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Chest Imaging and Pathology for Clinicians |

A 70-Year-Old Man With Large Cervical and Mediastinal LymphadenopathiesCervical and Mediastinal Lymphadenopathy FREE TO VIEW

Shraddha Narechania, MD; Jason Valent, MD; Carol Farver, MD; Adriano R. Tonelli, MD
Author and Funding Information

From Internal Medicine (Dr Narechania), Fairview Hospital; and the Department of Hematology and Oncology (Dr Valent), the Department of Anatomic Pathology (Dr Farver), and the Department of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute (Dr Tonelli), Cleveland Clinic, Cleveland, OH.

CORRESPONDENCE TO: Adriano R. Tonelli, MD, 9500 Euclid Ave A-90, Cleveland, OH 44195; e-mail: tonella@ccf.org


FUNDING/SUPPORT: Dr Tonelli is supported by CTSA KL2 [Grant RR024990] from the National Center for Research Resources (NCRR), a component of the National Institutes of Health (NIH), and by the NIH Roadmap for Medical Research.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2015;148(1):e8-e13. doi:10.1378/chest.14-3124
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Published online

We present a case of a 70-year-old man with enlarged mediastinal and cervical lymph nodes that provided interesting radiologic and pathologic observations. The 70-year-old black man was found to have enlarged mediastinal lymph nodes. He had symptoms of atypical chest pain and generalized weakness for 2 weeks prior to the diagnosis. He denied shortness of breath, fever, chills, or night sweats. He was treated for hypertension and onychomycosis. Basic laboratory findings were within normal limits. Pulmonary function tests at the time of presentation showed FEV1, FVC, and FEV1/FVC ratio of 123% predicted, 133% predicted, and 0.7, respectively. Meanwhile, total lung capacity and carbon monoxide diffusing capacity were 103% and 107% predicted, respectively. Two weeks before he presented to our institution, the patient underwent bronchoscopy with transbronchial biopsies of the right lower lobe and endobronchial ultrasound-guided transbronchial needle aspiration of the right hilar lymph nodes.

Figures in this Article

The patient’s clinical examination was unremarkable except for a palpable right cervical lymph node of 3 cm in diameter with rubbery consistency. His lung examination was within normal limits.

Chest radiography showed a soft tissue prominence in the right suprahilar and subcarinal regions. CT scan of the chest revealed multiple enlarged homogeneous lymph nodes with fine stippled calcifications in the paratracheal, subcarinal, and right hilar regions, the largest being 3.5 cm in diameter (Fig 1). Tracheal dimensions and the appearance of the wall were normal (Fig 1F).

Figure Jump LinkFigure 1 –  A, Chest radiograph showing normal lung parenchyma and opacity at the level of the right paratracheal and hilar regions (arrows). B-E, Contrast CT scan of the chest with arrows pointing at the right cervical lymph node that underwent excisional biopsy (B), right paratracheal adenopathy (C, D), and hilar adenopathy (E). F, Coronal section at the level of the trachea in a noncontrast CT scan of the chest, done a few days before the contrast study. *Tracheal lumen.Grahic Jump Location

Interferon-γ release assay was positive; meanwhile, HIV enzyme-linked immunosorbent assay, histoplasma antigen, coccidioides antibody, syphilis IgG, and cryptococcal antigen were negative. Our initial differential diagnosis included sarcoidosis; TB or nontuberculous mycobacterial infection; lung, head, neck, or germinal cell cancer; lymphoma; histoplasmosis; and coccidioidomycosis.

Tissue slides from both bronchoscopy and endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), performed at an outside institution, were reviewed carefully. A biopsy specimen consisted of five fragments of alveolated lung parenchyma, which were unremarkable, and three fragments of bronchial wall, which showed patchy, mild, nonspecific chronic inflammation. A lymph node specimen did not show malignant cells. Gram and acid-fast bacillus stains and bacterial and mycobacterial cultures of the BAL specimen were negative.

During his first clinic visit, we performed a percutaneous biopsy of a right supraclavicular lymph node that showed inflammation and was negative for malignant cells. Flow cytometric analysis showed no immunophenotypic evidence of a lymphoproliferative disorder. Therefore, surgical lymph node excisional biopsy of the right supraclavicular lymph node was carried out. Interestingly, this biopsy specimen showed amorphous eosinophilic material that was Congo-red positive with apple-green birefringence under polarized light (Fig 2). Laser microdissection and tandem mass spectrometry of the tissue revealed λ-immunoglobulin light chains.

