TABLE 1

Demographics, localised or systemic variant, precursor protein, M-protein, free light-chain κ/λ ratio, and pulmonary function of 16 patients with amyloidosis of the respiratory system

No./sex/ageSystemic/localised (CT findings: single/multiple or DASA) or organ involvementPrecursor proteinM-protein/free light-chain κ/λ ratio (n: 0.25–1.80)Clinical presentationSmoking: pack-yearsComorbiditiesFVC % predFEV1/FVC
1/M/50L: Lung (single nodule)AL-λ(-)/NEAsymptomatic15None66%86%
2/F/69L: Lung (single nodule)AL-λIgG-λ/NEAsymptomaticNoneMGUS, DM137%71%
3/M/57L: Lung (multiple nodules)AL-λ(-)/NEAsymptomatic10None113%71%
4/M/74L: Lung (multiple nodules)AL-κIgG-κ/NEAsymptomatic10Pulmonary MALT lymphoma, MGUS119%69%
5/F/60L: Lung (multiple nodules)AL-κ(-)/NEAsymptomaticNoneSjögren's syndrome115%79%
6/F/72L: Lung (multiple nodules)AL-κNE/NEAsymptomaticNoneSjögren's syndrome, RA, NSIP, bronchiolitis72%78%
7/M/71L: Lung (multiple nodules)AL-λ(-)/NEDiverticulitis of colon49None89%62%
8/M/73L: Trachea-bronchusAL-κ(-)/NEBronchoscopy at CHPNoneCHP98%76%
9/F/63L: Mediastinal lymph nodeAL-λIgG-λ/5.73AsymptomaticNoneMGUS83%75%
10/M/61L: PleuraAL-κBence Jones-κ/NEDyspnoea, pleural effusion60MM, MM kidney, myeloma cell invasion to pleuraNENE
11/F/72S: Lung (DASA), mediastinal lymph node, pleura, heartAL-λ(-)/0.15DyspnoeaNoneNone73%85%
12/F/71S: Lung (DASA), kidney, heart, rectumAL-λIgG-λ, Bence Jones-λ/NEHaemosputum, cough, dyspnoeaNoneMM, cerebral infarction74%80%
13/F/62S: Lung (DASA), kidney, heartAA(-)/NEFever, dyspnoea of PCPNoneRA、PCPNENE
14/M/79S: Lung (DASA), heart, carpal tunnelATTRwtIgG-κ/NEHaemosputum35ATTRwt, MGUS, AF, pacemaker, carpal tunnel syndrome85%72%
15/F/84S: Lung (DASA), heartATTRwt(-)/0.94AsymptomaticNoneLung cancer55%73%
16/M/75S: Mediastinal lymph node, kidney, heart, rectumAL-λIgM-λ, Bence Jones-λ/0.05Dyspnoea50Waldenström macroglobulinemia, DM106%75%

CT: computed tomography; DASA: diffuse alveolar-septal amyloidosis; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s/FVC ratio; L: localised; S: systemic; AL: immunoglobulin-derived light-chain; NE: not examined; MGUS: monoclonal gammopathy of undetermined significance; DM: diabetes mellitus; MALT: mucosa-associated lymphoid tissue; RA: rheumatoid arthritis; NSIP: nonspecific interstitial pneumonia; CHP: chronic hypersensitivity pneumonitis; MM: multiple myeloma; PCP: pneumocystis pneumonia; ATTRwt: wild-type transthyretin amyloidosis; AF: atrial fibrillation.