Does your baby often have a fever for no reason? Let's talk about these SAIDs (Systemic Autoinflammatory Diseases)

Does your baby often have a fever for no reason? Let's talk about these SAIDs (Systemic Autoinflammatory Diseases)

Does your little one just get a fever all the time? Does he get better after a fever for a few days, only to get another fever a few days later? You might be wondering why this happens without a virus or bacterial infection. This could be due to a rare condition called SAIDs (Systemic Autoinflammatory Diseases) , which many people have not heard of. Don't worry, let's talk about it in simple terms and understand everything.

What are SAIDs? Let's understand it simply.

Simply put, SAIDs are a group of diseases that cause frequent, recurring fevers without any infection (such as viruses or bacteria). The cause of this is a malfunction in our child's innate immune system . This was previously called 'Periodic Fever Syndromes'.

Now you may be wondering, is this an 'autoimmune' disease? We often hear about autoimmune diseases like Rheumatoid Arthritis or Lupus. No, this is different.

Imagine that we have a defense army inside our body, our immune system. This army has two main parts:

1. Innate Immune System: This is the army that we are born with, and is the first to attack any germ.

2. Adaptive Immune System: This is an army that is specially trained to fight off various germs as we grow up.

In SAIDs, the problem is with the innate immune system . That immune system gets agitated and starts a fight within the body without any enemy. The result of this fight is inflammation in the body and a fever.

But in the autoimmune diseases we talked about, the problem is with the adaptive army that is trained later. That army is unable to recognize the good cells in its own body and starts attacking them.

SAIDs are much rarer than autoimmune diseases. They are often caused by genetic causes, meaning they are inherited. These symptoms usually begin when a child is very young, such as during infancy or toddlerhood. The child suddenly has an episode or attack of illness with fever and other symptoms, which usually resolves within a few days. The child may be completely healthy when not sick. Although there is no cure, there are effective treatments that can control the symptoms.

What are the main types of SAIDs?

Researchers have identified about 60 types of SAIDs so far, and new types are being discovered all the time. Here are some of the most common types.

Name of the disease (Type of SAID) A brief introduction
Familial Mediterranean Fever (FMF) This is the most common type of genetically identified SAID, causing painful inflammation in the abdomen, chest, and joints.
Periodic Fever, Aphthous-Stomatitis, Pharyngitis, Adenitis (PFAPA) It starts in young children, especially before the age of 4. Symptoms such as sore throat, mouth sores, and swollen glands in the neck come with the fever. It usually goes away after the age of 10.
Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS) This can start in childhood or even adolescence.
Mevalonate Kinase Deficiency (MKD) This usually starts before the child is one year old.
NLRP3-Associated Autoinflammatory Diseases (CAPS) This is actually a combination of three different diseases.
Adult-onset Still's Disease (AOSD) As the name suggests, this is a disease that begins in adulthood.
Blau Syndrome This usually occurs in children under the age of 4. It mainly affects the skin, eyes, and joints.

What are the common symptoms of these diseases?

The main and most common symptom of SAIDs is recurrent fever . However, in addition to fever, other symptoms may also occur depending on the type of disease.

The important thing is that these symptoms only occur during an episode of illness. Once that period is over, the child can be completely healthy without any symptoms.

Name of the disease (Type of SAID) Symptoms to watch for
FMF Severe pain in the stomach, chest, and joints. Skin lesions on the lower legs or ankles.
PFAPA Sore throat, mouth ulcers, swollen lymph nodes in the neck.
TRAPS The body feels cold and shivers. There is pain in the muscles of the body, especially in the trunk and arms. Painful red spots may appear all over the body.
MKD The body feels cold and shivery, with headaches, stomach aches, loss of appetite, and symptoms similar to those of a common cold.
NLRP3 Diseases Skin rashes, headaches, fatigue, joint pain, and eye infections (conjunctivitis).
Blau Syndrome Skin lesions on the child's arms, legs, or trunk. Joint pain and eye pain.

Why do these diseases occur? What are the causes?

