目的:了解中国系统性硬化症(systemic sclerosis,SSc)治疗的现状.方法:通过向风湿科和皮肤科医师发放电子问卷,收集有关SSc治疗的信息.结果:286名医师完成了问卷.74.5％的受访者在SSc治疗中使用了肾上腺糖皮质激素(glucocorticoid,GC),并支持低剂量长期维持GC使用.75.5％的受访者将环磷酰胺(cyclophosphamide,CTX)作为皮肤累及和间质性肺病(interstitial lung disease,ILD)的一线免疫抑制剂,而甲氨蝶呤(methotrexate,MTX)则被33.6％的受访者选择.38.8％受访者将静脉注射免疫球蛋白(intravenous immunoglobulin,IVIG)作为SSc-ILD中最常用的免疫疗法.13.6％的受访者将抗白介素-6(interleukin 6,IL-6)受体抗体作为SSc-ILD中生物制剂的首选.约半数(47.9％～61.2％)受访者使用靶向药物磷酸二酯酶-5抑制剂、内皮素受体拮抗剂作为一线或联合治疗药物来治疗指/趾端血管病变和肺动脉高压(pulmonary arterial hypertension,PAH).此外,有31.5％的受访者曾尝试使用吡非尼酮治疗SSc-ILD.造血干细胞移植在皮肤受累、SSc-ILD和指/趾端血管病变方面进行了尝试,但在胃肠道(gastrointestinal,GI)受累(1.4％)和SSc-PAH(1.0％)方面尝试较少.受访者的背景对SSc-ILD、SSc-PAH、指/趾端血管病变和皮肤受累的一线免疫抑制剂的选择产生了影响,但对长期GC维持影响较小,职称比科别影响更大.结论:除了使用GC外,免疫抑制剂和靶向药物被广泛用于治疗SSc;并在难治性病例中尝试了生物制剂、IVIG和造血干细胞移植.风湿科和皮肤科医师在药物选择上存在差异.

皮肌炎(dermatomyositis,DM)是一种以累及横纹肌为主,同时又有皮肤病变的结缔组织病(connective tissue disease,CTD).肺间质病变(interstitial lung disease,ILD)是其常见并发症,部分患者肺部病变进展迅速,目前尚无有效的药物,病死率高.吡非尼酮是新型抗纤维化小分子化合物,被批准用于治疗特发性肺纤维化(idiopathic pulmonary fibrosis,IPF),疗效较好.国外已有应用吡非尼酮治疗CTD-ILD的个例报道,但国内尚无报道.本例DM-ILD患者应用吡非尼酮治疗9个月余,取得良好疗效,现报告如下.

OBJECTIVE:To discuss the diagnostic value of cardiac enzyme and troponin in acute organophosphorus pesticide poisoning (AOPP).METHODS:A retrospective study was performed in the document published in domestic journals and PubMed from 1979 to 2010. The data of the cardiac enzyme and troponin were collected. Statistical analysis was conducted with one-way ANOVA and rank sum test. 2129 cases with AOPP were enrolled.RESULTS:The levels of creatine kinase (CK), creatine kinase-MB (CK-MB) and cardiac troponin I (cTnI) in milder, moderate and severe poisoning groups were significantly elevated compared by the healthy control group (P < 0.05). The differences were also dramatic among three patients groups (P < 0.05). The ratios of CK-MB to CK in both moderate and severe groups were significantly lower than in healthy controls (P < 0.05). The levels of CK, CK-MB and cTnI were higher especially in patients with intermediate myasthenic syndrome (IMS) than patients without IMS. Meanwhile, the levels of CK and CK-MB were elevated in patients with respiratory failure compared by non-failure ones, but decreased in the ratios of CK-MB to CK (P < 0.05).CONCLUSIONS:The elevation of CK and CK-MB in serum could not be judged as the criteria of myocardial damage in AOPP, the ratio of CK-MB to CK were more valuable; the value of cTnI in myocardial damage was still in suspect. CK, CK-MB and cTnI could be used as auxiliary criteria of AOPP classification.