血管炎ジャーナル

Vasculitis is not an isolated condition but can occur in the context of systemic diseases. This article explores the intricate relationship between vasculitis and systemic diseases, highlighting the importance of a comprehensive approach to their diagnosis and management. Understanding these connections is crucial for healthcare professionals to provide appropriate care and improve patient outcomes. Several rheumatologic diseases, such as Systemic Lupus Erythematosus (SLE), Rheumatoid Arthritis (RA), and Sjögren's syndrome, can involve vasculitis as a manifestation. These systemic diseases have complex immune dysregulation mechanisms that can lead to vascular inflammation.

Vasculitis is a group of rare autoimmune diseases characterized by inflammation and damage to blood vessels. It occurs when the body's immune system mistakenly attacks healthy blood vessels, leading to a range of symptoms and complications. In this article, we will explore the underlying mechanisms of vasculitis, focusing on the immune system's role in initiating and perpetuating the disease. Understanding the immune system's attack on blood vessels is crucial for unraveling the complexities of vasculitis and developing targeted therapies to manage this condition effectively.

Background: Antibody-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a potentially life-threatening condition. Multiorgan involvement can occur and is associated with significant morbidity, particularly if there is lung and renal disease. Aggressive and early immunosuppressant therapy is the cornerstone of management and has transformed ANCA-associated vasculitis (AAV) into a chronic relapsing condition. Remission induction therapy typically consists of high dose glucocorticoids in combination with cyclophosphamide or rituximab or both for several months. Plasma exchange is recommended for life-threatening disease, although evidence is conflicting. Remission maintenance therapy is often continued for up to two years and usually consists of glucocorticoids, azathioprine or rituximab. Methotrexate and mycophenolate mofetil (MMF) can also be used. Rituximab has been the focus of clinical trials over the previous few years and has shown to be effective therapy for both remission induction and maintenance. There has also been a focus on reduction of glucocorticoids due to the risk of infections and other debilitating side-effects. There is an ongoing need for new therapies, particularly those with better safety-profiles than current medication. Avacopan, a C5a receptor inhibitor, has recently been approved by the European Medicines Agency and U.S Food and Drug Administration as a treatment option for AAV. The COVID-19 pandemic has added another layer of complexity to the management of AAV, and immunosuppression regimes have been altered especially with regards to timing of rituximab infusions, which reduces the effectiveness of the vaccines.

Summary: This review focuses on the management of AAV (GPA and MPA) and the key clinical trials are summarised.

Key messages: Immunosuppression regimes have been revised due to emerging evidence from clinical trials. Rituximab, avacopan and reduced-dose glucocorticoid schedules have been the focus of recent studies. The COVID-19 pandemic must be considered when determining immunosuppression regimes.