open access

Ahead of Print
Case report
Published online: 2021-04-19
Submitted: 2020-09-01
Accepted: 2020-11-06
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Amyloid cystic lung disease presenting in a patient with human immunodeficiency virus

Firas Mohammed El-Baba, Harsha Banavasi, Ayman Soubani
DOI: 10.5603/ARM.a2021.0007
·
Pubmed: 33871041

open access

Ahead of Print
CASE REPORTS
Published online: 2021-04-19
Submitted: 2020-09-01
Accepted: 2020-11-06

Abstract

A 30-year-old gentleman with a medical history of HIV, on HAART therapy and pulmonary amyloidosis proven by lung biopsy presented with progressive, worsening shortness of breath and chronic productive cough over the last year. Pulmonary function tests showed a severely obstructive process and CT scan of the thorax manifested diffusely thin-walled, air-filled cysts. After reviewing the patient’s prior histories, PFTs and CTof the thorax, the most likely cause of his symptoms was amyloid-related cystic lung disease. After being placed on a LABA/ICS, and LAMA inhaler, the man’s condition remained stable. Active oncologic and pulmonary surveillance were continued every three to six months as the patient’s respiratory status, PFTs and imaging remained stable. Thoughts of chemotherapy and lung transplant have been suggested, but not initiated at this time. Amyloid-related cystic lung disease is a rare presentation of amyloidosis in the thorax. It has been associated with collagen vascular diseases and mucosa-associated lymphoid tissue lymphoma. After an extensive literature search, it does not appear that this condition has been referenced in an HIV-positive patient; thus making this report unique. More case reports will need to be published and reviewed to see whether an association with HIV and amyloid-associated cystic lung disease exists.

Abstract

A 30-year-old gentleman with a medical history of HIV, on HAART therapy and pulmonary amyloidosis proven by lung biopsy presented with progressive, worsening shortness of breath and chronic productive cough over the last year. Pulmonary function tests showed a severely obstructive process and CT scan of the thorax manifested diffusely thin-walled, air-filled cysts. After reviewing the patient’s prior histories, PFTs and CTof the thorax, the most likely cause of his symptoms was amyloid-related cystic lung disease. After being placed on a LABA/ICS, and LAMA inhaler, the man’s condition remained stable. Active oncologic and pulmonary surveillance were continued every three to six months as the patient’s respiratory status, PFTs and imaging remained stable. Thoughts of chemotherapy and lung transplant have been suggested, but not initiated at this time. Amyloid-related cystic lung disease is a rare presentation of amyloidosis in the thorax. It has been associated with collagen vascular diseases and mucosa-associated lymphoid tissue lymphoma. After an extensive literature search, it does not appear that this condition has been referenced in an HIV-positive patient; thus making this report unique. More case reports will need to be published and reviewed to see whether an association with HIV and amyloid-associated cystic lung disease exists.

Get Citation

Keywords

amyloidoisis, amyloid cystic lung disease, HIV

About this article
Title

Amyloid cystic lung disease presenting in a patient with human immunodeficiency virus

Journal

Advances in Respiratory Medicine

Issue

Ahead of Print

Article type

Case report

Published online

2021-04-19

DOI

10.5603/ARM.a2021.0007

Pubmed

33871041

Keywords

amyloidoisis
amyloid cystic lung disease
HIV

Authors

Firas Mohammed El-Baba
Harsha Banavasi
Ayman Soubani

References (6)
  1. Kyle RA. Amyloidosis: a convoluted story. Br J Haematol. 2001; 114(3): 529–538.
  2. Zamora AC, White DB, Sykes AMG, et al. Amyloid-associated cystic lung disease. Chest. 2016; 149(5): 1223–1233.
  3. Pepys MB. Amyloidosis. Annu Rev Med. 2006; 57: 223–241.
  4. Gillmore JD, Hawkins PN. Amyloidosis and the respiratory tract. Thorax. 1999; 54(5): 444–451.
  5. Colombat M, Caudroy S, Lagonotte E, et al. Pathomechanisms of cyst formation in pulmonary light chain deposition disease. Eur Respir J. 2008; 32(5): 1399–1403.
  6. Ryu JH, Tian X, Baqir M, et al. Diffuse cystic lung diseases. Front Med. 2013; 7(3): 316–327.

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