About Autoinflammatory Conditions
Autoinflammatory conditions are often caused by genetic faults that cause our innate immune system to produce cells which secrete types of cytokines called Interleukins. Cytokines play an important part in inflammation and immunity. However, in Autoinflammatory conditions, there is an over production of cytokines causing an hyper inflammatory response which can damage organs and joints. The most common type of interleukin cytokines involved in Autoinflammatory conditions are Interleukin – 1 alpha and Interleukin – 1 beta. The biological therapies (injections) used to treat Autoinflammatory conditions suppress the over production of cytokines (Interleukin 1), minimising inflammation and prevents damage to organs and joints. These are also known as Interleukin 1 blockers.
Glossary
The innate immune system: the first line of defence. The innate immune system is the first part of the body to detect invaders such as viruses, bacteria, parasites, and toxins, or to sense wounds or trauma. Upon detection of these agents or events, the innate immune system activates cells to attack and destroy the outsider, or to initiate repair, while also informing and modulating the adaptive immune response that follows this first line of defence. [Center for Innate Immunity and Immune Disease, 2024]
Cytokines are critical mediators that oversee and regulate immune and inflammatory responses via complex networks and serve as biomarkers for many diseases [British Society for Immunology, 2024].
Interleukins are a type of Cytokine. They are produced by many other body cells. Interleukins “play essential roles in the activation and differentiation of immune cells, as well as proliferation, maturation, migration, and adhesion.” [Justiz Vaillant AA, Qurie A. Interleukin. [Updated 2022 Aug 22]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024.
Hyper inflammatory means too much inflammation. “Inflammation is your body’s response to an illness, injury or something that doesn’t belong in your body (like germs or toxic chemicals). Inflammation is a normal and important process that allows your body to heal. Fever, for example, is how you know your body’s inflammatory system is working correctly when you’re ill. But inflammation can harm you if it occurs in healthy tissues or goes on for too long.” [Cleveland Clinic, 2024]
Biological Therapies. “Unlike medicines that are made through chemical processes, and often come in tablet or liquid form, biologics are medicines that are made from living material such as proteins and sugars.[1] These are the natural tools bodies use to carry out all the functions that are needed to thrive and be well. Because they are made from these natural materials, biologic medicines for immune-mediated conditions can be designed to target the part of the immune system that is not functioning as it should and block it from causing inflammation or injury to joints, skin and organs like the heart or kidneys.[2],[3] One of the key benefits of biologic medicines is that they can be precision targeted to the particular part of the immune system that is not working as it should.” [GlaxoSmithKline, 2024]
A list of Autoinflammatory Conditions [This is not an exhaustive list]
- Cryoprin Associated Periodic Fever Syndrome (CAPS / MWS / NOMID / CINCA)
- Familial Cold Associated Syndrome (FCAS)
- NLRP12-associated autoinflammatory disorder (FCAS2)
- Mevalonate Kinase Deficiency
- Familial Mediterranean Fever (FMF)
- TNF Receptor Associated Periodic Fever Syndrome (TRAPS)
- Blau Syndrome
- Yao Syndrome
- Early Onset Sarcoidosis
- Majeed Syndrome
- Deficiency of Interleukin One Receptor Antagonist (DIRA)
- Deficiency of Adenosine Deaminase 2 (DADA2)
- PLCG2 associated antibody deficiency and immune dysregulation (PLAID/APLAID Syndrome)
- Pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA Syndrome)
- Pyrin associated autoinflammation with neutrophilic dermatosis (PAAND)
- STING-associated vasculopathy with onset in infancy (STING)
- A20 Haploinsufficiency (HA20)
- Schnitzler Syndrome
- Recurrent Idiopathic Pericarditis
- Behçet’s Disease (Visit Behcets UK)
- Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA)
- Adult Onset Still’s Disease (AOSD) [Visit International foundation for Autoimmune and Autoinflammatory Arthritis (IFAA)]
- Systemic juvenile idiopathic arthritis (sJIA) or Still’s disease
- PANS is an acronym for Paediatric Acute-onset Neuropsychiatric Syndrome which encompasses PANDAS (Paediatric Autoimmune Neuropsychiatric Disease Associated with Strep)
- Chronic recurrent multifocal osteomyelitis/ chronic nonbacterial osteomyelitis (CRMO/CNO)
- SAPHO Syndrome
- VEXAS Syndrome