Cyclic syndrome involving the TNF . receptor

Cyclical recurrent fever, painful swelling of the extremities, and muscle pain on movement are characteristic manifestations of cyclic syndrome involving tumor necrosis factor (TNF) receptors. What causes and diagnoses this syndrome?

Tumor Necrosis Factors (TNF) is one of the characteristic disorders of hereditary cyclic fever syndrome, also known as familial Hibernian fever. This disorder is autosomal dominant and is characterized by recurrent episodes of fever and muscle pain on movement.
In addition to the TNF receptor-associated cyclic syndrome, hereditary cyclic fever syndrome also has other disorders such as the PFAPA syndrome, which is characterized by recurrent fevers for 3-6 days, accompanied by inflammation. mouth ulcers , pharyngitis , lymphadenitis . This disorder is commonly seen in children between the ages of 2 and 5.

As mentioned above, genetic abnormalities, TNF receptor-related cyclic syndromes caused by mutations in genes encoding TNF receptors, namely TNF receptor 1, TNFR1 receptor positive Aggregates trigger a protein response and lead to abnormal inflammation.

Manifestations of TNF receptor-associated cyclic syndrome in exacerbations most often have an onset before adulthood (20 years of age). The average duration of each exacerbation is 10 days and ranges from 1 to 3 weeks, with the main symptoms being:
Recurrent fever in cycles Swollen fingertips and toes, muscle pain when moving Area The skin of the upper body is red, if it comes in contact, it will cause pain

Nausea, abdominal pain, constipation or diarrhea Headache, conjunctivitis, pain around the eye sockets, swelling Joint pain, rash Inguinal hernia, testicular pain in men Metabolic and endocrine disorders (or also known as amyloidosis) but is rare.

TNF receptor-associated cyclic syndrome is diagnosed by the following methods:
Clinical evaluation: The doctor takes the history and does a physical exam. Laboratory tests: Blood tests check for neutrophil levels (increased), acute phase reactants (increased), blood gamma globulin levels during exacerbations. Urine tests check for protein levels (which should be done regularly).

Treatment of cyclic syndromes associated with TNF receptors is mainly short-term corticosteroids with a minimum dose of 20 mg of prednisone once a day. Your doctor may prescribe an NSAID with or without treatment to stop exacerbations. High doses can occur when the doctor prescribes an increase in the dose and takes it for a long time. Alternatively, treatment with subcutaneous injection of 100 mg Anakinra once daily and 150 mg Canakinumab every 4 weeks can be used.
Diagnosis of TNF receptor-associated cyclic syndrome is mainly based on clinical examination and genetic testing.
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