Abouyahya Imane, Liem Sophie I E, Amoura Zahir, Fonseca João Eurico, Chaigne Benjamin, Cutolo Maurizio, Doria Andrea, Fischer-Betz Rebecca, Guimaraes Vera, Huizinga Thomas W J, van Laar Jacob M, Martin Thierry, Matucci-Cerinic Marco, Montecucco Carlomaurizio, Schneider Matthias, Smith Vanessa, Müller-Ladner Ulf, de Vries-Bouwstra Jeska K
Department of Rheumatology, Leiden University Medical Center, The Netherlands.
Service de Médecine Interne, Centre de Référence Maladies Autoimmunes Systémiques Rares d'Ile de France, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP) APHP-CUP, Hôpital Cochin, Université de Paris, France.
Clin Exp Rheumatol. 2022 May;40 Suppl 134(5):66-70. doi: 10.55563/clinexprheumatol/x5aras. Epub 2022 May 2.
Health-Related Quality of Life (HRQoL) in adult patients with mixed connective tissue disease (MCTD) has not been described so far. Therefore, we performed an explorative study to evaluate HRQoL in MCTD patients.
MCTD patients fulfilling the Kahn criteria and participating in the prospective follow-up cohort for MCTD of the Leiden University Medical Center were included; and matched to systemic sclerosis (SSc) patients based on age, sex and disease duration. Data on disease characteristics and HRQoL (SF36 and EQ-5D) were collected annually. HRQoL was compared between MCTD and SSc patients at baseline. Factors associated with HRQoL in MCTD were identified using linear regression and change in HRQoL over 3 years using linear mixed models.
Thirty-four MCTD patients (121 visits) and 102 SSc patients (424 visits) were included. At baseline, MCTD patients presented with interstitial lung disease, cardiac involvement, synovitis and myositis more frequently compared to SSc patients, while use of immunosuppressive medication was less frequent. In both groups, mean SF36 scores were lower than in the general Dutch population. The SF36 subscore "general health perception" was impacted most in both groups (MCTD: 38.5 [SD:7.0], SSc: 39.9 [SD:8.9]). During follow-up, SF36 scores improved in MCTD patients, while EQ5DNL remained stable. No specific characteristics were identified that associated with baseline HRQoL or change in HRQol over time.
Like in SSc, HRQoL in MCTD is significantly impaired, especially the general health perception of patients. Evaluation in larger prospective cohorts is needed to identify characteristics that impact HRQol most.
迄今为止，尚未对成年混合性结缔组织病（MCTD）患者的健康相关生活质量（HRQoL）进行描述。因此，我们开展了一项探索性研究，以评估MCTD患者的HRQoL。
纳入符合卡恩标准并参与莱顿大学医学中心MCTD前瞻性随访队列的MCTD患者；并根据年龄、性别和病程与系统性硬化症（SSc）患者进行匹配。每年收集疾病特征和HRQoL（SF36和EQ-5D）数据。在基线时比较MCTD和SSc患者的HRQoL。使用线性回归确定与MCTD患者HRQoL相关的因素，并使用线性混合模型确定3年内HRQoL的变化。
纳入了34例MCTD患者（121次就诊）和102例SSc患者（424次就诊）。在基线时，与SSc患者相比，MCTD患者更常出现间质性肺疾病、心脏受累、滑膜炎和肌炎，而免疫抑制药物的使用频率较低。在两组中，平均SF36评分均低于荷兰普通人群。两组中，SF36子评分“总体健康感知”受影响最大（MCTD：38.5[标准差：7.0]，SSc：39.9[标准差：8.9]）。在随访期间，MCTD患者的SF36评分有所改善，而EQ5DNL保持稳定。未发现与基线HRQoL或随时间变化的HRQol相关的特定特征。
与SSc一样，MCTD患者的HRQoL明显受损，尤其是患者的总体健康感知。需要在更大的前瞻性队列中进行评估，以确定对HRQol影响最大的特征。
