Semin Respir Crit Care Med 2002; 23(2): 155-166
DOI: 10.1055/s-2002-25304
Copyright © 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Pulmonary and Tracheobronchial Amyloidosis

John L. Berk1, 2, 3 , Anthony O'Regan1, 3 , Martha Skinner2, 3
  • 1The Pulmonary Center, Boston University School of Medicine, Boston, Massachusetts
  • 2Amyloid Treatment and Research Program, Boston University School of Medicine, Boston, Massachusetts
  • 3Department of Medicine, Boston University School of Medicine, Boston, Massachusetts
Further Information

Publication History

Publication Date:
24 April 2002 (online)

ABSTRACT

Amyloidosis is a collection of diseases in which different proteins are deposited as insoluble β-pleated sheets, disrupting organ function. Each precursor protein induces a separate spectrum of organ involvement, and different disease manifestations within the lung. Although autopsy data often demonstrate amyloid deposits in various compartments of the lung, few of the pathologic findings are expressed clinically. We review the pulmonary pathology, radiology, clinical presentations, and treatment options for each of the major systemic and localized forms of amyloidosis. This review focuses on amyloid derived from immunoglobulin light-chain protein (AL disease), which most frequently involves the lung in both systemic and localized forms of the disease. Manifestations of AL-related lung disease range from nodules identified on incidental chest films to diffuse alveolar+-septal deposition mimicking diffuse alveolar damage. We discuss respiratory failure due to diaphragm invasion, proximal tracheal disease, and diffuse alveolar-septal deposition. Guidelines for evaluation of patients with amyloid are presented.

