Abstract: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 trans… Show more
“…Consequently, 99m Tc-PYP scintigraphy is an essential non-invasive examination within the diagnostic pathway for ATTR cardiac amyloidosis. Although pulmonary involvement in amyloidosis is less frequent, it presents significant diagnostic challenges due to the non-specific nature of its radiological features and the scarcity of antemortem diagnosis [ 6 , 7 ].…”
Amyloidosis presents a diagnostic challenge, particularly when concomitant with severe conditions like acute exacerbations of idiopathic pulmonary fibrosis (IPF). In this report, we detail the case of a 73-year-old patient with acute exacerbation of IPF and simultaneous emergence of cardiac amyloidosis. The patient's clinical journey began with persistent exertional dyspnea, progressing to hypoxemia on admission. Chest CT scans showed extensive ground-glass opacities, consolidations, and pre-existing honeycombing-like cysts and reticular shadows, accompanied by a right-sided pleural effusion. The therapeutic strategy for acute exacerbation of IPF encompassed methylprednisolone pulse therapy, tacrolimus, and nintedanib, augmented with intravenous immunoglobulin and recombinant thrombomodulin. Concurrently, heart failure with preserved ejection fraction was managed with a pharmacological trio: empagliflozin, diuretics, and eplerenone. A hypertrophied heart and low limb voltage prompted an investigation for cardiac amyloidosis, which
99m
Technetium pyrophosphate (
99m
Tc-PYP) scintigraphy confirmed, yielding a probable diagnosis. Following steroid tapering, the patient was discharged home. This case prompted an investigation into the potential role of amyloidosis in pulmonary pathology. Our retrospective review of 10 patients, including four with cardiac amyloidosis, who underwent
99m
Tc-PYP scintigraphy, revealed a nonsignificant yet notable trend of increased pulmonary accumulation in cardiac amyloidosis cases (median (interquartile range): 5.4×10
4
(5.3-13.1×10
4
) vs. 3.6×10
4
(2.4-5.1×10
4
), p=0.0667). Notably, the pulmonary counts in this patient exceeded the negative cohort's mean values, hinting at a possible contribution of amyloid deposition to pulmonary pathology. This study, pioneering in evaluating lung field accumulation of
99m
Tc-PYP in cardiac amyloidosis, may provide novel insights into the influence of amyloidosis on pulmonary conditions.
“…Consequently, 99m Tc-PYP scintigraphy is an essential non-invasive examination within the diagnostic pathway for ATTR cardiac amyloidosis. Although pulmonary involvement in amyloidosis is less frequent, it presents significant diagnostic challenges due to the non-specific nature of its radiological features and the scarcity of antemortem diagnosis [ 6 , 7 ].…”
Amyloidosis presents a diagnostic challenge, particularly when concomitant with severe conditions like acute exacerbations of idiopathic pulmonary fibrosis (IPF). In this report, we detail the case of a 73-year-old patient with acute exacerbation of IPF and simultaneous emergence of cardiac amyloidosis. The patient's clinical journey began with persistent exertional dyspnea, progressing to hypoxemia on admission. Chest CT scans showed extensive ground-glass opacities, consolidations, and pre-existing honeycombing-like cysts and reticular shadows, accompanied by a right-sided pleural effusion. The therapeutic strategy for acute exacerbation of IPF encompassed methylprednisolone pulse therapy, tacrolimus, and nintedanib, augmented with intravenous immunoglobulin and recombinant thrombomodulin. Concurrently, heart failure with preserved ejection fraction was managed with a pharmacological trio: empagliflozin, diuretics, and eplerenone. A hypertrophied heart and low limb voltage prompted an investigation for cardiac amyloidosis, which
99m
Technetium pyrophosphate (
99m
Tc-PYP) scintigraphy confirmed, yielding a probable diagnosis. Following steroid tapering, the patient was discharged home. This case prompted an investigation into the potential role of amyloidosis in pulmonary pathology. Our retrospective review of 10 patients, including four with cardiac amyloidosis, who underwent
99m
Tc-PYP scintigraphy, revealed a nonsignificant yet notable trend of increased pulmonary accumulation in cardiac amyloidosis cases (median (interquartile range): 5.4×10
4
(5.3-13.1×10
4
) vs. 3.6×10
4
(2.4-5.1×10
4
), p=0.0667). Notably, the pulmonary counts in this patient exceeded the negative cohort's mean values, hinting at a possible contribution of amyloid deposition to pulmonary pathology. This study, pioneering in evaluating lung field accumulation of
99m
Tc-PYP in cardiac amyloidosis, may provide novel insights into the influence of amyloidosis on pulmonary conditions.
Background
The convergence of pulmonary hemorrhage, pulmonary amyloidosis, and multiple myeloma is uncommon. Amyloidosis can affect the pulmonary parenchyma in a diffuse, tracheobronchial, or parenchymal pattern and may rarely be associated with pulmonary hemorrhage. Additionally, pulmonary amyloidosis is not a frequent manifestation of multiple myeloma. We present a case of a male patient with pulmonary hemorrhage as the initial manifestation of AL pulmonary amyloidosis, and ultimately, confirmation of multiple myeloma through bone marrow biopsy.
Case presentation
The clinical case involves a 60-year-old male with no significant medical history, who was admitted presenting a clinical picture evolving over 6 months characterized by hemoptoic cough, accompanied by dyspnea, a decrease in functional capacity, and constitutional symptoms. Thoracic CT images revealed multilobar ground-glass opacities with suspected alveolar hemorrhage. In response to this clinical presentation, bronchoalveolar lavage with cytology was performed, revealing the presence of hemosiderin-laden macrophages. Given the complexity of the case, further investigation included a wedge biopsy of the lung. The pathological report indicated an atypical lymphoplasmacytoid proliferation with deposits of eosinophilic amorphous material, suggestive of amyloidosis. Congo red staining confirmed the presence of amyloid material. Elevated Kappa light chains were detected in both serum and urine, with an increased K/L ratio. Immunoglobulins G and M were found to be decreased. As part of the comprehensive assessment, a bone marrow biopsy was conducted, confirming the diagnosis of multiple myeloma with 10% atypical plasma cells. In light of this diagnosis, appropriate treatment has been initiated to address this intricate medical condition effectively.
Conclusion
The present case report provides an illustrative perspective on an uncommon presentation of pulmonary amyloidosis secondary to multiple myeloma, with the initial manifestation being pulmonary hemorrhage. The findings from both the physical examination and laboratory tests were consistent with pulmonary amyloidosis, and definitive confirmation of the multiple myeloma diagnosis was achieved through bone marrow biopsy. This case highlights the significance of considering pulmonary amyloidosis as a potential cause of hemoptysis, especially in patients with associated risk factors for multiple myeloma. Early recognition of this clinical association is pivotal for precise diagnosis and prompt therapeutic intervention. The complexity of this case underscores the importance of a comprehensive diagnostic approach in unraveling intricate medical conditions.
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