Pulmonary amyloidosis

Last revised by Mostafa Elfeky on 21 Oct 2021

Pulmonary amyloidosis is a rare manifestation of amyloidosis characterized by involvement of lung parenchyma. Two patterns are described:

Sometimes tracheobronchial amyloidosis is included under this entity, although the term thoracic amyloidosis is more accurate.

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Amyloidosis is of unknown incidence but is estimated to affect approximately 8 in 100,000 people from Western Europe and the United States 10.

The disease is characterized by extracellular deposition of fibrillary protein in the lungs. With lung parenchymal involvement there are two distinct forms:

The disease can be primary or secondary where the term primary pulmonary amyloidosis is given when the disease is confined to the lungs 3. With pulmonary amyloidosis, the primary form tends to be more common 3. 30% of patients with primary pulmonary amyloidosis are ultimately diagnosed with multiple myeloma or B cell lymphoma, and 10-15% of patients with myeloma or lymphoma will develop pulmonary amyloidosis 9.

The most common pulmonary manifestation is amyloid deposition in small pulmonary artery branches 11.

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There are many imaging patterns depending on the form and it may be better to refer to individual subtypes.

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