Amyloidosis and the lung

Lachmann, HJ; Hawkins, Philip N.

doi:10.1177/1479972306070066

Cited by 62 publications

(47 citation statements)

References 122 publications

(128 reference statements)

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“…Pulmonary amyloidosis is usually due to primary amyloidosis with deposition of immunoglobulin light-chain fragments (5). A broad array of intrathoracic manifestations is associated with amyloidosis, ranging from tracheobronchial parenchymal (consolidation, nodular, cystic, and interstitial) and pleural involvement to mediastinal and hilar lymphadenopathy (4).…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary involvement in amyloidosis can be classified as tracheobronchial disease, parenchymal nodules, localized or diffuse interstitial infiltrates, intrathoracic lymphadenopathy, and pleural disease (4,5). Pulmonary amyloidosis is usually due to primary amyloidosis and can be difficult to diagnose (4,5).…”

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confidence: 99%

“…Pulmonary amyloidosis is usually due to primary amyloidosis and can be difficult to diagnose (4,5). Sometimes the presentation can resemble a malignancy, particularly when it presents with pulmonary nodules, intrathoracic lymphadenopathy, or pleural effusion.…”

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confidence: 99%

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¹⁸F-FDG PET Scanning in Pulmonary Amyloidosis

Baqir

Lowe

et al. 2014

J Nucl Med

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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¹⁸F-FDG PET Scanning in Pulmonary Amyloidosis

Baqir

Lowe

et al. 2014

J Nucl Med

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“…Pulmonary involvement in systemic AL is probably underdiagnosed and, as in this case, can cause reduction in diffusing capacity. 81 The decision was made to treat with both rituximab and MDex initially and proceed to a standard BEAM SCT after mobilization. High-dose melphalan may have worked as well, although support for the efficacy of BEAM SCT for lymphoma is substantial.…”

Section: Commentsmentioning

confidence: 99%

How I treat amyloidosis

Comenzo

2009

Blood

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Amyloidosis is an uncommon disorder in which proteins change conformation, aggregate, and form fibrils that infiltrate tissues, leading to organ failure and death. The most frequent types are light-chain (AL) derived from monoclonal B-cell disorders producing amyloidogenic immunoglobulin light chains, and the hereditary and "senile systemic" (ATTR) variants from mutant and wild-type transthyretin (TTR). Diagnosis requires tissue biopsy. AL is more frequent and causes more organ disease than ATTR. Although both can cause cardiomyopathy and heart failure, AL progresses more quickly, so survival depends on timely diagnosis. Typing is usually based on clinical and laboratory findings with monoclonal gammopathy evaluation and, if indicated, TTR gene testing. Direct tissue typing is required when one patient has 2 potential amyloid-forming proteins. In coming years, widespread use of definitive proteomics will improve typing. New therapies are in testing for ATTR, whereas those for AL have followed multiple myeloma, leading to improved survival. Challenges of diagnosing and caring for patients with amyloidosis include determination of type, counseling, and delivery of prompt therapy often while managing multisystem disease. Recent advances grew from clinical research and advocacy in many countries, and global husbandry of such efforts will reap future benefits for families and patients with amyloidosis. (Blood. 2009;114:3147-3157) IntroductionLess than 50 years ago, the composition of AL amyloid fibrils was a matter of controversy. [1][2][3] The connection between a monoclonal gammopathy (itself a powerful concept of that era) and amyloid was not clearly comprehended. 4 Investigators were caught in a logical conundrum: amyloid fibrils were composed of an aberrant protein but not always of the same one. 5 There were different types of amyloid as in Table 1. 6 Since that time, investigators developed a taxonomy based on the several dozen precursor proteins that cause amyloidosis. We now confront the problem of diagnostic confidence in typing amyloid, knowing that different therapies exist for different types. [7][8][9] We also struggle with the core clinical issue: the heart of the matter is that amyloidosis often attacks the heart. 10 How I treat amyloidosis begins with typing the disease, determining the extent of organ (particularly heart) involvement, and deciding on appropriate therapy, whenever feasible on a clinical trial. Effective therapy requires a comprehensive approach, monitoring both the precursor protein we seek to reduce or eliminate and the markers of organ disease, while providing best supportive care. In this report, I use a case-and-comments approach focused on AL, the type afflicting 10 patients per million personyears, including 10% to 15% of patients with myeloma or Waldenström macroglobulinemia. 11Case 1: a woman with hepatosplenomegaly, proteinuria, and bleeding A 49-year-old woman presented with early satiety, right upper quadrant fullness, hepatosplenomegaly, alkaline phosphatase 380 U/L (norm...

