What is TNF Receptor Associated Periodic Syndrome?
TNF receptor associated periodic syndrome (henceforth referred to as TRAPS) is a condition caused by a mutation. Specifically, it is a disorder that affects the bodies receptors for cell necrosis factors, which automatically govern the death of the cells in the body such that they can be cleared out and replaced efficiently. It is a periodic fever syndrome, a type of disorder tied to the malfunction of the body’s inflammation reaction. Under normal circumstances, parts of the body become inflamed as the immune system combats various pathogens. With TRAPS, the body becomes inflamed in response to falsely-flagged cells.
TNF Receptor Associated Periodic Syndrome is fairly uncommon.
Who gets TNF Receptor Associated Periodic Syndrome?
TRAPS is a genetic condition. More specifically, it is an autosomal genetic condition. Sex-linked genetic traits are attached to the X or Y chromosomes in women or men, specifically the first chromosomes in ‘XX’, which is the typical female genotype, or ‘XY’, which is the typical male genotype. In autosomal genetic conditions, the condition is attached to the shared chromosomes between typical males and typical females. Consequently, genetically typical men and women are equally likely to suffer from TRAPS, and the intersexed are equally vulnerable.
As a genetic condition, however, it cannot be contracted. By and large, individuals suffering from TRAPS are born from parents that suffer from TRAPS or otherwise carry the genes for it.
Individuals with TRAPS tend to exhibit it from a young age. It exists from birth; age plays no role, and once again, it cannot be contracted.
What causes TNF Receptor Associated Periodic Syndrome?
TRAPS is caused by the malfunction of the tumor necrosis factor receptors. While the exact process that goes into the cause of TRAPS is still being studied and determined (albeit slowly), it has been tied down to a few possible scenarios: the most compelling possibility is that the tumor necrosis factor receptor is more difficult to get rid of.
This results in a flood of macrophages periodically swarming falsely-flagged cellular targets in the body. Ordinarily, macrophages are the white blood cells that surround and consume pathogens to neutralize the threat they pose. The activation of the immune system for this purpose frequently causes fever and inflammation. In the case of TRAPS, the deluge of macrophages is unnecessary, and thus results in an erroneous reaction.
What does TNF Receptor Associated Periodic Syndrome cause?
TRAPS causes a number of symptoms, none of which are pleasant, one of which is dangerous. The symptoms most readily associated with TRAPS due to visibility are its strong inflammation. This inflammation causes outbreaks of rashes in the skin, many if not all of which will present with bright redness. Areas affected by inflammation will be warm, if not hot to the touch, and may be exceptionally sensitive. Puffiness around the eyes is very common, which may obscure vision to some degree and cause the eyes to water. The joints may become swollen, stiff and painful, which can lead to some difficulty moving them normally.
However, the most dangerous symptom is the fever. Fevers are a sign that the body is fighting off an infection; anyone with a fever is almost unquestionably ill and fighting off a foreign pathogen, causing the temperature of the body to raise. This can be beneficial to an extent as it can be just hot enough to kill a viral or bacterial pathogen. However, fevers are not always benign, and when high enough, they can cause serious damage to the body proper. This is even more true in TRAPS; in TRAPS, there isn’t even a true viral or bacterial pathogen to fight off, so there is absolutely no benefit to be drawn from the fever, and the fever can possibly be quite high. High fevers themselves can cause damage to generalized systems, posing a neurological risk in particular.
How serious is TNF Receptor Associated Periodic Syndrome?
TNF Receptor Associated Periodic Syndrome is a reasonably serious condition. The gravity of this condition stems primarily from its chronic nature; it does not go away. An individual suffering from TRAPS will continue to suffer from it indefinitely. It may go into remission, but it will almost always recur. Individuals with TRAPS will have TRAPS for life.
That said, TRAPS is not fatal. Complications can occur that may contribute to the severity of other symptoms one is suffering from, but TRAPS itself cannot cause enough damage to cause lasting harm. However, because it is a chronic condition that exacerbates as many symptoms as it does without discrimination or external (and therefore avoidable trigger), TRAPS can be debilitating and distracting at best.
What does TNF Receptor Associated Periodic Syndrome treatment look like?
TNF receptor associated periodic syndrome treatment options are regrettably quite limited. There are several medications that have been tested with respect to treating TRAPS, but due to the rarity of TRAPS as a whole, test data is scarce. Due to a lack of appropriately controlled data, treatment of TRAPS directly is effectively a matter of conjecture and educated guesses. Most treatment is symptomatic in nature, to curb the effects of TRAPS outbreaks.
Various immunosuppressants may have an impact on TRAPS. Etanercept and Infliximab are the two primary candidates for this, as they interfere directly with the tumor necrosis factor and work to shut it down. With reduced function of the tumor necrosis factor comes reduced outbreak of TRAPS symptoms. Other immunosuppressants have been used for the same purpose, including generalized immunosuppressants more commonly associated with transplant rejection, and those associated with rheumatoid arthritis. The risk of these treatments is, however, the limitation on the immune system. Immunosuppressants do reduce the impact of the immune system when it acts erroneously and causes harm to the body, but even erratic immune systems continue to play a role defending the body against all manner of pathogens. There is rarely a moment that goes by that the body isn’t actively fighting off something, however mild. Exposure to pathogens happens on a daily basis to all but the most careful individuals, and it is never observed because the body’s immune system simply fights the invaders off and leaves them destroyed without trace. Consequently, suppressing the immune system can pose some risk.
Immunosuppression does not leave one without an immune system; trying to exist without an immune system for even a short period of time under controlled conditions is extremely risky, let alone trying to live normally without an immune system. Immunosuppressants are not, in and of themselves, dangerous, in that they only suppress the immune system’s action, mitigating what it can do without turning it off. They carry their own risks, but used properly they can naturally improve one’s quality of life (or else they wouldn’t be prescribed).
Symptomatic treatment is most likely to consist of analgesics or other fever reducers, and anti-inflammatories to alleviate the pain and discomfort in the joints. Other treatments and home remedies that apply to fever are also likely candidates for employment, such as keeping the individual well hydrated and keeping their external temperature more carefully regulated, if only to keep them comfortable.
How do I know if I have TNF Receptor Associated Periodic Syndrome?
TNF receptor associated periodic syndrome cannot be accurately self-diagnosed. The symptoms are generic, and could very easily be the product of a dermatitis condition or some other, far more innocuous ailment. TRAPS is a very rare condition, caused by a very unlikely mutation.
TRAPS can be identified by a qualified physician by its symptoms. The primary element of TRAPS that should be watched for its frequent recurrence; recurrent symptoms may indicate an appropriate allergy, whereas frequent recurrence (seemingly with no trigger) is more likely to indicate some intrinsic problem with the system.