Rheumatische Manifestationen von Primären Immundefekten
摘要
Primary immunodeficiencies, also referred to as inborn errors of immunity, can present with rheumatic manifestations as a form of immune dysregulation. Both autoimmune and autoinflammatory processes can be involved. Identifying an underlying immunodeficiency is crucial for optimizing treatment, prophylaxis and monitoring of complications. Because immunodeficiencies are often diagnosed late in the context of rheumatic symptoms, atypical disease courses or additional clinical findings should always prompt consideration of an underlying immunological disorder.