We describe a case of an 82-year-old Japanese woman with pulmonary amyloidosis and hemosiderosis associated with multiple myeloma. She had a background of end-stage renal failure of unknown etiology and had been on maintenance dialysis for 2 years. She complained of exertional dyspnea for four months. High-resolution CT of the chest revealed diffuse ground-glass opacities with mosaic attenuation, consolidation in the left lingular lobe, and wedge-shaped, subpleural nodules in the bilateral lower lobes. A transbronchial lung biopsy of the left lingular lobe showed deposition of amorphous, eosinophilic amyloid at the smooth muscle layer of bronchial tissue, with a positive Congo red staining signal in polarized light. Bronchoalveolar lavage fluid was brownish-yellow, and numerous hemosiderin-laden macrophages were detected with Berlin blue staining. From these findings, a diagnosis of pulmonary amyloidosis complicated with pulmonary hemosiderosis was made. Further work-up led to a diagnosis of multiple myeloma. Pulmonary amyloidosis complicated with pulmonary hemosiderosis is a rare disorder and may be underdiagnosed. Physical examination, such as the appearance of the tongue, can assist the diagnosis of systemic amyloidosis. Use of bronchoscopy allows physicians make an early diagnosis of pulmonary amyloidosis that is minimally invasive.
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http://dx.doi.org/10.1016/j.rmcr.2021.101400 | DOI Listing |
J Am Coll Cardiol
December 2024
Cardiovascular Division, Brigham and Women's Hospital, Boston, Massachusetts, USA. Electronic address:
Background: Outpatient worsening heart failure (HF), defined by initiation or intensification of diuretics, is adversely prognostic for patients with either reduced or preserved ejection fraction.
Objectives: This study sought to investigate the prognostic value of outpatient worsening HF in transthyretin amyloidosis with cardiomyopathy and the effect of patisiran treatment.
Methods: Post hoc analyses of the APOLLO-B trial (NCT03997383) evaluated the associations between outpatient worsening HF (defined by oral diuretic initiation or intensification), measures of disease progression, and a composite endpoint of all-cause mortality and cardiovascular (CV) events.
Expert Rev Respir Med
January 2025
Division of Pulmonary & Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
Introduction: Amyloidosis, a polymeric deposition disease classified according to protein subtype, may have varied pulmonary manifestations. Its anatomic-radiologic phenotypes include nodular, cystic, alveolar-septal, and tracheobronchial forms. Clinical presentation may range from asymptomatic parenchymal nodules to respiratory failure from diffuse parenchymal infiltration or diaphragmatic deposition.
View Article and Find Full Text PDFJ Cardiol Cases
January 2025
Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan.
Unlabelled: Scarring of the left atrial (LA) wall from atrial ablation (AF) leads to the development of stiff LA syndrome. Multiple ablation treatments have been considered to be associated with the development of LA calcification (LAC). We report a case of wild-type transthyretin cardiac amyloidosis (CA) who presented with worsening heart failure due to stiff LA syndrome despite the condition after initial ablation for AF.
View Article and Find Full Text PDFAm J Cardiovasc Dis
December 2024
Prince Sultan Cardiac Centre, Heart Function Unit, Department of Adult Cardiology Riyadh, Saudi Arabia.
Background: Transthyretin cardiac amyloidosis (ATTRCA) is a prevalent disease, and it can be associated with heart failure (HF), left ventricle hypertrophy (LVH), atrial fibrillation (AF), and aortic stenosis (AS).
Aim: The study aims to detect the prevalence of ATTRCA in the symptomatic AS population.
Method: A single-center prospective study screening for ATTRCA in patients diagnosed with symptomatic severe AS undergoing aortic valve (AV) intervention.
Orphanet J Rare Dis
January 2025
Department of Cardiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.
Background: There is no unified prognostic scoring system for light chain cardiac amyloidosis (AL-CA), particularly stage IIIb AL-CA. This study aimed to use invasive haemodynamic information to investigate markers that can more accurately evaluate the prognosis of patients with stage IIIb AL-CA.
Methods: In this retrospective cohort study, we conducted invasive haemodynamic measurements concurrently with myocardial biopsies to diagnose AL-CA.
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