Since immune checkpoint inhibitors became the standard of care for an increasing number of indications, more patients have been exposed to these drugs and physicians are more challenged with the management of a unique spectrum of immune-related adverse events (irAEs) associated with immune checkpoint inhibitors. Those irAEs of autoimmune or autoinflammatory origin, or both, can involve any organ or tissue, but most commonly affect the dermatological, gastrointestinal and endocrine systems. Rheumatic/systemic irAEs seem to be less frequent (although underreporting in clinical trials is probable), but information on their management is highly relevant given that they can persist longer than other irAEs. Their management consists of anti-inflammatory treatment including glucocorticoids, synthetic and biologic immunomodulatory/immunosuppressive drugs, symptomatic therapies as well as holding or, rarely, discontinuation of immune checkpoint inhibitors. Here, we summarize the management of rheumatic/systemic irAEs based on data from clinical trials but mainly from published case reports and series, contextualize them and propose perspectives for their treatment.