From the Guidelines
Autoimmune diseases are characterized by an adaptive immune response against self-tissues, whereas autoinflammatory diseases result from an innate immune response dysregulation, leading to inappropriate inflammation without autoantibodies or antigen-specific T cells. The key difference between autoimmune and autoinflammatory diseases lies in their underlying immune system mechanisms. Autoimmune diseases, such as rheumatoid arthritis and systemic lupus erythematosus, involve the adaptive immune system and are often associated with specific autoantibodies and genetic links to HLA haplotypes 1. In contrast, autoinflammatory diseases, including familial Mediterranean fever, are related to the innate immune system and typically present with recurrent episodes of fever and inflammation, often having a strong genetic component related to mutations in genes regulating innate immunity and inflammasome function. Some of the main characteristics of autoinflammatory diseases include:
- Dysregulation of the innate immune system
- Inappropriate inflammation without evidence of autoantibodies or antigen-specific T cells
- Recurrent episodes of fever and inflammation
- Strong genetic component related to mutations in genes regulating innate immunity and inflammasome function
- Response to therapies targeting specific inflammatory cytokines, such as IL-1 inhibitors like anakinra or canakinumab In the context of familial Mediterranean fever, a common autoinflammatory disease, management with appropriate medications and monitoring can effectively control the condition, as highlighted in the EULAR recommendations 1. Understanding the distinction between autoimmune and autoinflammatory diseases is essential for accurate diagnosis and appropriate management, as treatment approaches differ significantly between the two.
From the FDA Drug Label
The levels of the naturally occurring IL-1Ra in synovium and synovial fluid from RA patients are not sufficient to compete with the elevated amount of locally produced IL-1 Spontaneous mutations in the CIAS1/NLRP3 gene have been identified in a majority of patients with cryopyrin-associated periodic syndromes such as NOMID. DIRA is an autosomal recessive monogenic autoinflammatory disease caused by mutations in the IL1RNgene leading to loss of secretion of the interleukin-1 receptor antagonist (IL-1Ra).
The main difference between autoimmune and autoinflammatory diseases is:
- Autoimmune diseases, such as rheumatoid arthritis (RA), are related to immune system disorders, where the immune system attacks the body's own tissues.
- Autoinflammatory diseases, such as NOMID and DIRA, are related to inflammatory disorders, where there is an abnormal regulation of the inflammatory response, often due to genetic mutations affecting the production or function of inflammatory mediators, such as IL-1Ra 2.
From the Research
Definition and Characteristics
- Autoimmune diseases are related to immune system disorders, involving the activation of T and B cells, and are associated with type I interferon (IFN) signatures in blood 3.
- Autoinflammatory diseases, on the other hand, are related to inflammatory disorders, driven by endogenous danger signals, metabolic mediators, and cytokines, and are mostly driven by inflammasome-induced IL-1β and IL-18 production 3.
Disease Mechanisms
- Autoinflammatory diseases are characterized by recurrent systemic inflammation due to dysfunction of the innate immune system, whereas autoimmune diseases involve adaptive immune responses 4, 5.
- The efficacy of biologic agents directed against proinflammatory cytokines, such as IL-1β and TNF, also highlights differences between autoinflammatory and autoimmune processes 3.
Therapeutic Responses
- Autoinflammatory diseases can be treated with biologic agents that inhibit IL-1β, such as canakinumab, whereas autoimmune diseases may require different treatment strategies 4.
- A better understanding of the pathogenic pathways of autoinflammation and autoimmunity can help design targeted treatment strategies for both types of diseases 6.
Spectrum of Diseases
- Rheumatic and musculoskeletal diseases can be placed along a spectrum, with autoinflammatory diseases and autoimmune diseases representing the two ends of this spectrum 6.
- Some diseases, such as spondyloarthritis and rheumatoid arthritis, can exhibit a mixed pattern of autoinflammation and autoimmunity 6, 7.