Figure Jump LinkFigure 2 –  Pathology examination of the right cervical lymph node (magnification × 100). A, Amyloid with Congo-red positivity. B, Apple-green birefringence with Congo-red staining.Grahic Jump Location

Further workup included a bone marrow biopsy, which showed unremarkable trilineage hematopoiesis. There was no demonstrable population of clonal plasma cells. CD138-positive plasma cells were increased slightly, but remained below 5% and had polytypic κ and λ expression. Cytogenetic karyotyping showed normal male karyotype.

What is the diagnosis?
Diagnosis: Primary amyloidosis localized to pulmonary lymph nodes without systemic involvement

Serum and urine protein electrophoresis, free light-chain assay, cardiac biomarkers, echocardiogram, skeletal survey, and bone marrow biopsy showed no evidence of systemic amyloidosis.

Amyloidosis is the term used to denote a heterogeneous group of disorders characterized by deposition of fibrillar proteins in the extracellular matrix of one or more organs. There are several forms of amyloidosis. The two major types are primary (AL) and secondary (AA) amyloidosis. AL amyloidosis, the most common form of systemic amyloidosis, is an acquired plasma cell dyscrasia characterized by deposition of monoclonal immunoglobulin light chain fragments in various organs of the body.1 AL amyloidosis is the type that most commonly affects the lungs.2-4 AA amyloidosis results from chronic inflammatory conditions that increase the acute-phase reactant serum amyloid A that then deposits as amyloid fibrils.5 This form most commonly affects the kidneys.5 Another particular and less common type of amyloidosis is caused by transthyretin (prealbumin) deposition. This condition can be (1) age related,6 with deposition of this thyroxine and retinol carrier protein particularly in the heart, or (2) familial, with accumulation of the mutant protein in the peripheral nervous system and heart.7 Because the mutant protein is produced in the liver, liver transplant can prevent progression of the disease in the familial group.7,8

Pulmonary amyloidosis can present as part of a widespread process or be localized to the respiratory tract.9 It is classified based on the site where amyloid deposits (Table 1): (1) trachea-bronchial, the most common; (2) diffuse interstitial, diffuse parenchymal, or alveolar-septal disease, characterized by amyloid deposition in the pulmonary interstitium; (3) nodular disease, in which amyloid forms nodules (amyloidomas); (4) intra- and extrathoracic adenopathy; (5) pleural disease; (6) laryngeal disease; and, rarely, (7) diaphragmatic disease.2,9 Localized lymph node amyloidosis is rare4,10-12 because the lung parenchyma is usually affected.13

Table Graphic Jump Location
TABLE 1 ]  Salient Features of Different Pulmonary Forms of Amyloidosis
Clinical Discussion

The signs and symptoms of pulmonary amyloidosis are nonspecific. A common presentation includes shortness of breath, cough, expectoration, hemoptysis, and chest tightness. Dyspnea and cough are the most frequent manifestations of tracheobronchial amyloidosis. In this condition, plaques in the tracheobronchial tree may cause tracheal stenosis or distal airway obstruction, resulting in recurrent pneumonia or postobstructive atelectasis.14 Nodular amyloidosis is usually asymptomatic but may be associated with recurrent pneumonia, atelectasis, and respiratory failure.9,14

Respiratory failure is more likely to occur in the diffuse alveolar-septal form, which portends a worse prognosis. Rare manifestations include recurrent pleural effusions,15 pulmonary hypertension,16 and pulmonary infarction with hemoptysis caused by amyloid deposition in the pulmonary blood vessels.17 Laryngeal amyloidosis is also uncommon and is characterized by hoarseness, stridor, and dysphagia.18 Pulmonary function testing varies according to presentation, from normal to obstructive (tracheobronchial amyloidosis) or restrictive patterns (diffuse alveoloseptal disease or pleural effusions).9 Proximal airways disease can present as fixed airway obstruction on flow-volume loops.

