Multifaceted pulmonary manifestations of amyloidosis: state-of-the-art update

Amyloidosis, a polymeric deposition disease classified according to protein subtype, may have varied pulmonary manifestations. Its anatomic-radiologic phenotypes include nodular, cystic, alveolar-septal, and tracheobronchial forms. Clinical presentation may range from asymptomatic parenchymal nodules to respiratory failure from diffuse parenchymal infiltration or diaphragmatic deposition.

In this review, we systematically describe the molecular subtypes of amyloidosis and their clinical and radiologic findings in the lungs as well as key extrapulmonary organ systems. We detail novel treatment approaches to systemic amyloidosis. We also discuss prognostic elements for each subtype. We identify key clinical scenarios where reaching a precise diagnosis can be complicated, and we offer insights on the varied presentations of pulmonary amyloidosis.

Pulmonary amyloidosis is often difficult to diagnose as it may mimic other conditions, including fibrotic interstitial lung diseases and neoplasms, or can co-exist with certain connective tissue diseases. Despite some early artificial intelligence screening tools, improved familiarity among clinicians can aid in the more accurate and timely diagnosis of this multidimensional clinical entity. We additionally believe that multidisciplinary clinical pathwaysto diagnose and/or treat pulmonary amyloidosis have the potential to improve awareness, decrease diagnostic delay, and further elucidate knowledge on this multifaceted disease.