Kaufman L D, Gruber B L, Gregersen P K
Department of Medicine, State University of New York, Stony Brook 11794-8161.
Lancet. 1991 May 4;337(8749):1071-4. doi: 10.1016/0140-6736(91)91717-9.
31 patients with L-tryptophan-associated eosinophilia-myalgia syndrome (EMS) that developed during the United States outbreak in 1989 were followed up prospectively at a university hospital outpatient rheumatology clinic for 16 to 24 months from the onset of their illness. Another patient with EMS associated with L-tryptophan in 1988 was followed up for 30 months. 93% of the 28 survivors from the 1989 cohort continue to have symptoms affecting 1-4 organ systems (median 3) and 3 have died, so the disorder produces considerable morbidity and mortality. The chronic sequelae most often associated with long-term disability are sclerodermatous skin thickening (54%), sensorimotor polyneuropathy (61%), proximal myopathy (36%), and severe episodic myalgias (64%). Thrombocytopenia developed in 1 patient. HLA-class II typing revealed a non-significant trend towards an association with HLA-DR4. Early therapy with corticosteroids did not seem to prevent the development of chronic manifestations.
1989年美国爆发期间出现的31例与L-色氨酸相关的嗜酸性粒细胞增多性肌痛综合征(EMS)患者,在一所大学医院门诊风湿病诊所从发病起进行了16至24个月的前瞻性随访。另一名1988年患有与L-色氨酸相关的EMS的患者接受了30个月的随访。1989年队列中的28名幸存者中有93%仍有影响1至4个器官系统(中位数为3个)的症状,3人死亡,因此该疾病会导致相当高的发病率和死亡率。最常与长期残疾相关的慢性后遗症是硬皮病样皮肤增厚(54%)、感觉运动性多发性神经病(61%)、近端肌病(36%)和严重发作性肌痛(64%)。1例患者出现血小板减少症。HLA-II类分型显示与HLA-DR4存在无统计学意义的关联趋势。早期使用皮质类固醇治疗似乎无法预防慢性表现的发展。