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“…16 Tracheobronchial amyloidosis is characterized by amyloid deposits in the trachea and large bronchi, with extension at times into segmental bronchi. 17 Tracheal stenosis often results from thickening of the tracheal wall. Any inflammatory or granulomatous process demonstrates similar features as amyloid on CT. 18,19 However, on MR imaging, the signal intensities mentioned above should suggest the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Severe Diffuse Systemic Amyloidosis with Involvement of the Pharynx, Larynx, and Trachea: CT and MR Findings

Gilad¹,

Milillo²,

Som³

2007

American Journal of Neuroradiology

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SUMMARY:Amyloidosis is a term applied to a diverse group of disorders that share the deposition of amyloid protein in various extracellular tissues. Systemic amyloidosis may involve almost any organ system in the body including regions in the head and neck; however, pharyngeal involvement is rare, with only 12 cases having been previously reported. Ten of these cases were localized disease, and only 2 cases were systemic amyloidosis. We present the case of a patient with severe diffuse systemic amyloidosis with extensive involvement of the pharynx, larynx, trachea, lungs, eyelids, and breasts. We also review the imaging characteristics and pertinent literature.A myloidosis is a rare, infiltrative condition characterized by deposition of abnormal protein in various tissues. When systemic amyloidosis involves regions of the head and neck, the larynx and trachea are the most common sites. To date, there have been only 2 prior reported cases of systemic amyloidosis involving the pharynx. We present a case of severe diffuse systemic amyloidosis in the chest, head and neck, with extensive involvement of the pharynx and we review the literature. Case ReportThe patient was a 64-year-old woman with a history of myasthenia gravis for 30 years, type 1 diabetes mellitus, and amyloidosis for 4 years. In the past, the patient was found to have amyloid deposits in the eyelids and breasts. A biopsy confirmed findings consistent with the AL type (amyloid light chain) of amyloidosis. The patient presented with increased shortness of breath and hoarseness on the most recent hospital admission. There was no past medical history of renal failure or multiple myeloma. The patient was being treated for myasthenia gravis with pyridostigmine (Mestinon) and high-dose steroids and had received plasmapheresis therapy in the past. Physical examination was significant for bilateral ptoses and indurations of the eyelids, pharyngeal fullness, shortness of breath, and a decreased range of motion of the neck. Her urine was negative for Bence-Jones protein, and the results of serum protein and urine protein electrophoreses were unremarkable.A tracheostomy was performed, and a high-resolution chest CT study showed extensive interstitial lung disease, abnormally attenuated breast tissue (Fig 1) for the patient's stated age, and small nodules in the tracheal mucosa with marked thickening of the tracheal wall with significant stenosis (not shown). An endoscopic examination of the trachea revealed nodular deposits and plaques on the mucosal surface along its entire length. Noncontrast MR imaging of the neck was performed on a 1.5T magnet. Contrast was not administered because of a reported allergy to gadolinium. The images showed large submucosal, nodular masses in the pharynx and supraglottic larynx, and thickening of the tracheal wall with stenosis. These masses had intermediate T1-weighted signal intensity (Figs 2A) and low T2-weighted signal intensity ( Figs 2B, C, D). The large submucosal supraglottic laryngeal deposits were the primary cause of h...

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Amyloidosis and the lung

Cited by 62 publications

References 122 publications

¹⁸F-FDG PET Scanning in Pulmonary Amyloidosis

¹⁸F-FDG PET Scanning in Pulmonary Amyloidosis

How I treat amyloidosis

Severe Diffuse Systemic Amyloidosis with Involvement of the Pharynx, Larynx, and Trachea: CT and MR Findings

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Amyloidosis and the lung

Cited by 62 publications

References 122 publications

18F-FDG PET Scanning in Pulmonary Amyloidosis

18F-FDG PET Scanning in Pulmonary Amyloidosis

How I treat amyloidosis

Severe Diffuse Systemic Amyloidosis with Involvement of the Pharynx, Larynx, and Trachea: CT and MR Findings

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Resources

About

¹⁸F-FDG PET Scanning in Pulmonary Amyloidosis

¹⁸F-FDG PET Scanning in Pulmonary Amyloidosis