Interleukin-1 and autoinflammatory diseases
The interleukin 1 (IL-1) family is a group of proinflammatory cytokines that play a central role in regulating the body's immune response. By binding to the IL-1 receptors on cells, they play a major role in acute and chronic inflammatory reactions.
In addition, the IL-1 system is also involved in several other biological functions, such as metabolic and haematopoietic activities. Members of the IL-1 family have emerged as therapeutic targets for an expanding number of autoinflammatory diseases where inhibition of IL-1 activity may form the basis for novel treatments.
IL-1 is an important factor in Still’s disease, a rare, systemic autoinflammatory disease characterised by high fevers, joint pain and a rash. In rheumatoid arthritis patients, IL-1 is also elevated and correlates with various parameters of disease activity.
Familial Mediterranean Fever (FMF) is a rare, hereditary autoinflammatory disease. It is characterised by recurrent fever attacks and pain and is therefore assigned to the periodic fever syndromes. Those affected usually have a change in the genetic information in the so-called MEVF (Mediterranean Fever) gene, which leads to uncontrolled inflammation in the body. In about 90% of those affected, disease occurs before the age of 20, in 75% before the age of 10. Typically, an FMF episode lasts between one and three days.
CAPS (cryopyrin-associated periodic syndromes), a group of rare and potentially fatal autoinflammatory conditions, are characterised by excessive production of the protein interleukin 1ß (IL-1ß). Common symptoms include rash, periodic fevers, headaches, malaise and joint pain. The most severe form of CAPS is known as NOMID (neonatal-onset multisystem inflammatory disease) or CINCA (chronic infantile neurological, cutaneous and articular syndrome).
Rheumatoid Arthritis (RA) is the most common inflammatory disease of the joints with a chronic-progressive course. The causes and triggers and rheumatoid arthritis are diverse and not yet fully understood. Due to a disturbance of immune regulation and an overproduction of proinflammatory cytokines, the joints become inflamed, swell, and become sensitive to pressure. The cartilage cells in the joints are increasingly destroyed.