<p>Immune checkpoint inhibitors (ICI) lead to immune-mediated toxicity, so-called immune-related adverse events (irAE) in nearly all patients treated. These also include exacerbations of pre-existing autoimmune diseases. This update focuses on the evidence for and the management of rheumatic irAEs, which occur in patients with an underlying rheumatic disease. Despite the existing gaps in evidence and knowledge, it is highlighted that in rheumatological patients a thorough interdisciplinary consideration and planning of the baseline and irAE medications can be decisive for the outcome. From the current literature and also own experience in the MalheuR project, it can be derived that in general ICI treatment can be carried out even in patients with pre-existing rheumatic diseases with acceptable safety and good efficacy. The present update and the advisory service of the MalheuR project aim to increase the number of patients with pre-existing rheumatological diseases who receive the possibility of a (successful) ICI treatment in an interdisciplinary setting.</p>