Radiologic Discussion

The imaging characteristics of amyloidosis are nonspecific. Chest radiographs are normal in most cases. CT scan findings include nodular and reticular opacities, diffuse and irregular airway wall thickening with calcifications and luminal narrowing, and pleural plaques.19 Tracheobronchial amyloidosis may cause tracheal stenosis or postobstructive atelectasis.20 Nodular amyloidosis is associated with peripheral subpleural nodular densities of variable size (0.5 mm to 5 cm) with sharp, lobulated margins and calcifications.21,22 These nodules are generally localized to the lower lobes. The alveolar-septal form of amyloidosis is characterized by interlobular septal thickening, diffuse interstitial or alveolar infiltrates, and, rarely, diffuse parenchymal nodules.23,24 In lymph node amyloidosis, the mediastinal and hilar lymph nodes are enlarged and may show speckled calcification.24,25 Scintigraphy with radioisotope-labeled amyloid P component evaluates the extent and distribution of amyloid deposits in the body26 and has a sensitivity and specificity for diagnosing AL or AA amyloidosis of 90% and 93%, respectively.2718F-fludeoxyglucose PET scanning has been found to be positive in patients with intrathoracic amyloid.28 In patients with biopsy-proven AL pulmonary amyloidosis, PET scan positivity varies from 30% to 70%29,30 with standardized uptake values ranging from 1.8 to 6.8.999mTc-DPD (3,3-diphosphono-1,2-propanodicarboxylic acid) scintigraphy specifically detects transthyretin-related cardiac amyloidosis.31

Pathologic Discussion

Amyloidosis is the pathologic accumulation of extracellular proteins in a β-pleated configuration. Biopsies of the airways, lung parenchyma, and/or mediastinal lymph nodes are required for the diagnosis of pulmonary amyloidosis. The yield of EBUS-TBNA and percutaneous or transbronchial fine-needle aspiration for the diagnosis of thoracic amyloidosis is unknown because the data are limited. There are few case reports of mediastinal amyloidosis diagnosed by EBUS-TBNA32,33 or amyloid pulmonary nodules diagnosed by fine-needle aspiration.4,34-36

Amyloid appears as an amorphous eosinophilic waxy material on hematoxylin and eosin staining. It typically shows apple-green birefringence under polarized microscopy with Congo-red staining and intense yellow-green fluorescence on thioflavin T stain. Amyloidosis must be distinguished from amyloid-like deposits, which are made of amorphous eosinophilic material but show negative histochemical and immunohistochemical stains for amyloid. Amyloid-like deposits are frequently, but not always, associated with nonamyloid immunoglobulin light-chain deposition disease.37 Amyloidomas consist of well-circumscribed dense amorphous eosinophilic amyloid protein, surrounded by an inflammatory cell infiltrate that consists of plasma cells and lymphocytes with an occasional granulomatous reaction.38

The type of amyloidosis is determined by immunohistochemical staining of the biopsy specimen. κ or Λ chains on immunofluorescence microscopy are seen in AL amyloidosis. AA amyloidosis shows positive staining of the serum amyloid A component. Laser microdissection of the amyloid-containing tissue with subsequent tandem mass spectrometry is the most accurate method for identifying the subtype of amyloid deposition. Using laser microdissection, cells of interest are isolated from Congo-red-stained paraffin sections placed on glass microscopic slides. The amyloid type is identified with mass spectrometry-based proteomic analysis, a technique that has a very high sensitivity and specificity (both ≥ 98%).39 Chromosomal abnormalities, such as monosomy of chromosome 18 (most common) and trisomy of chromosomes 7, 9, 11, 18, and X, are seen frequently in patients with AL amyloidosis.40

Treatment varies widely from conservative to aggressive interventions. The nodular form of pulmonary amyloidosis is generally asymptomatic and can be managed conservatively by observation. In these patients, an indolent B-cell lymphoproliferative disorder must be excluded.41 The diffuse alveolar-septal form of pulmonary amyloidosis has a poor prognosis and often requires systemic chemotherapy or hematopoietic stem cell transplant. If the patient is not a candidate for stem cell transplant, a combination of melphalan and high-dose dexamethasone is known to improve survival.42 Local bronchoscopic treatment with balloon dilation, stent placement, laser surgery, or radiation therapy is indicated for tracheobronchial amyloidosis. Localized lymph node amyloidosis is usually managed conservatively in asymptomatic patients; however, in symptomatic patients, surgical debulking, laser, or radiation therapy can be used.43 Pleural amyloidosis with effusions is managed by repeated thoracentesis, chest tube placement, and pleurodesis.

