Lamberts R, Helmchen U, Creutzfeldt W
Abteilung Gastroenterologie und Endokrinologie der Medizinischen Universitätsklinik Göttingen.
Dtsch Med Wochenschr. 1989 Jun 16;114(24):945-50. doi: 10.1055/s-2008-1066698.
For three months a 17-year-old boy had been suffering from laryngitis, pharyngitis and conjunctivitis, weight loss, nocturnal sweating and signs of changing cranial-nerve involvement (dizziness, nausea, nystagmus). The diagnosis of recurrent polychondritis was made only when, in addition to a definite inspiratory stridor there also developed a painful swelling of the left ear cartilage and a saddle nose due to loss of the cartilaginous portion of the nasal skeleton. Histological examination of the inflamed ear cartilage confirmed the diagnosis. After treatment with prednisone (100 mg daily, gradually reduced to 25 mg) and azathioprine (100 mg daily, increased to 150 mg after three weeks), there was clinical improvement, but the airway resistance (R = 6.2 cm H2O.s/l) rose further, requiring tracheostoma twelve months after onset of symptoms.
一名17岁男孩患喉炎、咽炎和结膜炎3个月,伴有体重减轻、夜间盗汗以及颅神经受累变化的体征(头晕、恶心、眼球震颤)。仅当除明确的吸气性喘鸣外,左耳软骨出现疼痛性肿胀且因鼻骨软骨部分缺失形成鞍鼻时,才诊断为复发性多软骨炎。对发炎的耳软骨进行组织学检查确诊了该病。在用泼尼松(每日100mg,逐渐减至25mg)和硫唑嘌呤(每日100mg,3周后增至150mg)治疗后,临床症状有所改善,但气道阻力(R = 6.2 cm H2O.s/l)进一步升高,症状出现12个月后需要进行气管造口术。