Quality of Life after Idiopathic Multicentric Castleman Disease in China: A Cross-Sectional, Multi-Center Survey of Patient Reported Outcome and Caregiver Reported Outcome
Authors: Chen Jia , Zhang Miaoyan , Gao Yuhan , Zhang Lu , Li Jian 
Published: December 2024

The majority of multicentric Castleman disease (MCD) patients in China are idiopathic MCD (iMCD) with systemic manifestations. Given the potentially high symptom burden in iMCD, accurately and reliably assessing the impact of reported symptom presentation on the physical and mental health of patients, as well as on their daily life and on the burden of caregiving, is of utmost importance. However, little is known about the quality of life, mental and psychological status, social function, and caregiving burden of iMCD patients.
Our study aims to comprehensively describe the disease burden in iMCD patients and caregivers by patient-reported outcome measurements (PROMs) and caregiver-reported outcome measurements (CROMs).
A cross-sectional web-based survey of iMCD patients and caregivers was conducted across China. PROMs were performed using four self-administered questionnaires (MCD-SS, SF-36, PHQ-9, and WPAI:GH). CROMs were performed using three questionnaires (SF-36, Zarit-22, CRA) to assess the caregiving burden. Correlation analysis was performed in patient-caregiver dyads.
A total of 178 iMCD patients and 82 caregivers who met the inclusion criteria filled in the informed consent and completed the online survey, including 42 patient-caregiver dyads. Interviewees were from various regions across China. In patients, the median number of symptoms reported by MCD-SS was 9 (range 1-16, indicating that the majority of patients reported multiple symptoms. The mean score of Fatigue domain was relatively high at 4.34 (SD 2.56). Physical and mental health measures by SF-36 in iMCD patients had a significant decline in the mean scores as compared with Chinese general population (p&lt;0.001). Compared to a year ago, 61.0% of iMCD patients reported improvement, 20.0% reported about the same, and 19.0% reported worsening of their health. Based on the PHQ-9 scores, approximately 65% of the patients exhibited depressive symptoms, and 28.6% of the patients might have mild to severe major depression. Of the 178 patients, only 84 (47.2%) reported that they were currently employed. Measured by WPAI:GH, 28.5% of the working time, 21.2% of the work efficiency, and 29.4% of the daily activities were impaired in iMCD patients due to their health problems. In caregivers, most SF-36 component scores were higher than those of iMCD patients but lower than those of the general population (p&lt;0.05). The overall caregiving burden was mild, while caregivers still reported prominent feeling of self-criticism (mean score 5.1, SD 2.3) and relatively high score in the lack of family support (mean 3.5, SD 0.4) and financial problems (mean 3.4, SD 0.5). Correlation analysis showed that in iMCD patients, prominent fatigue symptoms in exacerbate their physical and mental health and impaired their ability to work and carry out daily lives.
In paried analysis, forty-two patient-caregiver dyads were recruited for the study. Among them, the ratio of patients diagnosed with iMCD-TAFRO in patient-caregiver dyads was higher than in all individuals surveyed (16.7% vs 6.2%, p=0.026). Other demographic characteristics of the dyads were not significantly different with the whole group. Caregivers of iMCD-TAFRO patients had higher levels of family financial problems and psychometric burden (r: 0.31~0.55). Moderate correlations were found between caregivers with heavier caregiving burden and iMCD patients with heavier symptom burden, worse quality of life, or more depressive symptoms (correlation coefficient: 0.31~0.55).
Our study concluded that the fatigue and depressive symptoms prevalent in iMCD patients impact their life quality and social well-being. The disease also affect the physical and mental health of iMCD caregivers, leading to feelings of guilt and the sensation of a lack of family support. Further longitudinal studies are needed to assess the correlations between PROMs and the clinical response to treatment.