The diagnosis of respiratory amyloidosis is challenging because of its nonspecific clinical and radiologic presentation; therefore, surgical biopsy is necessary. Because the patient had symptoms (chest pain/dysphagia), he received oral dexamethasone, with good symptomatic response. The patient’s latent TB was treated simultaneously with isoniazid for 9 months.

Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Role of sponsors: NIH only provided salary support for Adriano R. Tonelli.

Other contributions: CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

Merlini G, Comenzo RL, Seldin DC, Wechalekar A, Gertz MA. Immunoglobulin light chain amyloidosis. Expert Rev Hematol. 2014;7(1):143-156. [CrossRef] [PubMed]
 
Berk JL, O’Regan A, Skinner M. Pulmonary and tracheobronchial amyloidosis. Semin Respir Crit Care Med. 2002;23(2):155-165. [CrossRef] [PubMed]
 
Utz JP, Swensen SJ, Gertz MA. Pulmonary amyloidosis. The Mayo Clinic experience from 1980 to 1993. Ann Intern Med. 1996;124(4):407-413. [CrossRef] [PubMed]
 
Hiller N, Fisher D, Shmesh O, Gottschalk-Sabag S, Dollberg M. Primary amyloidosis presenting as an isolated mediastinal mass: diagnosis by fine needle biopsy. Thorax. 1995;50(8):908-909. [CrossRef] [PubMed]
 
Pinney JH, Lachmann HJ. Systemic AA amyloidosis. Subcell Biochem. 2012;65:541-564. [PubMed]
 
Westermark P, Bergström J, Solomon A, Murphy C, Sletten K. Transthyretin-derived senile systemic amyloidosis: clinicopathologic and structural considerations. Amyloid. 2003;10(suppl 1):48-54. [PubMed]
 
Sekijima Y. Recent progress in the understanding and treatment of transthyretin amyloidosis. J Clin Pharm Ther. 2014;39(3):225-233. [CrossRef] [PubMed]
 
Benson MD. Liver transplantation and transthyretin amyloidosis. Muscle Nerve. 2013;47(2):157-162. [CrossRef] [PubMed]
 
Lund ME, Bakaya P, Hoag JB. Pulmonary Manifestations of Amyloidosis.. In:Güvenç IA., ed. Amyloidosis - An Insight to Disease of Systems and Novel Therapies. Rijeka, Croatia: InTech; 2011.
 
Matsuguma H, Suzuki H, Ishikawa Y, et al. Localized mediastinal lymph node amyloidosis showing an unusual unsynchronized pattern of enlargement and calcification on serial CT. Br J Radiol. 2008;81(969):e228-e230. [CrossRef] [PubMed]
 
Jenkins MC, Potter M. Calcified pseudotumoural mediastinal amyloidosis. Thorax. 1991;46(9):686-687. [CrossRef] [PubMed]
 
Plöckinger B, Müller MR, Eckersberger F. Isolated amyloidosis of hilar lymph nodes [in German]. Langenbecks Arch Chir. 1993;378(3):167-170. [CrossRef] [PubMed]
 
Naschitz JE, Yeshurun D, Pick AI. Intrathoracic amyloid lymphadenopathy. Respiration. 1986;49(1):73-76. [CrossRef] [PubMed]
 
Pitz MW, Gibson IW, Johnston JB. Isolated pulmonary amyloidosis: case report and review of the literature. Am J Hematol. 2006;81(3):212-213. [CrossRef] [PubMed]
 
Berk JL. Pleural effusions in systemic amyloidosis. Curr Opin Pulm Med. 2005;11(4):324-328. [CrossRef] [PubMed]
 
Eder L, Zisman D, Wolf R, Bitterman H. Pulmonary hypertension and amyloidosis—an uncommon association: a case report and review of the literature. J Gen Intern Med. 2007;22(3):416-419. [CrossRef] [PubMed]
 
Chung JH, Sharma A, Mino-Kenudson M, Lanuti M, Shepard JA, Digumarthy SR. Amyloidosis presenting as pulmonary infarcts: a case report. J Thorac Imaging. 2010;25(4):W138-140.
 
