Gómez Rodríguez F, Muñoz Lucena F, Peña Griñán N, Umbria Domínguez S
Servicio Respiratorio y Medicina Interna, Hospital Universitario N, Sra., de Valme, Sevilla.
An Med Interna. 1991 Feb;8(2):85-6.
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.
穆-韦综合征的特征为荨麻疹、发热、多关节痛、耳聋及继发性淀粉样变(AA型)反复发作,为具有常染色体显性特征的家族型;仅有少数病例被描述。本文报告了1例特发性间质性肺病患者,在临床淀粉样变发病前7年被诊断。该患者有淋巴结肿大及肾病综合征,18个月后因肾功能不全死亡。我们未在任何医学文献中发现此前有过这种关联的描述。