REFERENCES

  • 1 Virchow V R. Ueber einem Gehirn und Rueckenmark des Menschen aufgefundene Substanz mit chemischen reaction der Cellulose.  Virchows Arch Pathol Anat . 1854;  6 1358
  • 2 Gertz M A. Secondary amyloidosis (AA).  J Intern Med . 1992;  232 517-518
  • 3 Celli B R, Rubinow A, Cohen A S, Brody J S. Patterns of pulmonary involvement in systemic amyloidosis.  Chest . 1978;  74 543-547
  • 4 Smith R R, Hutchins G M, Moore G W, Humphrey R L. Type and distribution of pulmonary parenchymal and vascular amyloid: correlation with cardiac amyloid.  Am J Med . 1979;  66 96-104
  • 5 Utz J P, Swensen S J, Gertz M A. Pulmonary amyloidosis: the Mayo Clinic experience from 1980 to 1993.  Ann Int Med . 1996;  124 407-413
  • 6 Johnson W J, Lie J T. Pulmonary hypertension and familial Mediterranean fever: a previously unrecognized association.  Mayo Clin Proc . 1991;  66 919-925
  • 7 Livneh A, Langevitz P, Pras M. Pulmonary associations in familial Mediterranean fever.  Curr Opin Pulm Med . 1999;  5 326-331
  • 8 Westermark P, Sletten K, Johansson B, Cornwell III G G. Fibril in senile systemic amyloidosis is derived from normal transthyretin.  Proc Natl Acad Sci U S A . 1990;  87 2843-2845
  • 9 Benson M D. Amyloidosis. In: Scriver CR, Beaudet AK, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Diseases. New York: McGraw-Hill 2000: 4159-4191
  • 10 Jacobson D R, Pastore R, Pool S. Revised transthyretin Ile 122 allele frequency in African-Americans.  Hum Genet . 1996;  98 236-238
  • 11 Kunze W P. Senile pulmonary amyloidosis.  Pathol Res Pract . 1979;  164 413-422
  • 12 Pitkanen P, Westermark P, Cornwell III G G. Senile systemic amyloidosis.  Am J Pathol . 1984;  117 391-399
  • 13 Gertz M A, Kyle R A, Thibodeau S N. Familial amyloidosis: a study of 52 North American-born patients examined during a 30-year period.  Mayo Clin Proc . 1992;  67 428-440
  • 14 Simms R W, Prout M N, Cohen A S. The epidemiology of AL and AA amyloidosis.  Baillieres Clin Rheumatol . 1994;  8 627-634
  • 15 Falk R H, Comenzo R L, Skinner M. The systemic amyloidoses: current approaches to diagnosis and treatment.  New Engl J Med . 1997;  337 898-909
  • 16 Berg A M, Troxler R F, Grillone G. Localized amyloidosis of the larynx: evidence for light chain composition.  Ann Otol Rhinol Laryngol . 1993;  102 884-889
  • 17 Miura K, Shirasawa H, Lambda III. Subgroup immunoglobulin light chains are precursor proteins of nodular pulmonary amyloidosis.  Am J Clin Pathol . 1993;  100 561-566
  • 18 Westermark P, Sletten K, Pitkanen P, Natvig J B, Lindholm C E. Localized laryngeal amyloidosis: partial characterization of an amyloid fibril protein AL.  Mol Immunol . 1982;  19 447-450
  • 19 Cordier J F, Loire R, Brune J. Amyloidosis of the lower respiratory tract: clinical and pathologic features in a series of 21 patients.  Chest . 1986;  90 827-831
  • 20 Kyle R A, Gertz M A. Primary systemic amyloidosis: clinical and laboratory features in 474 cases.  Sem Hematol . 1995;  32 45-59
  • 21 Road J D, Jacques J, Sparling J R. Diffuse alveolar septal amyloidosis presenting with recurrent hemoptysis and medial dissection of pulmonary arteries.  Am Rev Resp Dis . 1985;  132 1368-1370
  • 22 Cools F J, Kockx M M, Boeckxstaens G E, Heuvel P V, Cuykens J J. Primary systemic amyloidosis complicated by massive thrombosis.  Chest . 1996;  110 282-284
  • 23 Liu Y Y, Lee Y C, Chou T Y, Perng R P. Pulmonary alveolar-septal amyloidosis presenting as heart failure with unilateral pleural effusion: a case report.  Chung Hua I Hsueh Tsa Chih (Taipei) . 1999;  62 838-843
  • 24 Strange C, Heffner J E, Collins B S, Brown F M, Sahn S A. Pulmonary hemorrhage and air embolism complicating transbronchial biopsy in pulmonary amyloidosis.  Chest . 1987;  92 367-369
  • 25 Hui A N, Koss M N, Hochholzer L, Wehunt W D. Amyloidosis presenting in the lower respiratory tract: clinicopathologic, radiologic, immunohistochemical, and histochemical studies on 48 cases.  Arch Pathol Lab Med . 1986;  110 212-218
  • 26 Kyle R A, Bayrd E D. Amyloidosis: review of 236 cases.  Medicine (Baltimore) . 1975;  54 271-299
  • 27 Rakhit R D, Clesham G J, Cleland J G. Sarcoid, amyloid and heart failure.  Int J Cardiol . 1993;  41 180-182
  • 28 Rainfray M, Meyrier A, Valeyre D, Tazi A, Battesti J P. Renal amyloidosis complicating sarcoidosis.  Thorax . 1988;  43 422-423
  • 29 Sharma O P, Koss M, Buck F. Sarcoidosis and amyloidosis: is the association causal or co-incidental?.  Sarcoidosis . 1987;  4 139-141
  • 30 Smith F B, Brown R B, Maguire G, Oliver J. Localized pleural microdeposition of type A amyloid in a patient with rheumatoid pleuritis: histologic distinction from pleural involvement in systemic amyloidosis.  Am J Clin Pathol . 1993;  99 261-264
  • 31 Knapp M J, Roggli V L, Kim J, Moore J O, Shelburne J D. Pleural amyloidosis.  Arch Pathol Lab Med . 1988;  112 57-60
  • 32 Kavuru M S, Adamo J P, Muzaffar A, Mehta A C, Gephardt G N. Amyloidosis and pleural disease.  Chest . 1990;  98 20-23
  • 33 Lundin P, Simonsson B, Winberg T. Pneumopleural amyloid tumor.  Acta Radiol . 1961;  55 139-144
  • 34 Keane J, Vosburgh E, O'Regan A, Falk R, Berk J L. Recurrent symptomatic pleural effusion in patients with AL amyloidosis: a marker of concomitant pleural disease.  Am J Respir Crit Care Med . 1998;  157 A741.
