If your children develop autoinflammatory disease, you should attentively consider their core body temperature. Autoinflammatory disease is a rare syndrome that is mostly unheard of, even by those who suffer from the disorder, causing a lack of understanding of it by family members or friends. Severe symptoms can be life-threatening. Therefore, we must pay close attention to children with periodic fever because it is not just a fever.

Elevated Temperature Is Likely an Inflammatory Flares Trigger

A recent study from the Garvan Institute of Medical Research has shown that core body temperature can be associated with mevalonate kinase deficiency (MKD). If the body temperature elevates, it may trigger inflammatory flares in patients with this rare autoinflammatory disease.

MKD is an inborn error of metabolism caused by two copies of a mutant gene from each parent. This recessive disorder has mutations in genes for mevalonate kinase (an enzyme present in all cells of the body). The deficiency of this enzyme leads to the buildup of defective proteins in immune cells, thus triggering inflammation. The study is published in the Journal of Clinical Investigation.

The disorder usually occurs in early childhood with regular symptoms such as high fever, skin rashes, ulcers, swollen lymph nodes, and abdominal pain. 

Mechanisms of Inflammatory Flares Poorly Understood

Because of the uncommonness of autoinflammatory disease, many people, including medical professionals, do not pay much attention to the disorder, thereby not having a full understanding of the condition.

“There has been very little progress in understanding MKD, and in particular, what causes disease flares in MKD patients. One of the main reasons for this lack of knowledge is the absence of appropriate animal models to study the mechanisms of disease,” says Garvan’s Dr. Marcia Munoz, lead author of the study.

Setting up the experiment, researchers modified the gene in mouse models which accurately mimics the mutations in MKD patients.

Munoz revealed that an increased core body temperature exacerbated the impact of mutant enzymes and led to the buildup of abnormal proteins, a likely cause of inflammatory flares in patients.

According to Professor Mike Rogers, Head of the Bone Therapeutics lab at Garvan, “The disease is caused by having two copies of the mutant gene, and there are more than 250 known mutations, so it’s difficult to predict what combination causes a mild or severe version of MKD.”

This is why MKD has a broad range of disease severity and is poorly understood.

Certain Levels of Enzyme Activity Have an Effect on the Proteins

To have a clearer vision of the mechanism of this disorder, researchers developed different mutant combinations in models with enzyme (mevalonate kinase) activity at 10 percent or 20 percent of normal levels. They discovered that there is no disease at the level of 20 percent or above. However, something occurred when the level dropped below this threshold.

“Disease starts to appear if enzyme activity falls below this threshold, when the effect on proteins really kicks in,” says Rogers.

Animals with 10 percent activity experienced clear signs of disease and higher levels of abnormal or defective proteins. Principally, elevated temperatures decreased the enzyme activity to nearly undetectable levels, increasing the amounts of abnormal proteins. 

These findings may help predict the severity of the symptoms of the disease, such as measuring the level of abnormal proteins in samples of blood. Clinicians may also use this information to diagnose and manage the disease, according to Rogers.

In addition, researchers also found that the protein NLRP3, involved in many inflammatory disorders in general, plays a role in the inflammatory process of MKD. Researchers are interested in discovering new treatments for MKD by targeting this protein and hopefully developing NLRP3 blockers for clinical use.

“Our research provides exciting new insights into the underlying physiology of MKD and what may be triggering the inflammatory flares, opening up potential new ways of treating this devastating disorder,” says Rogers.

How to Drop the Core Body Temperature Naturally

Parents whose children have experienced periodic fever should check their body temperature regularly. There are medically verified methods and home remedies that experts have suggested to lower the core body temperature naturally and safely. 

• Drink cool fluid—Drinking cool fluid is a good way to lower body temperature. An adequate amount of water can prevent dehydration, but sugary drinks and ​​popsicles can lead to further dehydration. Also, coconut water has an innate cooling effect.
• Relax in a cool-air environment—Experts recommend that air conditioning helps prevent heatstroke and heat-related deaths. Sitting in front of a fan is also a good way to cool off the body.
• Take fever-reducing medications—Antipyretic (temperature-reducing) medications are safe and effective to decrease body heat. Children with autoinflammatory disease should follow the doctor’s guidance before taking the medications.
• Avoid extreme or strenuous activities—Our body will be heated up during hot or humid conditions, so a high level of workout intensity will increase the body temperature. Trying to exercise with mild intensity is recommended, such as walking and cycling, but be sure to take frequent breaks. Swimming can be a good way to exercise and be submerged in cool water at the same time.
• Wear light-colored and loose-fitting clothing to reduce heat retention—Light colors reflect the sunlight rather than absorbing it, thus preventing the body from getting too hot. Larger size clothes help the air to circulate over the skin and cool off the body, but be sure to cover skin from the sun exposure.
• Avoid spicy and fatty foods—Body temperature can be elevated quickly by hot and spicy foods because they increase one’s metabolism, stimulating body heat. Consuming fatty foods increases the fat stored in body cells, thereby trapping the heat and warming up the body more.
• Take a lukewarm water bath—Lukewarm water helps reduce body temperature as the water evaporates. When not bathing, placing cool washcloths on the forehead and back of the neck can also help reduce the temperature. On the other hand, cold showers or ice baths are also recommended to cool the skin quickly, especially when an individual is at risk of heatstroke.

Besides that, having a hydrating diet, eating fruits and vegetables high in vitamin C, or applying aloe vera gel on the skin can also help in balancing body temperature. These are remedies that help change a patient’s lifestyle in a more positive way to confront MKD and autoinflammatory disease in general.