Hajialilo Mehrzad, Ghorbanihaghjo Amir, Khabbazi Alireza, Kolahi Sousan, Jafari Nakhjavani Mohammad Reza, Ebrahimi Ali Asghar, Zareh Hamid, Malek Mahdavi Aida
Connective Tissue Diseases Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.
Biotechnology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.
Int J Rheum Dis. 2018 Aug;21(8):1627-1633. doi: 10.1111/1756-185X.13352.
This study aimed to follow up patients with polymyositis (PM) and/or dermatomyositis (DM) to determine survival rate, pattern of disease, response to treatment, malignancy incidence and poor prognostic factors (PPFs).
A total of 76 patients with PM (n = 47) and/or DM (n = 29) based on Bohan and Peter diagnostic criteria referred to the Imam-Reza Hospital were followed up from 2004 to 2016. The follow-up period was considered from diagnosis to patient's death or last visit. All patients underwent physical examinations and data including age, sex, disease duration, disease subtype, pattern of disease, PPFs and malignancy incidence were collected.
Mean age at diagnosis was 45.49 ± 10.88 years and women were predominant (84.2%). Course of disease in the majority of patients (52.6%) was polyphasic, followed by monophasic (31.6%) and chronic-progressive (5.3%). The 1-, 5- and 10-year survival rates were 96%, 93% and 92%, respectively. Delay in treatment and dysphagia were common PPFs in the present study. The majority of patients responded to treatment (88.2%) and there were significant differences in cancer and dysphagia between responders and non-responders to treatment (P < 0.05). The most common cause of death was cancer in four of eight deaths. There was significant difference in survival rates between patients with and without pulmonary involvement (P = 0.001). Moreover, the survival rates were significantly lower in patients with malignancy (P = 0.001).
Presence of dysphagia and cancer were associated with poor response to treatment. Pulmonary involvement and cancer incidence significantly affect survival rate. Furthermore, since cancer is the most common cause of death, so this study emphasizes the importance of careful cancer screening in these patients.
本研究旨在对多发性肌炎（PM）和/或皮肌炎（DM）患者进行随访，以确定其生存率、疾病模式、对治疗的反应、恶性肿瘤发生率及不良预后因素（PPF）。
根据Bohan和Peter诊断标准，2004年至2016年期间对转诊至伊玛目-礼萨医院的76例PM（n = 47）和/或DM（n = 29）患者进行了随访。随访期从诊断至患者死亡或最后一次就诊。所有患者均接受了体格检查，并收集了包括年龄、性别、病程、疾病亚型、疾病模式、PPF和恶性肿瘤发生率等数据。
诊断时的平均年龄为45.49±10.88岁，女性占多数（84.2%）。大多数患者（52.6%）的病程为多相性，其次是单相性（31.6%）和慢性进行性（5.3%）。1年、5年和10年生存率分别为96%、93%和92%。治疗延迟和吞咽困难是本研究中常见的PPF。大多数患者对治疗有反应（88.2%），治疗反应者和无反应者在癌症和吞咽困难方面存在显著差异（P < 0.05）。8例死亡患者中有4例的最常见死因是癌症。有肺部受累和无肺部受累患者的生存率存在显著差异（P = 0.001）。此外，恶性肿瘤患者的生存率显著较低（P = 0.001）。
吞咽困难和癌症的存在与治疗反应不佳相关。肺部受累和癌症发生率显著影响生存率。此外，由于癌症是最常见的死因，因此本研究强调了对这些患者进行仔细癌症筛查的重要性。