Gaurav K, Panda M. An uncommon cause of bilateral pulmonary nodules in a long-term smoker. J Gen Intern Med. 2007;22(11):1617-1620. [CrossRef] [PubMed]
 
Urban BA, Fishman EK, Goldman SM, et al. CT evaluation of amyloidosis: spectrum of disease. Radiographics. 1993;13(6):1295-1308. [CrossRef] [PubMed]
 
Gilad R, Milillo P, Som PM. Severe diffuse systemic amyloidosis with involvement of the pharynx, larynx, and trachea: CT and MR findings. AJNR Am J Neuroradiol. 2007;28(8):1557-1558. [CrossRef] [PubMed]
 
Xu L, Cai BQ, Zhong X, Zhu YJ. Respiratory manifestations in amyloidosis. Chin Med J (Engl). 2005;118(24):2027-2033. [PubMed]
 
Ozer Simsek Z, Oymak FS, Tutar N, Canoz O, Demir R. A rare cause of diffuse parenchymal lung disease together with granulomatous reaction: pulmonary amyloidosis. Case Rep Pulmonol. 2013;2013:837190. [PubMed]
 
Kim HY, Im JG, Song KS, et al. Localized amyloidosis of the respiratory system: CT features. J Comput Assist Tomogr. 1999;23(4):627-631. [CrossRef] [PubMed]
 
Graham CM, Stern EJ, Finkbeiner WE, Webb WR. High-resolution CT appearance of diffuse alveolar septal amyloidosis. AJR Am J Roentgenol. 1992;158(2):265-267. [CrossRef] [PubMed]
 
Wilson SR, Sanders DE, Delarue NC. Intrathoracic manifestations of amyloid disease. Radiology. 1976;120(2):283-289. [CrossRef] [PubMed]
 
Hawkins PN. Serum amyloid P component scintigraphy for diagnosis and monitoring amyloidosis. Curr Opin Nephrol Hypertens. 2002;11(6):649-655. [CrossRef] [PubMed]
 
Hazenberg BP, van Rijswijk MH, Piers DA, et al. Diagnostic performance of 123I-labeled serum amyloid P component scintigraphy in patients with amyloidosis. Am J Med. 2006;119(4):355.e315-355.e324. [CrossRef]
 
Grubstein A, Shitrit D, Sapir EE, Cohen M, Kramer MR. Pulmonary amyloidosis: detection with PET-CT. Clin Nucl Med. 2005;30(6):420-421. [CrossRef] [PubMed]
 
Baqir M, Lowe V, Yi ES, Ryu JH. 18F-FDG PET scanning in pulmonary amyloidosis. J Nucl Med. 2014;55(4):565-568. [CrossRef] [PubMed]
 
Mekinian A, Jaccard A, Soussan M, et al; Centre de Référence des Amyloses immunoglobulinémiques et autres maladies liées aux dépots des immunoglobulines monoclonales. 18F-FDG PET/CT in patients with amyloid light-chain amyloidosis: case-series and literature review. Amyloid. 2012;19(2):94-98. [CrossRef] [PubMed]
 
Rapezzi C, Guidalotti P, Salvi F, Riva L, Perugini E. Usefulness of99mTc-DPD scintigraphy in cardiac amyloidosis. J Am Coll Cardiol. 2008;51(15):1509-1510. [CrossRef] [PubMed]
 
Ishiguro T, Takayanagi N, Katoh N, et al. Waldenström’s macroglobulinemia accompanying systemic amyloidosis: the usefulness of endobronchial ultrasound-guided transbronchial needle aspiration for detecting amyloid deposits. Intern Med. 2014;53(24):2789-2793. [CrossRef] [PubMed]
 
Khor YH, Steinfort DP, Buchanan MR, Gunawardana D, Antippa P, Irving LB. A 69-year-old smoker with mediastinal and hilar lymphadenopathy. Thorax. 2010;65(2):132-138. [CrossRef] [PubMed]
 
Yang MC, Blutreich A, Das K. Nodular pulmonary amyloidosis with an unusual protein composition diagnosed by fine-needle aspiration biopsy: a case report. Diagn Cytopathol. 2009;37(4):286-289. [CrossRef] [PubMed]
 
Dundore PA, Aisner SC, Templeton PA, Krasna MJ, White CS, Seidman JD. Nodular pulmonary amyloidosis: diagnosis by fine-needle aspiration cytology and a review of the literature. Diagn Cytopathol. 1993;9(5):562-564. [CrossRef] [PubMed]
 
Kitamura H, Kobayashi T, Kaneko M, et al. Pulmonary amyloidosis diagnosed by CT-guided transbronchial biopsy: a case report. Jpn J Clin Oncol. 2001;31(5):209-211. [CrossRef] [PubMed]
 
