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Semin Respir Crit Care Med 2020; 41(02): 299-310
DOI: 10.1055/s-0040-1708059
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Amyloid in the Lung

Misbah Baqir
1   Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota
,
Anja C. Roden
2   Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
,
Teng Moua
1   Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota
› Author Affiliations
Further Information

Publication History

Publication Date:
12 April 2020 (online)

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Abstract

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.

Keywords

amyloidosis - AL amyloid - AA amyloid - transthyretin amyloidosis - tracheobronchial amyloid
 

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