Evolving understanding of Kaposi sarcoma and associated diseases in the ART era.

Abstract

Kaposi sarcoma (KS)-associated herpesvirus (KSHV), otherwise known as HHV-8, causes KS and several lymphoproliferative disorders including KSHV-associated multicentric Castleman disease, KSHV-associated inflammatory cytokine syndrome, primary effusion lymphoma, and KSHV-positive diffuse large B-cell lymphoma not otherwise specified. KS represents a substantial burden of disease in sub-Saharan Africa and in people with HIV worldwide. Diagnosis relies on pathology, but quantitative KSHV DNA measurements can help identify multicentric Castleman disease and KSHV-associated inflammatory cytokine syndrome and monitor treatment response. Alongside antiretroviral therapy, a stage-stratified approach to KS treatment indicates systemic therapy for advanced disease or to mitigate the risk of the related immune reconstitution inflammatory syndrome. The latter condition constitutes a therapeutic challenge, as do multicentric Castleman disease and KSHV-associated inflammatory cytokine syndrome, which are likely to be underdiagnosed. Research on novel treatment approaches, such as immunomodulatory regimens, as well as diagnostic and monitoring strategies, is underway.