OBJECTIVES : Idiopathic Multicentric Castleman’s disease (iMCD) is a rare disease that causes distressing and debilitating symptoms which have a negative impact on patients’ lives and may lead to life-threatening complications. This systematic review aimed to identify and quantify the humanistic burden of disease in adults with MCD who are not infected with HIV and HHV-8.

METHODS : Medline and Embase databases were searched in December 2018. There were no limits on publication language, but only studies from 2008 were included. Two reviewers screened articles against pre-defined criteria, a third reviewer resolved discrepancies. One analyst extracted data and another quality-controlled it. The DistillerSR Systematic Review Software was used in the studies selection process.

RESULTS : Five studies (four in the same cohort, all with treatment with siltuximab) were identified. Patient reported outcomes and health-related quality of life (HRQoL) were measured using the Multicentric Castleman Disease Symptom Scale (MCD-SS) (n=4), the SF-36 (n=3), and the FACIT-Fatigue questionnaire (n=2). MCD-SS outcomes reported most frequently were very mild and mild: shortness of breath (40%), lack of energy (33%), feeling weak (33%), tiredness (32%) and pain (32%). The FACIT questionnaire showed that at baseline, >50% patients reported lower fatigue scores than the normal population. In a comparative study, both siltuximab and placebo treated patients had comparable mean total FACIT score at baseline (32.4 vs 31), while siltuximab-treated patients reported much higher mean values than the patients on placebo during follow-up (38.6 vs 26.9, respectively). Treatment with siltuximab more often resulted in an SF-36 score equal to, or greater than the population norm at final visit compared to placebo. Generally, siltuximab was associated with better fatigue and depressive symptoms scores.

CONCLUSIONS : Data is limited, but MCD is associated with poor patient reported outcomes. Available data showed that siltuximab was associated with improvements in fatigue, functioning and well-being compared with placebo.