Abstract
AbstractThe etiology of unexplained periodic fever is often complex, and hereditary factors play an important role. This article describes a 26-year-old chinese women with intermittent fever for 9 years, with 10-year history of IgA nephropathy. Her fever is relieved during pregnancy, but after a baby is born, fever reappears, accompanied by headache, gasping after activity, chest pain, abdominal pain, blood in the stool, ataxia, intermittent back erythema, skin biopsy suggests amyloidosis, the autoinflammatory PLCG2 associated antibody deficiency and immune dysregulation was diagnosed by genetic testing. The fever was gradually relieved after treatment with rilonacept.
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