They will affect multiple family members.
An acquired autoinflammatory disease can develop at any time during childhood or in adulthood.
Early symptoms usually includemouth sores, with genital sores commonly occurring.
Diagnosis depends on your symptoms and medical history, since there are no specific tests.
There is no cure, but Behcet’s can be managed with medication and lifestyle changes.
FMF is usually diagnosed in childhood.
Between attacks, people affected by FMF will not have symptoms.
While there is no cure for FMF, treatment can relieve or prevent symptoms.
FMF can be managed as long as a person with the condition sticks to their treatment plan.
FCAS is an extremely rare condition affecting one out of every one million people.
This condition causes periods of skin rash, fever, and joint pain.
It can lead to hearing loss and kidney disease.
People with NOMID have skin rash present at birth and that persists throughout their lives.
NOMID can cause chronic meningitis inflammation of the tissues of the brain and spinal cord.
It can also cause intellectual disability, hearing loss, and vision problems.
NOMID is extremely rare with only about 100 cases reported worldwide.
TRAPS affects about one person in every million people.
Fever episodes will occur without reason.
Episodes may come about because of triggers like an injury, infection, stress, and hormone changes.
These episodescalled flarescan begin at any age, but most people have the first episode during childhood.
People with TRAPS will sometimes developamyloidosis, an abnormal buildup of amyloid protein in the kidneys.
Amyloidosis canlead to kidney problems.
About 15% to 20% of people with TRAPS will develop amyloidosis, usually in middle adulthood.
In autosomal recessive inheritance, both copies of a gene will have mutations.
These conditions are not normally seen in every generation of an affected family.
DIRA causes severe skin and bone inflammation and it may affect the internal organs.
DIRA can be fatal especially in early childhood.
Bone and joint inflammation can cause the skin over the affected areas to be inflamed as well.
Children with DIRA experience intense chronic pain that may affect feeding and growth and cause severe suffering.
A new mutation can also cause HIDS.
But having the mutation does not mean you will develop the condition.
HIDS is a lifelong condition, but it may improve in adulthood.
HIDS causes attacks that start with chills and a fever that can last for days.
Additional symptoms include:
The intensity of a HIDS flare will taper off after a few days.
Flares will occur often and can be triggered by trauma or stress.
Ilaris (canakinumab) is amonoclonal antibodydrug that’s approved by the Food and Drug Administration for AOSD treatment.
Autoinflammatory vs. Autoimmune Diseases
Both autoimmune and autoinflammatory diseases involve immune system malfunction.
They may cause similar symptoms, such as joint pain and swelling, rashes, and fatigue.
What is distinct between these two types of disease categories is their underlying causes.
Differences in their causes mean these diseases are not treated the same.
They can also cause different long-term problems and complications.
Autoinflammatory diseases affect the innate immune system, while autoimmune diseases affect the adaptive immune system.
The adaptive immune system has learned throughout a persons life what pathogens to attack.
The adaptive immune system is specific in its attacks.
The bodys innate immune system isnt specific or adaptive.
Rather, it will use white blood cells and acute inflammation (short-term inflammation) to attack a pathogen.
Fever is the primary symptom of this response.
Autoinflammatory Disease Symptoms
The most common symptom of autoinflammatory conditions is recurrent fever.
Additional symptoms include:
Because these conditions cause systemic inflammation, they may affect multiple organs and body systems.
While rare, autoinflammatory diseases can also result from a new gene mutation that develops early in embryonic development.
More recent advances in genetics have helped researchers to identify changes in the genes responsible for these conditions.
This has helped researchers to diagnose specific autoinflammatory diseases through the genes that cause them.
This is because having a gene mutation doesnt mean a person will develop the condition associated with it.
Corticosteroid therapyandnonsteroidal anti-inflammatory drugs(NSAIDs) are used early on to treat inflammation.
Antitumor necrosis factor (TNF) therapyhas also been used successfully to treat several types of autoinflammatory diseases.
They are also challenging to live with, but researchers are continually working to recognize and diagnose these conditions.
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