Cordier J F
Hôpital Cardiovasculaire et Pneumologique Louis-Pradel, Université Claude-Bernard, Lyon.
Rev Mal Respir. 1989;6(1):5-14.
Amyloid is defined by its affinity for Congo red, which gives it a characteristic green birefringence in polarised light. This peculiarity is the result of its structure in beta-fibrillary folds, which is common to all biochemical varieties of amyloidosis whatever the origins of the immunoglobulin, reactive AA or prealbumin for example. Tracheobronchial amyloidosis exists in two forms: a pseudotumoral mass of a few millimeters in diameter discovered by chance at endoscopy without producing any clinical signs; and multi-focal sub-mucosal plaques which lead to bronchial stenosis and which can be destroyed by laser. Parenchymal amyloid can be nodular, or diffuse and interstitial. The amyloid nodules are single or multiple and their size varies from a few millimeters to several centimeters; they lead to few symptoms and do not require any treatment in the majority of cases if the diagnosis has been achieved by transparietal puncture for example (but the diagnosis is made above all by the excision of a mass which is presumed to be neoplastic). Diffuse interstitial parenchymal amyloid involves the alveolar region: it is a not uncommon finding at necropsy when it only infiltrates the vessels, it can give rise to the symptomatology of an interstitial pneumonia when there is widespread infiltration of the alveolar-capillary zone. The distinction between this type of diffuse interstitial amyloid and the miliary micro-nodular amyloidosis is sometimes difficult with overlapping between these two entities. Amyloid may also involve the pleura, the mediastinal nodes, the respiratory muscles and in particular the diaphragm; macroglossia may be responsible for obstructive sleep apnoea. Tracheobronchial amyloid and pulmonary nodules are generally localised to the respiratory system, whereas diffuse interstitial amyloid is combined in a group along with systemic amyloid. There is no specific treatment for amyloidosis.
淀粉样蛋白的定义基于其对刚果红的亲和力,这使其在偏振光下呈现出特征性的绿色双折射。这种特性是其β-纤维状折叠结构的结果,这是所有淀粉样变性生化类型所共有的,无论免疫球蛋白、反应性AA或前白蛋白等的来源如何。气管支气管淀粉样变性有两种形式:一种是在内镜检查时偶然发现的直径几毫米的假肿瘤性肿块,不产生任何临床症状;另一种是多灶性粘膜下斑块,可导致支气管狭窄,可用激光破坏。实质淀粉样变可为结节状、弥漫性或间质性。淀粉样结节可为单个或多个,大小从几毫米到几厘米不等;它们很少引起症状,在大多数情况下,如果通过经皮穿刺等方法确诊(但诊断主要通过切除疑似肿瘤的肿块来进行),则无需任何治疗。弥漫性间质性实质淀粉样变累及肺泡区域:在尸检时并不罕见,当它仅浸润血管时,当肺泡-毛细血管区域广泛浸润时,可引起间质性肺炎的症状。这种弥漫性间质性淀粉样变与粟粒性微结节淀粉样变之间的区别有时很困难,因为这两种实体之间存在重叠。淀粉样蛋白还可能累及胸膜、纵隔淋巴结、呼吸肌,尤其是膈肌;巨舌可能导致阻塞性睡眠呼吸暂停。气管支气管淀粉样变和肺结节通常局限于呼吸系统,而弥漫性间质性淀粉样变则与系统性淀粉样变合并在一起。淀粉样变性没有特效治疗方法。