Summary of autoinflammation of unknown cause
If someone has symptoms of autoinflammation, but these are not compatible with one of the known autoinflammatory diseases, we say this patients has autoinflammation of unknown cause. Autoinflammation of unknown cause is diagnosed by excluding other causes for the symptoms and by proving that treatment with the anti-inflammatory drug anakinra is effective.
Several autoinflammatory diseases are known
Several autoinflammatory diseases are known. Examples are: familial Mediterranean fever (FMF), Cryopyrin associated periodic syndrome (CAPS), Hyperimmunoglobulin D and periodic fever syndrome (HIDS), TNF-receptor associated periodic syndrome (TRAPS), Schnitzler syndrome, Adult onset Still's disease, Behcet's disease, periodic fever, aphtous ulcers, pharyngitis and adenitis (PFAPA) syndrome and pyoderma gangrenosum, pyogenic arthritis and acne (PAPA) syndrome. You can find more information on some of these diseases elsewere on this website.
Some people suffer from signs and symptoms of an autoinflammatory disease, although one of the known autoinflammatory diseases cannot be found. This is called autoinflammation of unknown cause.
Autoinflammation of unknown cause is an ... Read more »