Clinical Characteristic and Quality of Life Assessment in Adulthood of Patients With Cryopyrin Associated Periodic Syndrome
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 发起方
- 入组人数
- 54
- 试验地点
- 1
- 主要终点
- Clinical characteristics of patients with CAPS in adulthood
研究概览
简要总结
The patients included in this study were followed up in the Internal Medicine and Paediatrics Departments of the Lille CHU, the Paediatric Rheumatology and Immunology Department of the Necker Enfant Malade Hospital in Paris and the Paediatric Rheumatology Department and Internal Medicine of the Bicêtre Hospital in Paris. All patients selected presented one of the 3 CAPS clinical phenotypes (CINCA/NOMID, Muckle-Wells or Cold Urticaria). The mutation and the determination of the variant had to be confirmed by genetic analysis.
Patient data were collected from their medical records, retrospectively. Data collected concern childhood period from appearance of symptoms, adulthood period, in the last year and patients' way of life and quality of life upon the assessment. In addition, we collected demographic data related to the patients' lifestyle (intoxications, living arrangements, level of education) and we conducted individual telephone interviews lasting 15 minutes to complete a quality of life questionnaire including the SF36 questionnaire.
The study aimed to describe the clinical symptoms of patients in adulthood and to assess quality of life. We also wanted to compare the clinical phenotypes of patients according to their genetic variant.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Control
- 时间视角
- Retrospective
入排标准
- 年龄范围
- 16 Years 至 —（Child, Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Diagnosis of FCAS, Muckle-Wells or NOMID/CINCA;
- •age < 16 years at the apparition of the first symptoms of the disease;
- •age ≥ 16 years old at inclusion
- •follow-up in a CEREMAIA reference or competence center.
排除标准
- •Age < 16 years at inclusion;
- •non-consenting patient
结局指标
主要结局
Clinical characteristics of patients with CAPS in adulthood
时间窗: Baseline
次要结局
- Assessment of the quality of life of patients with CAPS in adulthood(Baseline)
- Compare the clinical status between childhood and adulthood(Baseline)
- Compare the clinical status of patients according to their genetic variant.(Baseline)