Azevedo Francisco Vileimar Andrade de, Albuquerque Juliana de Deus E, Gonçalves Deborah Pereira
Hospital Geral Dr. Waldemar Alcântara, Fortaleza, CE, Brasil.
Hospital Geral Dr. Waldemar Alcântara, Fortaleza, CE, Brasil.
Rev Bras Reumatol. 2015 Mar-Apr;55(2):174-6. doi: 10.1016/j.rbr.2014.03.030. Epub 2014 Nov 1.
Relapsing polychondritis is an uncommon, immune-mediated condition characterized by episodes of inflammation of cartilaginous structures, especially the ears, nose, joints and respiratory tract. RP also affects proteoglycan-rich structures such as the eyes, heart, blood vessels and inner ear. Around one third of cases are associated with other diseases such as vasculitides, connective tissue diseases or myelodysplastic syndrome. Disorders of the inner ear occur in 40-50% of patients. Profound hearing loss is rare. The aim of this study was to describe the case of a patient with relapsing polychondritis associated with severe bilateral hearing loss and clinical manifestations of systemic vasculitis. This study reinforces the importance of an early diagnosis and immediate treatment in case of severe manifestations of the disease.
复发性多软骨炎是一种罕见的免疫介导性疾病,其特征为软骨结构(尤其是耳朵、鼻子、关节和呼吸道)发生炎症发作。复发性多软骨炎还会影响富含蛋白聚糖的结构,如眼睛、心脏、血管和内耳。约三分之一的病例与其他疾病相关,如血管炎、结缔组织病或骨髓增生异常综合征。40%至50%的患者会出现内耳疾病。严重听力损失很少见。本研究的目的是描述一例患有复发性多软骨炎并伴有严重双侧听力损失及系统性血管炎临床表现的患者病例。本研究强调了在疾病出现严重表现时早期诊断和立即治疗的重要性。