Muckle-Wells syndrome associated with idiopathic interstitial pneumopathy
Gómez Rodríguez, F.; Muñoz Lucena, F.; Peña Griñán, N.; Umbria Domínguez, S.
Anales de Medicina Interna 8(2): 85-86
1991
ISSN/ISBN: 0212-7199 PMID: 1893010 Document Number: 381687
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.