Systemic Vasculitis Associated With Immune Check Point Inhibition: Analysis and Review

Immunotherapy with immune checkpoint inhibitors (ICIs) has become a well-established modality to treat a number of different malignancies, especially in cases with advanced stages and/or recurrent diseases. These agents have been associated with development of a variety of autoimmune disorders as immune-related adverse events (IRAEs or irAEs). This review focuses on development of vasculitis with use of ICI.

Available information on vasculitis associated with immune checkpoint inhibition is limited primarily to case reports at this time. Most immune-related adverse events will not present as vasculitis, and it is an uncommon manifestation and/or is under-reported. There are no current well-established guidelines for treating vasculitis associated with ICIs; initial management would usually start with consideration of discontinuing the ICI and administering corticosteroids. Collaboration between treating oncologists and rheumatologists is necessary for a combined approach to management. While arthralgias, myalgias, and inflammatory arthritis frequently occur as irAEs, vasculitis is an uncommon presentation. Vasculitis has been reported with all of the available ICI agents, and there seems to be no clear difference in the risk based on small numbers. Large vessel vasculitis and vasculitis of the nervous system were the most commonly reported types of vasculitis but cases of vasculitis involving medium and small vessels have also been reported. It is challenging to know if the underlying disease or ICIs are the main culprit in development of vasculitis and requires a collaborative relationship between the treating oncologist and rheumatologist. Except in very mild cases, development of vasculitis during ICI therapy requires temporary or permanent discontinuation of ICI.