Muckle-Wells syndrome is characterized by recurrent episodes of urticaria, fever, polyarthralgia, deafness and secondary amyloid (AA type), familial type with autosome dominant features; few cases have been described. A case of a patient with idiopathic interstitial pneumopathy, diagnosed 7 years before the onset of clinical amyloid, is presented. The patient had lymph glands enlargement and nephrotic syndrome and died 18 months later due to renal insufficiency. We have not found this association previously described in any medical literature.