Does your child have frequent fevers? Could it be due to SAIDs (Systemic Autoinflammatory Diseases)?

Oh, does your little one get a fever frequently these days? Just when you think you've gotten better, does it flare up again? Sometimes the cause of this may not be a common virus or bacteria. Today we're going to talk about a condition that's a bit complicated, but it's very important to know about. This is called SAIDs, which stands for Systemic Autoinflammatory Diseases . In the past, this was called `(Periodic Fever Syndromes)` or `(Recurrent Fever Syndromes)`.

What are SAIDs? Let's understand it simply.

Simply put, SAIDs are a group of diseases that are caused by a malfunction or regulatory problem in our body's innate immune system . This is why a child can get frequent fevers even without an infection (such as a virus or bacteria).

The most important thing is that these conditions called SAIDs should not be confused with the `(Autoimmune Diseases)` (for example `(Rheumatoid Arthritis)` or `(Lupus)` that you may have heard of. In `(Autoimmune)` diseases, due to some error in our body's `(adaptive immune system)`, that system attacks its own healthy cells. But in SAIDs, something different happens.

SAIDs are much rarer than autoimmune diseases. They are often hereditary and caused by a genetic mutation .

Usually, but not always, SAID symptoms begin when a child is very young, either as an infant or toddler . Your child may have "periods" of fever and other symptoms with this condition. In between these periods, your child may have no symptoms at all. Although there is no cure for SAIDs, there are often treatments to help control your child's symptoms.

What are the main types of SAIDs?

Researchers have identified about 60 types of SAIDs, and new ones are still being discovered. Here are some of the most common types of SAIDs:

• Familial Mediterranean fever (FMF): This is the most common of the genetically inherited, recurrent fever syndromes. It can cause painful swelling of the abdomen, chest, and joints in children.
• Periodic fever, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): This usually starts in young children before the age of 4. Sometimes, the condition can get better on its own after the age of 10.
• Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): This can occur in childhood, adolescence, or even adulthood.
• Mevalonate kinase deficiency (MKD): Previously called ``Hyper-IgD syndrome'', this usually begins before the child is one year old.
• NLRP3-associated autoinflammatory diseases: This was previously called `(Cryopyrin-associated periodic syndromes - CAPS)`. There are three other sub-types of diseases within this.
• Adult-onset Still's disease (AOSD): As the name suggests, this is an adult-onset form of the childhood condition ``Systemic Juvenile Idiopathic Arthritis (JIA)``.
• NOD-2-associated granulomatous disease (Blau syndrome): This usually begins before the age of 4. It mainly affects the child's skin, eyes, and joints.

What are the symptoms of SAIDs?

Symptoms of SAIDs usually begin in childhood. The main and most common symptom is periodic fever . During the fever periods, people can usually be healthy and have no symptoms. However, there may be other symptoms that are specific to each type of SAID. Let's see what they are:

• FMF: This can cause swelling and severe pain in the child's abdomen, chest, and joints . There may also be a rash on the lower legs or ankles.
• PFAPA: This can cause a sore throat, mouth sores, and swollen lymph nodes in the neck . For example, if your child is showing symptoms like frequent tonsillitis and mouth ulcers, it could be PFAPA.
• TRAPS: This can cause chills and muscle pain in the trunk/torso and arms . It can also cause a painful red rash that starts on the arms and legs and spreads down the body.
• MKD: This can cause symptoms such as chills, headache, stomach ache, loss of appetite, and flu-like symptoms .
• NLRP3 diseases: This condition can cause skin rashes, headaches, malaise, joint pain, and pink eye/conjunctivitis .
• AOSD: This is a condition characterized by skin rashes, joint pain, and muscle pain . Some people may also experience sore throat, stomach aches, fatigue, and malaise.
• Blau syndrome: This can cause skin lesions on your baby's arms, legs, or midsection . It can also cause joint pain and eye pain .

What are the causes of SAIDs?

Many SAIDs are genetic diseases . That is, a specific mutation (variant) in a specific gene causes each syndrome. Imagine that our body has a set of instructions (genes) that tell us how to do each job, so if one letter in that book is wrong, it can cause the rest of the work to go wrong.

Here are the main types of SAIDs and the genes associated with them:

• FMF: The gene `MEFV`. This is what gives instructions on how to make the protein `(pyrin)`.
• PFAPA: The exact cause of this has not yet been determined .
• TRAPS: The gene `TNFRSF1A`. This instructs the production of a protein called `(tumor necrosis factor receptor - TNFR)`.
• MDK: The gene called `MVK`. This instructs the production of a protein called `(mevalonic kinase)`.
• NLRP3 diseases: The gene `NLRP3`. This instructs the production of a protein called `(cryopyrin)`.
• AOSD: The exact cause for this has not yet been found .
• Blau syndrome: The gene `NOD2`. This instructs the production of a protein called `(NOD2)`.

