Light chain deposition disease (pulmonary manifestations)

Last revised by Ciléin Kearns on 17 Apr 2022

Citation, DOI, disclosures and article data

Citation:

Weerakkody Y, Silverstone L, Kearns C, et al. Light chain deposition disease (pulmonary manifestations). Reference article, Radiopaedia.org (Accessed on 19 Apr 2024) https://doi.org/10.53347/rID-24849

DOI:

https://doi.org/10.53347/rID-24849

Permalink:

https://radiopaedia.org/articles/24849

rID:

24849

Article created:

16 Sep 2013, Yuranga Weerakkody ◉

Disclosures:

At the time the article was created Yuranga Weerakkody had no recorded disclosures.

View Yuranga Weerakkody's current disclosures

Last revised:

17 Apr 2022, Ciléin Kearns

Disclosures:

At the time the article was last revised Ciléin Kearns had no recorded disclosures.

View Ciléin Kearns's current disclosures

Revisions:

7 times, by 5 contributors - see full revision history and disclosures

Systems:

Chest

Tags:

stub, refs, cases

Synonyms:

Pulmonary manifestations of LCCD
Pulmonary manifestation of light chain deposition disease

Pulmonary light chain deposition disease is a rare manifestation of systemic light chain deposition disease (LCDD).

Pathology

Fragments of immunoglobulin light chains secreted by a plasma clone are deposited as amorphous eosinophilic material in the alveolar walls, small airways, and vessels ²and may involve the interlobular septa ⁴. LCDD is Congo Red negative (in contrast to the Congo Red positive amyloid fibrils present in amyloidosis) ⁵.

Radiographic features

CT

HRCT manifestations include cysts, nodules, and lymphadenopathy ⁴. Cysts may be the result of small airway dilatation ¹. They are usually thin-walled, spherical and perivascular with vessels in the wall or traversing the cyst ⁴. Zonal distribution is diffuse. Cysts may slowly increase in size and number, coalescing into irregular shapes ⁴.