  • 35 Graham D R, Ahmad D. Amyloidosis with pleural involvement.  Eur Respir J . 1988;  1 571-572
  • 36 Bontemps F, Tillie-Leblond I, Coppin M C. Pleural amyloidosis: thoracoscopic aspects.  Eur Respir J . 1995;  8 1025-1027
  • 37 Ikeda S, Takabayashi Y, Maejima Y. Nodular lung disease with five year survival and unilateral pleural effusion in AL amyloidosis.  Amyloid . 1999;  6 292-296
  • 38 Streeten E A, de la Monte M S, Kennedy T P. Amyloid infiltration of the diaphragm as a cause of respiratory failure.  Chest . 1986;  89 760-762
  • 39 Santiago R M, Scharnhorst D, Ratkin G, Crouch E C. Respiratory muscle weakness and ventilatory failure in AL amyloidosis with muscular pseudohypertrophy.  Am J Med . 1987;  83 175-178
  • 40 Pickford H A, Swensen S J, Utz J P. Thoracic cross-sectional imaging of amyloidosis.  Am J Roentgenol . 1997;  168 351-355
  • 41 Graham C M, Stern E J, Finkbeiner W E, Webb W R. High-resolution CT appearance of diffuse alveolar septal amyloidosis.  Am J Roentgenol . 1992;  158 265-267
  • 42 Gross B H. Radiographic manifestations of lymph node involvement in amyloidosis.  Radiology . 1981;  138 11-14
  • 43 Fukuda T, Takao M, Uchida T, Iwanaga S, Hayashi K, Okimoto T. High attenuation lymphadenopathy caused by amyloidosis.  Br J Radiol . 1997;  70 213-214
  • 44 Desai R A, Mahajan V K, Benjamin S, Van Ordstrand S H, Cordasco E M. Pulmonary amyloidoma and hilar adenopathy: rare manifestations of primary amyloidosis.  Chest . 1979;  76 170-173
  • 45 Thompson P J, Citron K M. Amyloid and the lower respiratory tract.  Thorax . 1983;  38 84-87
  • 46 Rubinow A, Celli B R, Cohen A S, Rigden B G, Brody J S. Localized amyloidosis of the lower respiratory tract.  Am Rev Resp Dis . 1978;  118 603-611
  • 47 Ayuso M C, Gilabert R, Bombi J A, Salvador A. CT appearance of localized pulmonary amyloidosis.  J Comput Assist Tomogr . 1987;  11 197-199
  • 48 Himmelfarb E, Wells S, Rabinowitz J G. The radiologic spectrum of cardiopulmonary amyloidosis.  Chest . 1977;  72 327-332
  • 49 Diaz-Jimenez J P, Rodriguez A, Ballarin J IM, Castro M J, Argemi T M, Manresa F. Diffuse tracheobronchial amyloidosis.  J Bronchol . 1999;  6 13-17
  • 50 O'Regan A, Fenlon H M, Beamis Jr F J, Steele M P, Skinner M, Berk J L. Tracheobronchial amyloidosis: the Boston University experience from 1984 to 1999.  Medicine (Baltimore) . 2000;  79 69-79
  • 51 Capizzi S A, Betancourt E, Prakash U B. Tracheobronchial amyloidosis.  Mayo Clin Proc . 2000;  75 1148-1152
  • 52 Crestani B, Monnier A, Kambouchner M, Battesti J P, Reynaud P, Valeyre D. Tracheobronchial amyloidosis with hilar lymphadenopathy associated with a serum monoclonal immunoglobulin.  Eur Respir J . 1993;  6 1569-1571
  • 53 Condon R E, Pinkham R D, Hames G H. Primary isolated nodular pulmonary amyloidosis: report of a case.  J Thorac Cardio Surg . 1964;  48 498
  • 54 Weiss L. Isolated multiple nodular pulmonary amyloidosis.  Am J Clin Pathol . 1960;  33 318
  • 55 Kirchner J, Jacobi V, Kardos P, Kollath J. CT findings in extensive tracheobronchial amyloidosis.  Eur Radiol . 1998;  8 352-354
  • 56 Skinner M, Anderson J, Simms R. Treatment of 100 patients with primary amyloidosis: a randomized trial of melphalan, prednisone, and colchicine versus colchicine only.  Am J Med . 1996;  100 290-298
  • 57 Kyle R A, Gertz M A, Greipp P R. A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine.  N Engl J Med . 1997;  336 1202-1207
  • 58 Comenzo R L, Vosburgh E, Falk R H. Dose-intensive melphalan with blood stem-cell support for the treatment of AL (amyloid light-chain) amyloidosis: survival and responses in 25 patients.  Blood . 1998;  91 3662-3670
  • 59 Breuer R, Simpson G T, Rubinow A, Skinner M, Cohen A S. Tracheobronchial amyloidosis: treatment by carbon dioxide laser photoresection.  Thorax . 1985;  40 870-871
  • 60 Flemming A F, Fairfax A J, Arnold A G, Lane D J. Treatment of endobronchial amyloidosis by intermittent bronchoscopic resection.  Brit J Dis Chest . 1980;  74 183-188
  • 61 Thompson P J, Ryan G, Laurence B H. Laser photoradiation therapy for tracheobronchial amyloid.  Austra New Zeal J Med . 1986;  16 229-230
  • 62 Yap J C, Wang Y T, Poh S C. A case of primary diffuse tracheobronchial amyloidosis treated by laser therapy.  Sing Med J . 1992;  33 198-200
  • 63 Shapiro S J, Shapiro S D, Mill W B, Campbell E J. Prospective study of long-term pulmonary manifestations of mantle irradiation.  Int J Radiat Oncol Biol Phys . 1990;  19 707-714
  • 64 Kurrus J A, Hayes J K, Hoidal J R, Menendez M M, Elstad M R. Radiation therapy for tracheobronchial amyloidosis.  Chest . 1998;  114 1489-1492
  • 65 Shinoi K, Shirai-Shi U, Yahata J. Amyloid tumor of the trachea and lung resembling bronchial asthma.  Dis Chest . 1962;  42 442-445
  • 66 Rajan K G, Reynolds S P, McConnochie K, White J P. Localised amyloid-presenting as bronchial asthma.  Eur J Respir Dis . 1987;  71 213-215
  • 67 McDonald D R, Brunden K R, Landreth G E. Amyloid fibrils activate tyrosine kinase-dependent signaling and superoxide production in microglia.  J Neurosci . 1997;  17 2284-2294
  • 68 Tennent G A, Lovat L B, Pepys M B. Serum amyloid P component prevents proteolysis of the amyloid fibrils of Alzheimer disease and systemic amyloidosis.  Proc Natl Acad Sci U S A . 1995;  92 4299-4303
    >