ABSTRACT
Amyloidosis is a collection of diseases in which different proteins are deposited
as insoluble β-pleated sheets, disrupting organ function. Each precursor protein induces
a separate spectrum of organ involvement, and different disease manifestations within
the lung. Although autopsy data often demonstrate amyloid deposits in various compartments
of the lung, few of the pathologic findings are expressed clinically. We review the
pulmonary pathology, radiology, clinical presentations, and treatment options for
each of the major systemic and localized forms of amyloidosis. This review focuses
on amyloid derived from immunoglobulin light-chain protein (AL disease), which most
frequently involves the lung in both systemic and localized forms of the disease.
Manifestations of AL-related lung disease range from nodules identified on incidental
chest films to diffuse alveolar+-septal deposition mimicking diffuse alveolar damage.
We discuss respiratory failure due to diaphragm invasion, proximal tracheal disease,
and diffuse alveolar-septal deposition. Guidelines for evaluation of patients with
amyloid are presented.
KEYWORD
Amyloidosis - tracheobronchial amyloidosis - lung diseases
