Efficacy and safety of regimens used for the treatment of multicentric Castleman disease: A systematic review. (13th July 2022) - Rehman, Mohammad Ebad Ur
Abstract: Objectives: Treatment options for multicentric Castleman disease (MCD) remain limited. The only FDA‐approved drug is siltuximab for idiopathic MCD (iMCD), but the response rate with siltuximab is less than 50%. We performed a systematic review to examine the efficacy and safety of various regimens used for the treatment of MCD. Methods: A database search on PubMed, Embase, Cochrane, Web of Science, and Clinicaltrials.gov using the terms "Castleman disease, " "treatment outcome, " and "patient safety" was done. Results and Conclusions: Results from a randomized controlled trial and an extension study highlighted the efficacy and long‐term safety of siltuximab for iMCD; other trials showed tocilizumab to be a suitable alternative. A recent trial reported high response rates with thalidomide in iMCD patients. Promising results were reported for bortezomib in relapsed/ refractory MCD. For human herpesvirus‐8 (HHV8)‐associated MCD, rituximab along with doxorubicin therapy followed by maintenance with zidovudine and valganciclovir is the most effective therapy. A single‐arm trial has highlighted the potential role of tocilizumab in HHV8‐MCD. Data for these regimens are limited and mostly comprise nonrandomized trials. Further research on emerging agents could have a major impact on the treatment of this rare disease.