Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.
Semin Respir Crit Care Med. 2020 Apr;41(2):299-310. doi: 10.1055/s-0040-1708059. Epub 2020 Apr 12.
Amyloidosis is the term given to abnormal deposition of misfolded precursor proteins at single or multiple sites, leading to organ dysfunction or clinical signs and symptoms. Pulmonary manifestations are nonspecific and may be associated with several amyloid protein subtypes, commonly AL (light chain) and AA (autoimmune) amyloids. Signs or symptoms of amyloid disease may often involve more of the clinical abnormalities of other affected organs than the lungs themselves. Radiologic pulmonary findings include septal and parenchymal ground glass or nodular infiltrates, multiple nodules, cysts, and focal tracheobronchial abnormalities. Lymphadenopathy with or without calcification and pleural effusions has also been reported. Directed therapy is initiated in response to clinical signs or symptoms often as a result of systemic or secondary diseases or conditions. Long-term prognosis is more dependent on the extent of organ involvement where morbidity is often the highest in those with multisystemic disease.
淀粉样变性是指异常折叠的前体蛋白在单一或多个部位沉积,导致器官功能障碍或出现临床体征和症状。肺部表现是非特异性的,可能与几种淀粉样蛋白亚型有关,常见的有 AL(轻链)和 AA(自身免疫性)淀粉样变。淀粉样变性病的体征或症状通常比肺部本身涉及更多受影响器官的其他临床异常。放射学肺部表现包括间隔和实质磨玻璃或结节状浸润、多发结节、囊肿和局灶性气管支气管异常。也有报道伴有或不伴有钙化的淋巴结病和胸腔积液。靶向治疗是根据临床体征或症状发起的,通常是由于系统性或继发性疾病或病症引起的。长期预后更多地取决于器官受累的程度,在多系统疾病患者中,发病率往往最高。