To the Editors,
A 35-yr-old Caucasian female was admitted with a 2-week history of rapidly progressive dyspnoea on minimal exertion. She denied any associated cough, chest pain or systemic symptoms. She gave a background of a 5-yr history of dyspnoea on moderate exertion, with intermittent cough, but no diurnal variation. This had been previously attributed to asthma and she had been maintained on inhaled corticosteroids and short-acting β-agonists. She had no symptoms suggestive of any connective tissue disease. She had no family history of respiratory disease. She was a heavy smoker, having accrued a 10-pack-yr history of smoking, but denied any illicit drug use. She was unemployed. There was no history of exposure to hazardous dusts.
Physical examination revealed reduced air entry throughout all lung fields but worse on the right, with ipsilateral hyperresonance. A chest radiograph (fig. 1a×) showed a right pneumothorax with bilateral diffuse reticular infiltrates predominantly affecting the mid-zones. There were no previous radiographs for comparison. An intercostal drain was inserted and the lung re-expanded. She underwent a high-resolution computed tomography scan of the thorax (fig. 1b×c),揭示了上部区域中存在大的双侧大桶,并在支气管血管分布中浸润。随后,她进行了视频辅助的胸腔镜检查,对她的中叶以及手术胸膜固定术。组织学证实存在非造成肉芽肿的存在(图2×). Fungal and mycobacterial stains were negative. A diagnosis of bullous sarcoidosis was made.
Serum angiotensin-converting enzyme was elevated at 160 U·mL−1and α1- 抗抑制蛋白蛋白水平在正常范围内。
她在基线时的肺功能测试显示1 s的强迫呼气量(FEV11.18 L(预测3.26 L)和强制生命力(FVC)为2.24 L(3.75 L PRED),FEV1/FVC ratio of 52%. Diffusing capacity of the lung for carbon monoxide (Dl,co) and carbon monoxide transfer factor were 33% and 58% pred, respectively. Her 6-min walk distance was 389 m with exertional desaturation from 98% to 86% and a pre-test Borg score of 3 and post-test Borg score of 6. She was commenced on oral prednisolone, initially at 30 mg·day−1随后的月份逐渐逐渐逐渐逐渐缩小到10 mg·天−1。The patient noted symptomatic improvement after introduction of steroids, although no changes in her physiological parameters occurred over the subsequent 2 yrs of follow-up.
Sarcoidosis, a granulomatous disorder of unknown aetiology, characteristically involves multiple organs, with lung involvement being predominant. The diagnosis of sarcoidosis is based upon the association of compatible clinicoradiological findings, histological demonstration of noncaseating granulomas, and exclusion of other granulomatous disorders. The pulmonary manifestations of sarcoidosis are diverse, involving the intrathoracic lymph glands and pulmonary parenchyma, as well as the airways. Occasionally, sarcoidosis has been reported to have atypical thoracic manifestations. One such example is the bullous form of the disorder. The condition was first reported in 1949 by Zimmerman and Mann1。A Medline has further revealed a total of 17 more case reports of bullous sarcoidosis search since then1–10。
大胆结节病的诊断年龄从21至64岁不等,平均年龄为37岁。男性比女性以2:1的比率更频繁地受到影响。在只有一半的情况下是规定的种族1,3,5,7,9,10and no ethnic differences in incidence was noted. Most patients had been noted to have bullous changes within 3–4 yrs of symptom development2–4,7。In four patients, the symptoms had been attributed to asthma or chronic obstructive pulmonary disease (COPD)7,9,10。Smoking history was not specified in six of the 18 case reports. Of the remaining 12 patients, three were never-smokers2,4,7, while two had smoking history of <10 pack-yrs7。Seven patients had presented with single or recurrent pneumothoraces, affecting the right more frequently than the left2,4–6,10。
Lung function showed an obstructive picture in 11 cases1–3,4,7,在三种情况下限制2,5,6并在一种情况下保存肺活量测定法10。In all cases in whichDl,co被测量,注意到值严重损害4,7,10。Few of the reports specifically mentioned the site of predominance of the bullae, but were noted to involve the lower as well as the upper zones equally.
全身性类固醇有10个病人s, with variable outcomes2,4–7。在案例报告中,随访时间很大。五个报告的症状,肺功能和/或射线照相表现的改善或稳定性4,6,7。However, response to corticosteroid therapy was only transient in three further cases, with further deterioration in symptoms and lung function. Five patients underwent bullectomy, two of which were bilateral2,3,5,7,10。Of these, four had improved while one patient3had enlarging contralateral bullae and respiratory failure. One patient underwent single lung transplant7, owing to worsening lung function and was noted to remain stable on follow-up. Five of the cases were reported to die with respiratory failure and cor pulmonale, two of whom were aged <35 yrs1–3,8。
尚不清楚Bullae的形成基础的确切机制,并提出了各种机制7, as follows. 1) Ball-valve obstruction, whereby endobronchial involvement by sarcoid lesions causes peripheral air trapping and alveolar distension and rupture. However, it is noteworthy that endobronchial involvement with sarcoidosis has not been universally found in cases where the bronchi were biopsied10。2) Formation of bullae by retraction and collapse of surrounding disease lung. 3) Presence of an inflammatory alveolitis, with release of many inflammatory mediators, resulting in tissue destruction.
Although considered to be rare, it is not inconceivable that bullous sarcoidosis may in fact be under-recognised for a variety of reasons. Patients frequently present with nonspecific symptoms, including intermittent wheeze and cough, and these are often attributed to other conditions, such as asthma and smoking-related COPD, especially when there is concurrent heavy smoking history. Bullous lung changes, especially in the upper zones in smokers, may often be attributed to smoking and alternative diagnosis may not be sought. Also, paucity of granulomatous changes10在尝试组织学诊断时,沿着细菌和检查的Bullae墙已经注意到了Bullae的壁,并突出了对相对幸免的肺组织的活检。
There is an increasing number of case reports of bullous sarcoidosis, suggesting that the condition may not be a rare entity as previously considered. It is interesting to note that a recent histopathological review of lung volume reduction surgery specimens revealed noncaseating granulomatous changes in nine out of 80 cases11。Most of the patients retrospectively, however, did not have clinical characteristics attributable to sarcoidosis, although mediastinal adenopathy was noted in the vast majority of the patients. A prospective study of cases referred for bullectomy or lung volume reduction surgery would be useful.
从临床角度来看,应该在具有或没有大量吸烟病史的年轻患者中保持高度怀疑指数,尤其是当其他放射学异常或临床特征表明表明外肺结节病外的同时存在。重要的是要认识到,绝大多数患者可能会在患病的早期出现,患有大胆疾病,可能对皮质类固醇治疗的反应不佳,其中一个子群迅速发展为呼吸衰竭和COR肺部。尽管尚不清楚患者选择的标准,但应积极寻求对子宫切除术或肺移植的考虑。
Statement of interest
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