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Clinical, radiologic, and pathologic features and outcomes of pulmonary transthyretin amyloidosis.
Eggleston, Reid H; Hartman, Thomas E; Walkoff, Lara A; Yi, Eunhee S; Ryu, Jay H; Baqir, Misbah.
Affiliation
  • Eggleston RH; Mayo Clinic School of Graduate Medical Education, Mayo Clinic College of Medicine and Science, Mayo Clinic, Rochester, MN, USA.
  • Hartman TE; Department of Radiology, Mayo Clinic, Rochester, MN, USA.
  • Walkoff LA; Department of Radiology, Mayo Clinic, Rochester, MN, USA.
  • Yi ES; Division of Anatomic Pathology, Mayo Clinic, Rochester, MN, USA.
  • Ryu JH; Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
  • Baqir M; Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA. Electronic address: baqir.misbah@mayo.edu.
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.
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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

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