It's important to understand that when these are caused by genetic factors, it's not the parents' fault. These are very complex biological processes.

Complications caused by SAIDs

It is very important to get proper treatment for these conditions. Because, if inflammation continues in the body, it can lead to a condition called `(Amyloidosis). `(Amyloidosis)` is a protein deposit in the kidneys . This can cause permanent damage to the kidneys . Therefore, if there are symptoms, it is essential to seek medical advice immediately.

How are SAIDs identified?

To be honest, diagnosing SAIDs can be a bit challenging because the symptoms can be similar to those of other serious conditions, such as lupus or lymphoma. Therefore, it is important to see a doctor who has special training in inflammatory diseases (a rheumatologist) to accurately diagnose and manage your child's condition.

Your child's rheumatologist will use several things to diagnose SAIDs. He/she will ask about your child's symptoms and whether anyone in your family has had these conditions (biological family history). The doctor may suspect that your child has Periodic Fever Syndrome if:

• If you have frequent fevers
• If anyone in the family has a history of periodic fevers like this
• If you belong to a group of people who are prone to such fevers

What tests are used?

Your doctor may recommend several tests to confirm the diagnosis. These may include:

• Lab tests: Tests like `(C-reactive protein - CRP)` and `(Complete Blood Count - CBC)` can check for inflammation in the body. These tests are `(positive)` when there is a fever, and return to normal values ​​when the fever goes away.
• Urine test: A urine protein test can be done to check for high levels of protein in the urine. In MKD, a urine test for organic acids will show elevated levels of mevalonic acid.
• Genetic testing: This can check for genetic variants. However, some children with SAIDs may not have the genetic variant found. Therefore, even if the test results are ``negative``, the possibility of SAID cannot be ruled out.

How are SAIDs treated?

Treatment for SAIDs varies depending on the type and severity of the disease . Although these diseases cannot be cured completely, the symptoms of the child can be well controlled with the use of medications . For example, if your child only gets these colds a few times a year, painkillers and nonsteroidal anti-inflammatory drugs (NSAIDs) can usually control the symptoms. However, if the disease is more severe, the doctor may suggest other treatment options.

Here are some such treatments:

• FMF: This can be treated with a drug called `(Colchicine)` to reduce inflammation. If you can't take `(Colchicine)`, you can try a `(Biologic)` type of drug called `(Canakinumab)`.
• PFAPA: The flare-ups of PFAPA can be shortened by giving steroids (often prednisone). Some children have been able to control their symptoms by using cimetidine, a medicine used to treat stomach ulcers.
• TRAPS: The drug `(Canakinumab)` is very effective for TRAPS. Also, prescription anti-inflammatory medications like `(Glucocorticoids)` can also help relieve symptoms.
• MKD: `(Canakinumab)` is a good option for MKD treatment. Using `(NSAIDs)` or `(Steroids)` during fever may also be helpful.
• NLRP3 diseases: Other ``Immunomodulators'' drugs like ``Canakinumab'' and ``Rilonacept'' and ``Anakinra'' are successfully used to treat NLRP3 diseases.
• AOSD: Various types of anti-inflammatory medications are used for AOSD. These include `(Steroids)`, `(Disease-Modifying Anti-Rheumatic Drugs - DMARDs)`, and `(Biologics)`.
• Blau syndrome: Depending on the child's symptoms, immunosuppressants, tumor necrosis factor (TNF) inhibitors, and/or topical eye medications may be tried.

Does SAIDs condition get better on its own?

Some SAIDs can last a lifetime . Others may last for a few years and get better with age. While some conditions are lifelong, the frequency of fevers may decrease over time and the symptoms may become less severe . Your doctor can give you specific details about your child's specific condition.

Caring for a child with SAID can be challenging. Perhaps you have SAID yourself, but you can use your personal experiences to help your child care and help them cope with this lifelong condition.

The most important thing is to seek treatment from specialists who are well-versed in the intricacies of SAIDs. They can help manage your child's symptoms and help them have the best possible childhood.

The most important things to remember (Take-Home Message)

Okay, so let's summarize some of the things you need to remember from what we've talked about.

• If your child has frequent, unexplained fevers , talk to a doctor about it. It could be a sign of SAID.
• SAIDs are a problem of the innate immune system , not a common infectious disease.
• These are different from ``Autoimmune'' diseases .
• Most of the time, these are caused by genetic factors .
• It is important to see a Rheumatologist for an accurate diagnosis.
• Treatment can control symptoms well .
• Early treatment is essential to prevent kidney damage.

If you have any more questions about this, don't be afraid to ask your family doctor or pediatrician. They will be able to help you further.

` SAIDs, hereditary fever, frequent fever, fever in children, immune system, genetic diseases, rheumatologist