Symptoms of Tumor necrosis factor receptor associated periodic syndrome
• Recurrent episodes of fever, erysipelas-like rash (often quite painful), myalgia, serositis (peritonitis in 90%) and conjunctivitis. Periorbital swelling is common and is a distinguishing characteristic.
• Symptom onset generally in the first and second decade of life, but age of onset can vary up to middle-age.
• Rash is more common at the distal extremities and face (centrifugal pattern) and may be migratory.
• Arthralgias >> arthritis (arthritis is typically monoarticular, if present).
• Myalgia commonly involves the thigh; often migrates to additional areas.
• Inflammatory markers will be elevated during flares, which are often longer in duration than FMF attacks (>5-7 days, on average lasting 2–4 weeks).
• Recurrence of attacks: average 2 to 6 attacks per year.
• Amyloidosis occurs in about 10% of patients, which is less than what is seen in untreated FMF (monitor with urinalysis to identify proteinuria, as well as serum creatinine).