The COVID-19 pandemic and ANCA-associated vasculitis – reports from the EUVAS meeting and EUVAS education forum

Highlights

• A high proportion of patients with ANCA-associated vasculitis and COVID-19 have a severe disease course with an excess fatality rate.

• Antibody response to COVID-19 vaccination is impaired in most patients receiving immunosuppression, and absent in most patients with a recent dose of rituximab.

• Antibodies should be regularly monitored, and booster doses may be considered in those with low antibody titres, as neutralisation titres are per se reduced against emerging variants of concern and protection against COVID-19 directly relates to the level of antibody titres.

• Patients should continue social distancing measures and vaccination of close contacts is of importance.

Abstract

The Coronavirus Disease 2019 (COVID-19) pandemic influenced the management of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. A paucity of data exists on outcome of patients with vasculitis following COVID-19, but mortality is higher than in the general population and comparable to patients undergoing haemodialysis or kidney transplant recipients (reported mortality rates of 20–25%). Delays in diagnosis have been reported, which are associated with sequelae such as dialysis-dependency. Management of ANCA-associated vasculitis has not changed with the aim to suppress disease activity and reduce burden of disease. The use of rituximab, an important and widely used agent, is associated with a more severe hospital course of COVID-19 and absence of antibodies following severe acute respiratory syndrome (SARS)-CoV-2 infections, which prone patients to re-infection. Reports on vaccine antibody response are scarce at the moment, but preliminary findings point towards an impaired immune response, especially when patients receive rituximab as part of their treatment. Seropositivity was reported in less than 20% of patients when rituximab was administered within the prior six months, and the antibody response correlated with CD19⁺ B-cell repopulation. A delay in maintenance doses, if disease activity allows, has been suggested using a CD19⁺ B-cell guided strategy. Other immunosuppressive measures, which are used in ANCA-associated vasculitis, also impair humoral and cellular vaccine responses. Regular measurements of vaccine response or a healthcare-policy time-based strategy are indicated to provide additional doses (“booster”) of COVID-19 vaccines. This review summarizes a recent educational forum and a recent virtual meeting of the European Vasculitis Society (EUVAS) focusing on COVID-19.