Imaging findings and follow-up in thoracic amyloidosis

Congress:

ESTI 2019

Poster Number:

P-0076

Type:

Educational Poster

Keywords:

Connective tissue disorders, Treatment effects, Diagnostic procedure, CT, Lung

Authors:

M. Lacroix¹, J. S. Rech², C. DE MARGERIE-MELLON¹, H. Nunes ², Y. Uzunhan², B. Arnulf¹, P.-Y. Brillet³; ¹Paris/FR, ²Bobigny /FR, ³Bobigny/FR

DOI:

10.26044/esti2019/P-0076

DOI-Link:

https://dx.doi.org/10.26044/esti2019/P-0076

Fig. 2: Diffuse bronchial thickening

Fig. 4: Peripheral topography of the nodules

Fig. 5: Condensation form with calcifications

Fig. 7: Cystic form

Learning objectives

Amyloidoses are disorders caused by abnormal extracellular deposition of proteins in beta-pleated sheets. Most organs may be concerned by localized or systemic amyloidosis. Lower respiratory tract amyloidosis is considered rare, either localized or systemic. Autopsic case series and reviews, while insufficiently detailed, underlined the multiplicity of respiratory tract amyloidosis presentations.

Background

Thoracic imaging manifestations of lower respiratory tract amyloidosis are rare, with very little data on follow-up.Our goal was to analyze the imaging pattern and to evaluate the imaging follow-up under treatment according to initial thoracic CT pattern. We retrospectively study 73 patients with confirmed thoracic amyloidosis who were classified into four patterns: “Tracheobronchial”, “Nodular”, “Interstitial” and “Composite” forms. Among these patients, 43 had at least one available thoracic CT and 27 had a CT before and after treatment.

Imaging findings OR procedure details

The mean age at diagnosis was 62 yo (54-70) with43 AL, 1 AA and 29 indefinite typing. Among the 43 patients with analysed thoracic CT, composite patterns were frequent: 35% of patients (n= 15), including 16% proven pleura disease (=7). There were 33% of “tracheobronchial “ (n=14), 16% of “nodular” (n=7) and also 16% of “interstitial” pattern (n=7). “Tracheobronchial” and “nodular” patterns were more frequent in localized amyloidosis (respectively76% and 80%) whereas “interstitial” and “composite” patterns were more frequent in systemic amyloidosis (respectively 86 and...

Conclusion

CT patterns of Amyloidosis are quite various and the combinationof individual patterns helps to evoke the diagnosis. CT images of amyloidosis involvement does not seem to be reversible whatever the initial CT pattern or the treatment.

References

1. 1. Mahmood S, Bridoux F, Venner CP, Sachchithanantham S, Gilbertson JA, Rowczenio D, et al. Natural history and outcomes in localised immunoglobulin light-chain amyloidosis: a long-term observational study. Lancet Haematol. 2015;2 2.2. Baumgart J-V, Stuhlmann-Laeisz C, Hegenbart U, Nattenmüller J, Schönland S, Krüger S, et al. Local vs. systemic pulmonary amyloidosis—impact on diagnostics and clinical management. Virchows Arch [Internet]. 22 août 2018 [cité 7 sept 2018]. 3.3. Khoor A, Colby TV. Amyloidosis of the Lung. Arch Pathol Lab Med. févr 2017;141(2):247‑54. 4.4. Milani P,...