What is inflammation?
Inflammation is the body's normal, protective response to injuries or infections. It is triggered by the immune system, which is a cellular system within the body. The immune system monitors for injuries to the body and detects “intruders” such as bacteria and viruses. If the immune system detects an injury or intruder, it mounts a response called an inflammatory response and signals cells to the site of injury or infection.
You may hear the words infection and inflammation together, but they mean very different things. Infection refers to the invasion and multiplication of bacteria or viruses within the body, while inflammation is the body's protective response against infection.
Inflammation is a complex cellular process involving various types of immune cells, clotting proteins and signaling molecules. It can occur after an injury, such as a burn or a cut, and also when there is an infection present in the body. But, inflammation can sometimes also occur without an injury or infection.
What is auto-inflammation?
Sometimes, the immune system can over-react and can cause inflammation by attacking healthy tissues within the body. This process is called ‘auto-inflammation’. When auto-inflammation occurs, there is a dysfunction within the immune system that triggers the body to mount an inflammatory response against itself. It is not known why this occurs, but it is thought that a virus or another trigger in the environment may be the initial cause.
Signs of inflammation after injury or infection
When an injury occurs, the cells of our immune system immediately travel to the site of injury or irritation and the inflammatory response begins. This includes widening of local blood vessels to allow fluid and immune cells into surrounding injured tissue, which causes swelling, redness, warmth and pain at the site. This process protects the injured area and signals other cells to the site to begin repairing and healing the injury. Normally, inflammation slowly goes away after the irritation has been removed and the body is adequately protected, as can be seen with the example of the splinter in the skin.
Signs of inflammation during auto-inflammation
During auto-inflammation, cells of the immune system also travel to certain sites in the body. However, there are no injuries or infections at these sites. Instead of repairing and healing, the auto-inflammatory response often ends up harming healthy tissues.
Auto-inflammation can cause damage and destruction to the body tissues or organs that it affects. This can be seen with central nervous system (CNS) inflammatory diseases, where auto-inflammation causes dysfunction in the brain, spine and/or optic (eye) nerves. An auto-inflammatory event can be a one-time occurrence or it may develop into a chronic (long-term) issue.
What causes auto-inflammation?
The exact cause of auto-inflammatory conditions is not known. A dysfunction of the immune system causes auto-inflammation, but it is unknown why this dysfunction occurs. Various theories include exposure to a virus, possible environmental triggers, and genetics.
Tools of our immune system
The immune system is a cellular system within the body. White blood cells, also known as leukocytes, make up a large part of the cells of the immune system. White blood cells help fight infection by attacking invaders and consuming infected or dead cells. The body has various types of white blood cells, all of which are produced in the bone marrow (the soft middle part of our bones). People with auto-inflammatory conditions may have higher levels of white blood cells in their blood, or they may have white blood cells present where white blood cells are not normally present.
How is an auto-inflammatory condition diagnosed?
If your child has symptoms of auto-inflammation or a suspected auto-inflammatory disease, several tests may be completed including a physical exam, imaging tests such as magnetic resonance imaging (MRI), a biopsy, a lumbar puncture and other tests including blood, urine or stool tests. Your child’s health-care team will review the results of the testing with you.
Treatment of auto-inflammatory conditions
Children with auto-inflammatory diseases are typically treated with immune-modifying therapies or immunosuppressant treatments. These are medications that are given to suppress (turn off) the abnormal immune response. Immunosuppressant medications may be given in pill form, injection or by intravenous (IV) infusion. Your child’s health-care team will recommend specific medication(s) and/or treatments for your child.
- It is important that you child follow the treatment regime recommended to treat their auto-inflammatory condition. If your child develops side effects of the treatments, their health-care team should be contacted.
- Your child may be more at risk for infections when they are treated with immunosuppressant medications. If your child develops a fever or symptoms of an infection, their health-care team should be contacted.
- The effectiveness of vaccines can also be influenced by immune-modifying medications. It is important to talk to your child’s health-care team if your child requires any immunizations.
How to prevent complications
Active symptoms of your child’s auto-inflammatory disorder can be minimized by following the plan recommended by your child’s health-care team. This may include giving your child immunosuppressive medications, performing regular blood testing and imaging, and making sure your child is regularly attending their medical appointments. Providing your child with a healthy balanced diet, adequate sleep and regular physical activity can also reduce the risk of flare-ups and relapses.
Recurrences and relapses
Children with auto-inflammatory conditions may have periods where they have no symptoms of the illness. If your child develops similar or new symptoms after treatment, your child’s health-care team should be contacted.
Helping your child
After your child has been diagnosed with an auto-inflammatory disorder, they may experience anger, sadness or anxiety. Receiving support or counselling many be helpful. A social worker or counselor can assist your child and family with coping with their diagnosis.
Your child may require additional assistance at home with their activities of daily living or may require additional support at school. Resources and supports can be put in place in the home or school as needed.
The health-care team
Your child will be closely monitored by their health-care team, including doctors, nurse practitioners, nurses, social workers, occupational therapists, physiotherapists, dieticians and child life specialists. Your child may also be followed by other specialty teams such as ophthalmology, neurology, immunology or rheumatology.
Clinic visits
It is important to have your child regularly monitored, even after treatment has finished and your child is feeling healthy. Your child may develop complications that are only mildly symptomatic or absent (asymptomatic) that still require treatment. The health team will also need to monitor the potential effects of treatments by completing regular blood testing, imaging or other tests.
At many paediatric hospitals, a child may continue to be followed by a paediatric specialist until they are 18 years of age. When they turn 18, they will need adult care. Your child's specialist will be able to tell you what to expect in terms of visits and how to transition to adult care.
When to seek medical attention
Contact your child's health-care team, call 911 or go to the nearest Emergency Department right away if your child experiences any of the following:
- A fever
- A seizure
- Changes in their level of consciousness
If your child develops sudden symptoms of their auto-inflammatory illness, please contact their health-care team.