Does your child often have fevers for no reason? Let's learn about these SAIDs (Systemic Autoinflammatory Diseases)!

Does your little one sometimes just get a fever without a cold or any other illness? Does the fever come, subside in a few days, and then reappear a few days later? Some parents face such experiences. So today we are going to talk about such a frequent, difficult-to-find cause of fever. This is called SAIDs (Systemic Autoinflammatory Diseases) . In the past, this was called `(Periodic Fever Syndromes)` or `(Recurrent Fever Syndromes)`.

What are these SAIDs? Let's understand it simply!

Simply put, SAIDs are a group of diseases that are caused by a malfunction or regulation problem in our body's innate immune system . In this case, without any external infection (such as a virus or bacteria), our body simply creates inflammation. It's as if our body's defense system is going into overdrive.

Think about it, we have a group of soldiers inside our bodies (that's the immune system). Their job is to fight off disease-causing germs when they come in. But in the body of someone with SAIDs, these soldiers sometimes just get agitated and cause problems inside the body.

Now you might be wondering if this is something like `(autoimmune diseases)` (that is, autoimmune diseases). For example, `(rheumatoid arthritis)` or `(lupus)` are similar. No, these two are a little different. In autoimmune diseases, what happens is that our body's acquired or adaptive immune system mistakenly attacks its own healthy cells. But in SAIDs, the problem lies with the innate immune system.

SAIDs are much rarer than autoimmune diseases. Most of them are hereditary , meaning they are caused by a genetic mutation or variant.

SAIDs usually start in infancy or early childhood . But that's not always the case. Your child may have episodes or attacks of fever and other symptoms. Your child may be completely symptom-free between attacks. There is no cure for SAIDs. However, there are treatments that can help control symptoms in most cases.

What are the main types of SAIDs?

Researchers have now identified about 60 types of SAIDs, and more are being discovered. Let's take a look at some of the most common types.

• Familial Mediterranean Fever (FMF): This is the most common of the genetically inherited, recurrent fever syndromes. It can cause painful inflammation of the child's abdomen, chest, and joints .
• Periodic fever, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): This usually starts in young children before the age of 4. This condition may subside spontaneously after the age of 10.
• Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): This can occur in childhood, adolescence, and sometimes even adulthood.
• Mevalonate kinase deficiency (MKD): Previously called hyper-IgD syndrome, this usually begins in babies under 1 year of age.
• NLRP3-associated autoinflammatory diseases: This was previously called cryopyrin-associated periodic syndromes (CAPS). There are three other subtypes within this.
• Adult-onset Still's disease (AOSD): This is an adult-onset form of 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? How do we recognize them?

Symptoms of SAIDs usually begin in childhood. The most common and prominent symptom is periodic or episodic fever . People usually remain symptom-free between attacks. However, there may be other symptoms specific to each type of SAID.

Let's look at the specific symptoms of several types of SAID discussed above:

• FMF: This can cause severe pain in the child's abdomen, chest, and joints, along with inflammation . Sometimes, a rash can develop on the lower legs or ankles. Imagine, a child suddenly develops a stomachache and fever. When the parents are worried and take them to the hospital, they may think it is appendicitis. But then it subsides and comes back after a few days.
• PFAPA: This can cause symptoms such as a sore throat, mouth sores, and swollen lymph nodes in the neck .
• TRAPS: This can cause chills, muscle pain in the chest and arms , and a painful red rash that starts on the arms and legs and spreads throughout the body.
• MKD: This can also cause a feeling of discomfort, such as chills, headaches, stomach pain, loss of appetite, and flu-like symptoms .
• NLRP3 diseases: These can cause symptoms such as skin rashes, headaches, malaise, joint pain, and red eyes (conjunctivitis) .
• AOSD: This is a condition that commonly causes skin rashes, joint pain, and muscle pain . Some people may also experience a sore throat, stomach pain, extreme fatigue, and body aches.
• Blau syndrome: This can cause skin lesions on your baby's arms, legs, or body . It can also cause joint pain and eye pain .

Why do these SAIDs occur? What are the causes?

Most SAIDs are genetic diseases . That is, each syndrome is caused by a variation (variant) in a specific gene.

Let's look at the genes that influence several major types of SAID:

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

Can these SAIDs cause other complications?

Yes, this can cause problems if not treated properly . If inflammation continues in the body, it can lead to a condition called ``amyloidosis.`` That is, a type of protein is deposited in our kidneys. This can cause permanent damage to the kidneys . That is why it is important to diagnose the disease early and get treatment.