Khoor A, Myers JL, Tazelaar HD, Kurtin PJ. Amyloid-like pulmonary nodules, including localized light-chain deposition: clinicopathologic analysis of three cases. Am J Clin Pathol. 2004;121(2):200-204. [CrossRef] [PubMed]
 
Dacic S, Colby TV, Yousem SA. Nodular amyloidoma and primary pulmonary lymphoma with amyloid production: a differential diagnostic problem. Mod Pathol. 2000;13(9):934-940. [CrossRef] [PubMed]
 
Vrana JA, Gamez JD, Madden BJ, Theis JD, Bergen HR III, Dogan A. Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens. Blood. 2009;114(24):4957-4959. [CrossRef] [PubMed]
 
Fonseca R, Ahmann GJ, Jalal SM, et al. Chromosomal abnormalities in systemic amyloidosis. Br J Haematol. 1998;103(3):704-710. [CrossRef] [PubMed]
 
Grogg KL, Aubry MC, Vrana JA, Theis JD, Dogan A. Nodular pulmonary amyloidosis is characterized by localized immunoglobulin deposition and is frequently associated with an indolent B-cell lymphoproliferative disorder. Am J Surg Pathol. 2013;37(3):406-412. [CrossRef] [PubMed]
 
Lachmann HJ, Wechalekar AD, Gillmore JD. High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosis. N Engl J Med. 2008;358(1):91-92. [CrossRef] [PubMed]
 
Fu J, Seldin DC, Berk JL, et al. Lymphadenopathy as a manifestation of amyloidosis: a case series. Amyloid. 2014;21(4):256-260. [CrossRef] [PubMed]
 

Figures

Figure Jump LinkFigure 1 –  A, Chest radiograph showing normal lung parenchyma and opacity at the level of the right paratracheal and hilar regions (arrows). B-E, Contrast CT scan of the chest with arrows pointing at the right cervical lymph node that underwent excisional biopsy (B), right paratracheal adenopathy (C, D), and hilar adenopathy (E). F, Coronal section at the level of the trachea in a noncontrast CT scan of the chest, done a few days before the contrast study. *Tracheal lumen.Grahic Jump Location
Figure Jump LinkFigure 2 –  Pathology examination of the right cervical lymph node (magnification × 100). A, Amyloid with Congo-red positivity. B, Apple-green birefringence with Congo-red staining.Grahic Jump Location

Tables

Table Graphic Jump Location
TABLE 1 ]  Salient Features of Different Pulmonary Forms of Amyloidosis

References

Merlini G, Comenzo RL, Seldin DC, Wechalekar A, Gertz MA. Immunoglobulin light chain amyloidosis. Expert Rev Hematol. 2014;7(1):143-156. [CrossRef] [PubMed]
 
Berk JL, O’Regan A, Skinner M. Pulmonary and tracheobronchial amyloidosis. Semin Respir Crit Care Med. 2002;23(2):155-165. [CrossRef] [PubMed]
 
Utz JP, Swensen SJ, Gertz MA. Pulmonary amyloidosis. The Mayo Clinic experience from 1980 to 1993. Ann Intern Med. 1996;124(4):407-413. [CrossRef] [PubMed]
 
Hiller N, Fisher D, Shmesh O, Gottschalk-Sabag S, Dollberg M. Primary amyloidosis presenting as an isolated mediastinal mass: diagnosis by fine needle biopsy. Thorax. 1995;50(8):908-909. [CrossRef] [PubMed]
 
Pinney JH, Lachmann HJ. Systemic AA amyloidosis. Subcell Biochem. 2012;65:541-564. [PubMed]
 
Westermark P, Bergström J, Solomon A, Murphy C, Sletten K. Transthyretin-derived senile systemic amyloidosis: clinicopathologic and structural considerations. Amyloid. 2003;10(suppl 1):48-54. [PubMed]
 
Sekijima Y. Recent progress in the understanding and treatment of transthyretin amyloidosis. J Clin Pharm Ther. 2014;39(3):225-233. [CrossRef] [PubMed]
 
Benson MD. Liver transplantation and transthyretin amyloidosis. Muscle Nerve. 2013;47(2):157-162. [CrossRef] [PubMed]
 
Lund ME, Bakaya P, Hoag JB. Pulmonary Manifestations of Amyloidosis.. In:Güvenç IA., ed. Amyloidosis - An Insight to Disease of Systems and Novel Therapies. Rijeka, Croatia: InTech; 2011.
 