As we mentioned earlier, many SAIDs are genetic diseases . This means that these diseases are caused by a mutation (variant) in a particular gene.

Name of the disease (Type of SAID) Associated Gene
FMF MEFV gene
PFAPA The exact reason has not yet been found.
TRAPS TNFRSF1A gene
MKD MVK gene
NLRP3 Diseases NLRP3 gene
AOSD The exact reason has not yet been found.
Blau Syndrome NOD2 gene

What can happen if proper treatment is not received?

This is a very important issue. It is essential to control this condition by taking proper treatment. Because, if there is continuous inflammation in the body like this, it can lead to a condition called Amyloidosis . Simply put, a type of protein can be deposited in our kidneys, causing permanent damage to the kidneys. Therefore, it is very important to diagnose the disease and treat it quickly.

How does a doctor diagnose this disease?

To be honest, diagnosing SAIDs can be a bit challenging because the symptoms can be similar to those of other serious diseases, such as lupus.

Therefore, the most important thing is to see a doctor who specializes in these types of diseases. A doctor who specializes in diseases related to the joints and the immune system is called a Rheumatologist .

Your doctor will consider several factors to make a diagnosis. He or she will likely ask about your child's symptoms and whether there is a family history of the condition. Your doctor may suspect SAIDs, especially if:

  • If the child has frequent fevers.
  • If anyone in the family has had recurring flu-like illnesses like this.
  • Since these diseases are common among some ethnic groups, that is also being considered.

What tests are performed?

The doctor may recommend several tests to confirm the disease.

  • Blood tests (Lab tests): Tests like C-reactive protein (CRP) and CBC (Full Blood Count) can check for inflammation in the body. These levels increase during illness and return to normal levels when the body is well.
  • Urine test: Checks for elevated protein levels in the urine. In some diseases, such as MKD, urine tests can detect specific acids.
  • Genetic testing: This test can detect genetic mutations. However, sometimes, a child may have SAID but the genetic test may not detect it. Therefore, even if the test result is negative, it cannot be said 100% that the disease is not present.

What are the treatments for this?

Treatment for SAIDs depends on the type and severity of the disease. Although these diseases cannot be completely cured, medications can greatly control the symptoms.

  • FMF: A drug called Colchicine is used for this. It reduces inflammation in the body. If that drug doesn't work for the child, they can turn to drugs like Canakinumab .
  • PFAPA: Steroids like Prednisone can be given for a short period of time to quickly reduce the duration of the illness.
  • TRAPS: The drug Canakinumab is very effective. Also, anti-inflammatory drugs like Glucocorticoids are used.
  • MKD: The drug Canakinumab is effective for this as well. NSAIDs (painkillers) or steroids can help during illness.
  • NLRP3 Diseases: Immune-modifying drugs such as Canakinumab , Rilonacept , and Anakinra are used successfully for this.
  • Blau Syndrome: Depending on the symptoms, immunosuppressants, TNF inhibitors, and eye medications may be used.

Will this condition be completely cured?

Some SAIDs are lifelong. However, some, like PFAPA, may go away on their own as the child grows older. Even though they are lifelong conditions, the frequency of illness and the severity of symptoms may decrease over time. Your doctor will give you specific details about your child's condition.

Caring for a child with this condition can be challenging. But with the help of the right specialists, you can manage your child's symptoms and give them the best childhood possible.

Take-Home Message

  • If your child has frequent fevers for no apparent reason, don't just ignore them and see a doctor immediately.
  • SAIDs are a group of rare diseases caused by a problem in the innate part of the immune system.
  • These are not contagious diseases; they are often caused by genetic factors.
  • It is very important to see a rheumatologist (a doctor who specializes in joints and the immune system) to accurately diagnose the disease.
  • Although these cannot be completely cured, medications can help control the symptoms and allow you to live a normal life.

Fever, fever in children, SAIDs, periodic fever syndrome, autoinflammatory disease, genetic diseases, immune system

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What tests are performed?

The doctor may recommend several tests to confirm the disease.

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