Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.
ADVANCED SEARCH
Log in
Top
Journal of General Internal Medicine
Published in: Journal of General Internal Medicine 3/2007

01-03-2007 | Case Reports/Clinical Vignettes

Pulmonary Hypertension and Amyloidosis—an Uncommon Association: A Case Report and Review of the Literature

Authors: Lihi Eder, MD, Devy Zisman, MD, Raffael Wolf, MD, Haim Bitterman, MD

Published in: Journal of General Internal Medicine | Issue 3/2007

Login to get access

Abstract

Only a limited number of patients with amyloidosis and pulmonary hypertension have been reported in the literature. We report a 73-year-old female with AL type amyloidosis who developed respiratory insufficiency and right heart failure because of severe pulmonary hypertension. There were no signs of cardiac involvement with amyloid or findings consistent with interstitial lung disease. Previous reports of pulmonary hypertension without an apparent parenchymal lung or myocardial involvement with amyloidosis are summarized. Pulmonary hypertension due to deposition of amyloid in the pulmonary vasculature is an uncommon finding; however, it should be considered in cases of unexplained pulmonary hypertension in patients with amyloidosis.
Literature
1.
2.
WHO-IUIS Nomenclature Sub-Committee. Nomenclature of amyloid and amyloidosis. Bull World Health Organ. 1993;71:105–12
3.
Gertz MA, Merlini G, Treon SP. Amyloidosis and Waldenstrom’s macroglobulinemia. Hematology. 2004;257–82
4.
Gertz MA, Lacy MQ, Dispenzieri A. Amyloidosis: recognition, confirmation, prognosis and therapy. Mayo Clin Proc. 1999;74:490–4PubMed
5.
Kalova I, Niessen H. Amyloid in the cardiovascular system: a review. J Clin Pathol. 2005;58:125–33CrossRef
6.
Utz JP, Swensen SJ, Gertz MA. Pulmonary amyloidosis: the Mayo Clinic experience from 1980 to 1993. Ann Intern Med. 1996;124:407–13PubMed
7.
8.
Gilmore JD, Hawkins PN. Amyloidosis and the respiratory tract. Thorax. 1999;54:444–51CrossRef
9.
10.
Galie N, Manes A, Branzi A. Evaluation of pulmonary arterial hypertension. Curr Opin Cardiol. 2004;19:575–81PubMedCrossRef
11.
Antonioli E, Nozzoli C, Gianfaldoni G, Mannelli F, Rossi S, Betti S, Bernardeschi P, Fiorentini G, Bosi A. Pulmonary hypertension related to thalidomide therapy in refractory multiple myeloma. Ann Oncol. 2005;16:1849–50PubMedCrossRef
12.
Hattori Y, Shimoda M, Okamoto S, Satoh T, Kakimoto T, Ikeda Y. Pulmonary hypertension and thalidomide therapy in multiple myeloma. Br J Haematol. 2003;121:191–2CrossRef
13.
Younis TH, Alam A, Paplham P, Spangenthal E, McCarthy P. Reversible pulmonary hypertension and thalidomide therapy for multiple myeloma. Br J Haematol. 2003;123:563CrossRef
14.
Mandegar M, Fung YC, Huang W, Remillard CV, Rubin LJ, Yuan JX. Cellular and molecular mechanisms of pulmonary vascular remodeling: role in the development of pulmonary hypertension. Microvasc Res. 2004;68:75–103PubMedCrossRef
15.
Crotty TB, Li CY, Edwards WD, Suman VJ. Amyloidosis and endomyocardial biopsy: correlation of extent and patterns of deposition with amyloid immunophenotype in 100 cases. Cardiovasc Pathol. 1994;5:39–42
16.
Smith TJ, Kyle RA, Lie JT. Clinical significance of histopathologic patterns of cardiac amyloidosis. Mayo Clin Proc. 1984;59:547–55PubMed
17.
Suwaidi JA, Velianou JL, Gertz MA, Cannon RO, Higano ST, Holmes DR. Systemic amyloidosis presenting with angina pectoris. Ann Int Med. 1999;131:838–41PubMed
18.
Crawford F, Suo Z, Frang C, et al. Characteristics of the in vitro vasoactivity of beta-amyloid peptides. Exp Neurol. 1998;150:159–68PubMedCrossRef
19.
Chapman AD, Brown PA, Kerr KM. Right heart failure as the dominant clinical picture in a case of primary amyloidosis affecting the pulmonary vasculature. Scott Med J. 1999;44:116–17PubMed
20.
Shiue ST, McNally DP. Pulmonary hypertension from prominent vascular involvement in diffuse amyloidosis. Arch Int Med. 1988;148:687–89CrossRef
21.
Dingli D, Utz JP, Gertz MA. Pulmonary hypertension in patients with amyloidosis. Chest. 2001;120:1735–8PubMedCrossRef
22.
Johnson WJ, Lie JT. Pulmonary hypertension and familial Mediterranean fever: a previously unrecognized association. Mayo Clin Proc. 1991;66:919–25PubMed
23.
Lutz AE, Scneider U, Ehlerding G, Frenzel H, Koch KM, Kuhn K. Right ventricular cardiac failure and pulmonary hypertension in a long-term dialysis patient—unusual presentation of visceral beta 2-microglobulin amyloidosis. Nephrol Dial Transplant. 1995;10:555PubMed
24.
McLaughlin WV, Shillington A. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002;106:1477PubMedCrossRef
Metadata
Title
Pulmonary Hypertension and Amyloidosis—an Uncommon Association: A Case Report and Review of the Literature
Authors
Lihi Eder, MD
Devy Zisman, MD
Raffael Wolf, MD
Haim Bitterman, MD
Publication date
01-03-2007
Publisher
Springer-Verlag
Published in
Journal of General Internal Medicine / Issue 3/2007
Print ISSN: 0884-8734
Electronic ISSN: 1525-1497
DOI
https://doi.org/10.1007/s11606-006-0052-9

Other articles of this Issue 3/2007

Journal of General Internal Medicine 3/2007 Go to the issue

Original Article

Missed Opportunities: Family History and Behavioral Risk Factors in Breast Cancer Risk Assessment Among a Multiethnic Group of Women

Health Policy

Redesigning the Practice Model for General Internal Medicine. A Proposal for Coordinated Care

Original Article

Specificity of Clinical Breast Examination in Community Practice

Editorial

Comprehending Coordinated Comprehensive Care: The Devil is in the Dollars

Populations at Risk

Perceived Discrimination and Adherence to Medical Care in a Racially Integrated Community

Original Article

Cultural Sensitivity in Screening Adults for a History of Childhood Abuse: Evidence from a Community Sample
Obesity Clinical Trial Summary

At a glance: The STEP trials

Read more

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Pediatric Cardiology Video

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

Cardiopulmonary Comorbidity Video

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Valvular Heart Disease Video

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Heart Failure Video

Watch this official video from ACC.24. Dr. Biykem Bozkurt discuss last year's major advances in heart failure and cardiomyopathies.

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Heart Failure Video

Watch this official video from ACC.24. Dr. Biykem Bozkurt discuss last year's major advances in heart failure and cardiomyopathies.

Watch now

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

16-04-2024 Type 2 Diabetes News

Incentivised to exercise

11-04-2024 Lifestyle Modifications News

New intervention for STEMI-related cardiogenic shock

10-04-2024 Myocardial Infarction News

» View more highlights on the ACC 2024 congress hub page

Image Credits