ncbi.nlm.nih.gov/pubmed/8809440?tool=bestpractice.com
吞咽困难[186]Carpenter JR, Bunch TW, Engel AG, et al. Survival in polymyositis: corticosteroids and risk factors. J Rheumatol. 1977;4:207-214.http://www.ncbi.nlm.nih.gov/pubmed/881699?tool=bestpractice.com
心脏受累。[187]Danko K, Ponyi A, Constantin T, et al. Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases. Medicine (Baltimore). 2004;83:35-42.http://www.ncbi.nlm.nih.gov/pubmed/14747766?tool=bestpractice.com