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Rheumatology (Oxford). 2021 Aug 2;60(8):3799-3808. doi: 10.1093/rheumatology/keaa863.
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Colchicine intolerance in FMF patients and primary obstacles for optimal dosing.FMF 患者对秋水仙碱不耐受和最佳剂量的主要障碍。
Turk J Med Sci. 2020 Aug 26;50(5):1337-1343. doi: 10.3906/sag-2001-261.
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Development and validation of a quality of life scale in Familial Mediterranean Fever (FMFQoL).家族性地中海热生活质量量表(FMFQoL)的开发与验证
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Classification criteria for autoinflammatory recurrent fevers.自身炎症性反复发作性发热的分类标准。
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Effect of interleukin-1 antagonists on the quality of life in familial Mediterranean fever patients.白细胞介素-1 拮抗剂对家族性地中海热患者生活质量的影响。
Clin Rheumatol. 2019 Apr;38(4):1125-1130. doi: 10.1007/s10067-018-4384-8. Epub 2018 Dec 10.

影响家族性地中海热患者可接受症状状态和治疗决策的因素。

Factors affecting patient-acceptable symptom states and treatment decision in familial Mediterranean fever.

机构信息

Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkey.

Department of Internal Medicine, Abdurrahman Yurtaslan Education and Research Hospital, Ankara, Turkey.

出版信息

Turk J Med Sci. 2022 Dec;52(6):1991-1996. doi: 10.55730/1300-0144.5547. Epub 2022 Dec 21.

DOI:10.55730/1300-0144.5547
PMID:36945975
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10390155/
Abstract

BACKGROUND

Familial Mediterranean fever [FMF] is the most common autoinflammatory disease characterized by inflammatory attacks of fever and polyserositis. Patients' quality of life is significantly affected due to recurrent excruciating pain attacks and complications. This study is performed to evaluate the parameters most affecting patients' satisfaction from treatment.

METHODS

: Three hundred and forty-six consecutive patients diagnosed with FMF were enrolled in this study. Current treatment, acute phase proteins, number, type, and severity of predominant attacks, absenteeism from work/school in the last three months were recorded, and the participants were asked whether they needed additional treatment to evaluate Patient Acceptable Symptom State (PASS) status.

RESULTS

Mean age of the overall group was 38.2 ± 11.7 years (62.4% female, 37.6% male). Two hundred and twenty-seven patients were treated with colchicine, 97 patients with colchicine plus Interleukin-1 (IL-1) antagonist, and 22 only with IL-1 antagonist (67.1%, 26.3%, 6.64% in order). Of the overall group, 33.8% (n = 117) believed to need additional treatment options. Additional treatment need of patients was significantly affected by work impairment due to attacks, absent days from work, disease activity, the discomfort of patients during attacks, the number of attacks, and treatment options; but not by the level of acute-phase proteins between attacks.

DISCUSSION

PASS score is significantly related to clinical parameters and quality of life. Patients' PASS scores and treatment choices are notably affected by the severity and frequency of attacks and absenteeism from work/school. Clinical activity and quality of life should be evaluated at every visit to provide patients' satisfaction with treatment.

摘要

背景

家族性地中海热(FMF)是最常见的自身炎症性疾病,其特征为发热和多浆膜炎的炎症发作。由于反复发作的剧痛发作和并发症,患者的生活质量受到显著影响。本研究旨在评估影响患者对治疗满意度的主要参数。

方法

本研究纳入了 346 例连续确诊的 FMF 患者。记录当前治疗、急性期蛋白、主要发作的次数、类型和严重程度、过去三个月的缺勤情况,并询问患者是否需要额外治疗以评估患者可接受症状状态(PASS)。

结果

总体组的平均年龄为 38.2±11.7 岁(62.4%为女性,37.6%为男性)。227 例患者接受秋水仙碱治疗,97 例患者接受秋水仙碱联合白细胞介素-1(IL-1)拮抗剂治疗,22 例患者仅接受 IL-1 拮抗剂治疗(分别为 67.1%、26.3%、6.64%)。总体组中,33.8%(n=117)认为需要额外的治疗选择。患者额外治疗需求显著受发作导致的工作障碍、缺勤天数、疾病活动度、发作时患者的不适程度、发作次数以及治疗选择的影响;但不受发作间期急性期蛋白水平的影响。

讨论

PASS 评分与临床参数和生活质量显著相关。患者的 PASS 评分和治疗选择明显受发作严重程度和频率以及缺勤情况的影响。应在每次就诊时评估临床活动和生活质量,以提高患者对治疗的满意度。