Mole T M, Glover J, Sheppard M N
Department of Surgery, Royal Brompton National Heart and Lung Hospital, London, UK.
Thorax. 1995 Mar;50(3):280-3. doi: 10.1136/thx.50.3.280.
Sclerosing mediastinitis is a rare condition which causes dense fibrosis of the mediastinum. Few large studies have been reported to date. The clinical and pathological features of cases have been studied in a specialist referral centre in the UK.
The pathological files of the Royal Brompton Hospital were examined and 18 cases of sclerosing mediastinitis were identified between 1970 and 1993. The clinical notes were obtained and the pathological specimens analysed.
There were 12 men and six women of age range 9-64 years. Twelve patients presented with shortness of breath, six had haemoptysis, three had hoarseness, four had pleuritic chest pain, three general weakness, two had dysphagia, and one was asymptomatic. Nine patients had a previous history of pulmonary tuberculosis. Two had auto-immune disease--one rheumatoid arthritis and the other systemic lupus erythematosus. There were three cases of previous malignancy--two undifferentiated carcinoma of the lung and the other Hodgkin's disease. Serological tests revealed only one positive reaction to Histoplasma. The erythrocyte sedimentation rate and serum immunoglobulins were raised in nine patients. Diagnosis was usually by thoracotomy with biopsy. All cases had fibrosis and chronic inflammation with no active granulomas. No infective organisms or positive cultures were obtained in any case. Ten of the 18 cases are alive up to 15 years after diagnosis, with only two deaths and six lost to follow up.
Sclerosing mediastinitis is a slowly progressive condition associated with previous tuberculosis, mediastinal malignancy, and autoimmune disease. The outlook is excellent for those cases without underlying malignancy.
硬化性纵隔炎是一种罕见疾病，可导致纵隔致密纤维化。迄今为止，鲜有大型研究报道。英国一家专科转诊中心对病例的临床和病理特征进行了研究。
查阅皇家布朗普顿医院的病理档案，确定1970年至1993年间有18例硬化性纵隔炎病例。获取临床记录并分析病理标本。
患者年龄在9至64岁之间，男性12例，女性6例。12例患者出现呼吸急促，6例咯血，3例声音嘶哑，4例胸膜炎性胸痛，3例全身乏力，2例吞咽困难，1例无症状。9例患者既往有肺结核病史。2例患有自身免疫性疾病，1例类风湿关节炎，另1例系统性红斑狼疮。有3例既往有恶性肿瘤病史，2例为未分化肺癌，另1例为霍奇金病。血清学检测仅1例对组织胞浆菌呈阳性反应。9例患者红细胞沉降率和血清免疫球蛋白升高。诊断通常通过开胸活检。所有病例均有纤维化和慢性炎症，无活动性肉芽肿。所有病例均未发现感染性生物体或阳性培养物。18例中有10例在诊断后存活长达15年，仅2例死亡，6例失访。
硬化性纵隔炎是一种与既往肺结核、纵隔恶性肿瘤和自身免疫性疾病相关的缓慢进展性疾病。对于无潜在恶性肿瘤的病例，预后良好。
