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Auto-inflammatory

  • Article
  • 2021-01-29

Your immune system is the network of cells and tissues throughout your body that work together to protect you from viruses, bacteria and infections. It tries to identify, kill and eliminate the intruders that could hurt you.

Parts of the immune system include:

  • Acquired (or adaptive) immune system, which develops as you grow. Intruders provoke your body to produce antibodies so that your body 'remembers' those intruders. Your body can then fight them when they return.
  • Innate (or innate) immune system, which uses white blood cells instead of antibodies to destroy invaders.

What happens in auto-inflammatory conditions?

Auto-inflammatory diseases refer to problems with the responses of the innate immune system. Immune cells accidentally target the body's own healthy tissues and signal the body to attack them. This can cause intense attacks of inflammation resulting in symptoms such as fever, rash or swollen joints. These diseases also carry the risk of amyloidosis, a potentially fatal build-up of blood proteins in vital organs.

Types

Some examples of auto-inflammatory conditions and their symptoms are:

  • Familial Mediterranean fever (FMF), which causes recurrent attacks of fever. Other symptoms include:
    • Severe abdominal pain due to inflammation of the stomach cavity (peritonitis).
    • Arthritis (painful, swollen joints).
    • Chest pain due to inflammation of the lung cavity (pleurisy).
    • Skin rash.
  • Neonatal Onset Multisystem Inflammatory Disease (NOMID) affects the skin, joints, eyes and the central nervous system. For most children, the first sign of the disease is a rash that develops within the first six weeks of life. Other problems can follow, including:
    • Fever.
    • Meningitis.
    • Joint damage.
    • Loss of sight.
    • Hearing loss.
    • Mental retardation.
  • Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS) is associated with:
    • Long, dramatic episodes of high fever.
    • Severe pain in the abdomen, chest or joints.
    • Skin rash.
    • Inflammation in or around the eyes.
  • Deficiency of the interleukin-1 receptor blocker (DIRA) can cause the following serious conditions in children:
    • Swelling of bone tissue.
    • Bone pain and deformity.
    • Inflammation of the connective tissue layer around bone.
    • Skin rash that can cover most of the body.
  • Behçet's disease can cause the following symptoms:
    • Mouth or genital sores.
    • Redness and swelling in the eyes.
    • Arthritis.
    • Skin problems.
    • Swelling of the digestive system, brain and spinal cord.
  • Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) may include the following symptoms, which generally develop during the first year of life:
    • Recurring fever.
    • Purpura.
    • Joint pain.
    • Contractures.
    • Developmental delay.
    • Facial changes, including loss of facial fat, and swollen lips and eyelids.

Therapy

Treatments for autoinflammatory disease can include medications that:

  • Reduce inflammation.
  • Suppress the immune system.


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