How does a doctor diagnose this SAIDs condition?

In fact, diagnosing these autoinflammatory diseases can be a bit challenging , as their symptoms can mimic other serious conditions like lupus or cancers like lymphoma. Therefore, it is important to see a doctor who has special training in inflammatory diseases, a rheumatologist, to properly diagnose and manage your child's condition.

Your child's rheumatologist will use several factors to diagnose SAIDs. He or she will ask you about your child's symptoms and whether anyone in your family has these conditions (biological family history). The doctor may suspect that your child may have periodic fever syndrome if:

• If the child has frequent fever .
• If anyone in the family has a history of frequent fevers like this .
• If the child belongs to a group of people who are prone to periodic fevers like this .

What tests are being done for this?

To confirm the diagnosis, your child's rheumatologist may recommend several tests. These may include:

• Lab tests: Tests such as ``C-reactive protein (CRP)`` and ``complete blood count (CBC)`` can be used to check for inflammation in the body. These tests are positive during a fever "attack" and return to normal when the "attack" is over.
• Urine test: A urine protein test can be done to check for excess protein in the urine. In MKD, a urine test that looks at organic acids (acids containing one carbon atom) can show elevated levels of mevalonic acid.
• Genetic testing: This can check for genetic variants. However, some children with SAIDs may not have the genetic variants found, which can result in negative test results.

What are the treatments for these SAIDs?

Treatment for SAIDs varies depending on the type and severity of the condition . There is no cure for these conditions. However , your child's symptoms can usually be controlled with medication . If your child only has a few attacks a year, you may be able to give them painkillers such as nonsteroidal anti-inflammatory drugs (NSAIDs) to help reduce their symptoms. However, if the condition is more severe, your doctor may suggest other treatment options.

Here are some of them:

• FMF: A medicine called `(colchicine)` can be used to control this. This reduces inflammation. If the child cannot take colchicine, a `(biologic)` type of medicine called `(canakinumab)` can be tried.
• PFAPA: Steroids (usually prednisone) can be given to shorten the duration of a PFAPA "attack." In some children, cimetidine, a medicine used to treat stomach ulcers, can also help control symptoms.
• TRAPS: A drug called `(canakinumab)` is often effective for this. Also, symptoms can be relieved with anti-inflammatory medications prescribed by a doctor, such as `(glucocorticoids)`.
• MKD: Canakinumab is also an effective treatment for MKD. Giving NSAIDs or steroids during an "attack" can also be helpful.
• NLRP3 diseases: Immunomodulators such as canakinumab, rilonacept, and anakinra are used successfully for this.
• AOSD: Various types of anti-inflammatory medications are used for AOSD. Examples include steroids, disease-modifying anti-rheumatic drugs (DMARDs), and biologics.
• Blau syndrome: Depending on your child's symptoms, they may try immunosuppressants, tumor necrosis factor (TNF) inhibitors, and/or topical eye medications.

Are these SAIDs conditions permanent? Or will they go away?

Some autoinflammatory conditions are lifelong . Others may last for a few years and go away with age. Some conditions are lifelong, but over time, the frequency and severity of attacks may decrease. Your child's doctor can give you specific information about your child's condition.

Raising a child with an autoinflammatory disease can be challenging. If you have SAID, you can use your personal experiences to better support your child’s care. You can also use your experiences to help your child adjust to this lifelong condition.

The most important thing is to seek treatment from experienced specialists who are knowledgeable about SAIDs. Such doctors can help manage your child's symptoms and help them have the best possible childhood.

Final Take-Home Message

So, I hope you have some idea about the SAIDs we talked about today. Remember, if your child has frequent, unexplained fevers, definitely talk to a doctor about it . Don't just ignore it, thinking it's normal.

• SAIDs are a group of diseases that often cause fever and are caused by a problem with the immune system.
• These are often genetic and start at a young age.
• Although there is no permanent cure for this, there are treatments that can control the symptoms.
• It is essential to seek proper diagnosis and treatment from a specialist doctor (Rheumatologist).
• If treatment is started early, complications like amyloidosis can be prevented and the child can live a normal life.

If you have any more questions about this, don't hesitate to ask your family doctor or pediatrician.

` SAIDs, Systemic Autoinflammatory Diseases, Frequent Fevers, Genetic Diseases, Fevers in Children, Immune System, Rheumatologist