Matsuguma H, Suzuki H, Ishikawa Y, et al. Localized mediastinal lymph node amyloidosis showing an unusual unsynchronized pattern of enlargement and calcification on serial CT. Br J Radiol. 2008;81(969):e228-e230. [CrossRef] [PubMed]
 
Jenkins MC, Potter M. Calcified pseudotumoural mediastinal amyloidosis. Thorax. 1991;46(9):686-687. [CrossRef] [PubMed]
 
Plöckinger B, Müller MR, Eckersberger F. Isolated amyloidosis of hilar lymph nodes [in German]. Langenbecks Arch Chir. 1993;378(3):167-170. [CrossRef] [PubMed]
 
Naschitz JE, Yeshurun D, Pick AI. Intrathoracic amyloid lymphadenopathy. Respiration. 1986;49(1):73-76. [CrossRef] [PubMed]
 
Pitz MW, Gibson IW, Johnston JB. Isolated pulmonary amyloidosis: case report and review of the literature. Am J Hematol. 2006;81(3):212-213. [CrossRef] [PubMed]
 
Berk JL. Pleural effusions in systemic amyloidosis. Curr Opin Pulm Med. 2005;11(4):324-328. [CrossRef] [PubMed]
 
Eder L, Zisman D, Wolf R, Bitterman H. Pulmonary hypertension and amyloidosis—an uncommon association: a case report and review of the literature. J Gen Intern Med. 2007;22(3):416-419. [CrossRef] [PubMed]
 
Chung JH, Sharma A, Mino-Kenudson M, Lanuti M, Shepard JA, Digumarthy SR. Amyloidosis presenting as pulmonary infarcts: a case report. J Thorac Imaging. 2010;25(4):W138-140.
 
Gaurav K, Panda M. An uncommon cause of bilateral pulmonary nodules in a long-term smoker. J Gen Intern Med. 2007;22(11):1617-1620. [CrossRef] [PubMed]
 
Urban BA, Fishman EK, Goldman SM, et al. CT evaluation of amyloidosis: spectrum of disease. Radiographics. 1993;13(6):1295-1308. [CrossRef] [PubMed]
 
Gilad R, Milillo P, Som PM. Severe diffuse systemic amyloidosis with involvement of the pharynx, larynx, and trachea: CT and MR findings. AJNR Am J Neuroradiol. 2007;28(8):1557-1558. [CrossRef] [PubMed]
 
Xu L, Cai BQ, Zhong X, Zhu YJ. Respiratory manifestations in amyloidosis. Chin Med J (Engl). 2005;118(24):2027-2033. [PubMed]
 
Ozer Simsek Z, Oymak FS, Tutar N, Canoz O, Demir R. A rare cause of diffuse parenchymal lung disease together with granulomatous reaction: pulmonary amyloidosis. Case Rep Pulmonol. 2013;2013:837190. [PubMed]
 
Kim HY, Im JG, Song KS, et al. Localized amyloidosis of the respiratory system: CT features. J Comput Assist Tomogr. 1999;23(4):627-631. [CrossRef] [PubMed]
 
Graham CM, Stern EJ, Finkbeiner WE, Webb WR. High-resolution CT appearance of diffuse alveolar septal amyloidosis. AJR Am J Roentgenol. 1992;158(2):265-267. [CrossRef] [PubMed]
 
Wilson SR, Sanders DE, Delarue NC. Intrathoracic manifestations of amyloid disease. Radiology. 1976;120(2):283-289. [CrossRef] [PubMed]
 
Hawkins PN. Serum amyloid P component scintigraphy for diagnosis and monitoring amyloidosis. Curr Opin Nephrol Hypertens. 2002;11(6):649-655. [CrossRef] [PubMed]
 
Hazenberg BP, van Rijswijk MH, Piers DA, et al. Diagnostic performance of 123I-labeled serum amyloid P component scintigraphy in patients with amyloidosis. Am J Med. 2006;119(4):355.e315-355.e324. [CrossRef]
 
Grubstein A, Shitrit D, Sapir EE, Cohen M, Kramer MR. Pulmonary amyloidosis: detection with PET-CT. Clin Nucl Med. 2005;30(6):420-421. [CrossRef] [PubMed]
 
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