Clinical, radiologic, and pathologic features and outcomes of pulmonary transthyretin amyloidosis.
Respir Med
; 194: 106761, 2022 04.
Article
in En
| MEDLINE
| ID: mdl-35217402
INTRODUCTION: Amyloid transthyretin amyloidosis (ATTR) is characterized by deposition of a misfolded conformation of the transport protein TTR, most commonly in cardiac and nerve tissue, causing clinical disease. Pulmonary amyloidosis, or deposition of ATTR in lung tissue, is a poorly characterized manifestation of this disease. We present the clinical course, imaging characteristics, pathology results, and outcomes of a patient cohort diagnosed with pulmonary ATTR. METHODS: We retrospectively reviewed records of 28 patients with pulmonary ATTR seen at Mayo Clinic from September 30, 2005, through December 31, 2020. Data collected included information on demographics, subjective symptoms, tissue biopsy results, pulmonary function testing, imaging findings, and treatment. RESULTS: Of the patients, 89% were men; the median age was 74.5 years (range, 50-99 years). Patients were typically diagnosed after persistent dyspnea and abnormal chest imaging resulted in lung biopsy, which yielded the ATTR diagnosis. Most patients had a preexisting diagnosis of cardiac ATTR. The disease was wild-type in 62% and hereditary in 38%. Normal pulmonary function tests followed by a restrictive pattern were the most common presentation. Of the patients, 93% had chest computed tomography, with common findings of diffuse nodularity, calcified granulomas, interlobular septal thickening, and pleural effusions. Almost all patients had pulmonary vascular involvement, and half had interstitial involvement on tissue biopsy. One-third received either anti-amyloid pharmacotherapy or a heart transplant. Half of patients had died before the time of study inclusion. CONCLUSION: Pulmonary disease is a less common but clinically important manifestation of ATTR.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Amyloid Neuropathies, Familial
/
Lung Diseases
Type of study:
Observational_studies
/
Risk_factors_studies
Limits:
Aged
/
Humans
/
Male
Language:
En
Journal:
Respir Med
Year:
2022
Document type:
Article
Affiliation country:
United States
Country of publication:
United kingdom