Skip to main content

Advertisement

SpringerLink
Log in

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

  • Case Reports/Clinical Vignettes
  • Published:
Journal of General Internal Medicine Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

Reference

  1. Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med. 1997;337:898–909

    Article  PubMed  CAS  Google Scholar 

  2. WHO-IUIS Nomenclature Sub-Committee. Nomenclature of amyloid and amyloidosis. Bull World Health Organ. 1993;71:105–12

    Google Scholar 

  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;74:490–4

    PubMed  CAS  Google Scholar 

  5. Kalova I, Niessen H. Amyloid in the cardiovascular system: a review. J Clin Pathol. 2005;58:125–33

    Article  Google Scholar 

  6. Utz JP, Swensen SJ, Gertz MA. Pulmonary amyloidosis: the Mayo Clinic experience from 1980 to 1993. Ann Intern Med. 1996;124:407–13

    PubMed  CAS  Google Scholar 

  7. Thompson PJ, Citron KM. Amyloid and the lower respiratory tract. Thorax. 1983;38:84–7

    Article  PubMed  CAS  Google Scholar 

  8. Gilmore JD, Hawkins PN. Amyloidosis and the respiratory tract. Thorax. 1999;54:444–51

    Article  Google Scholar 

  9. Farber HW, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med. 2004;351:1655–65

    Article  PubMed  CAS  Google Scholar 

  10. Galie N, Manes A, Branzi A. Evaluation of pulmonary arterial hypertension. Curr Opin Cardiol. 2004;19:575–81

    Article  PubMed  Google Scholar 

  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;16:1849–50

    Article  PubMed  CAS  Google Scholar 

  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;121:191–2

    Article  Google Scholar 

  13. Younis TH, Alam A, Paplham P, Spangenthal E, McCarthy P. Reversible pulmonary hypertension and thalidomide therapy for multiple myeloma. Br J Haematol. 2003;123:563

    Article  Google Scholar 

  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;68:75–103

    Article  PubMed  CAS  Google Scholar 

  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

    Google Scholar 

  16. Smith TJ, Kyle RA, Lie JT. Clinical significance of histopathologic patterns of cardiac amyloidosis. Mayo Clin Proc. 1984;59:547–55

    PubMed  CAS  Google Scholar 

  17. Suwaidi JA, Velianou JL, Gertz MA, Cannon RO, Higano ST, Holmes DR. Systemic amyloidosis presenting with angina pectoris. Ann Int Med. 1999;131:838–41

    PubMed  Google Scholar 

  18. Crawford F, Suo Z, Frang C, et al. Characteristics of the in vitro vasoactivity of beta-amyloid peptides. Exp Neurol. 1998;150:159–68

    Article  PubMed  CAS  Google Scholar 

  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;44:116–17

    PubMed  CAS  Google Scholar 

  20. Shiue ST, McNally DP. Pulmonary hypertension from prominent vascular involvement in diffuse amyloidosis. Arch Int Med. 1988;148:687–89

    Article  CAS  Google Scholar 

  21. Dingli D, Utz JP, Gertz MA. Pulmonary hypertension in patients with amyloidosis. Chest. 2001;120:1735–8

    Article  PubMed  CAS  Google Scholar 

  22. Johnson WJ, Lie JT. Pulmonary hypertension and familial Mediterranean fever: a previously unrecognized association. Mayo Clin Proc. 1991;66:919–25

    PubMed  CAS  Google Scholar 

  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;10:555

    PubMed  CAS  Google Scholar 

  24. McLaughlin WV, Shillington A. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002;106:1477

    Article  PubMed  CAS  Google Scholar 

Download references

Potential Financial Conflicts of Interest

None diclosed.

Author information

Authors and Affiliations

  1. Department of Internal Medicine, Carmel Medical Center, Faculty of Medicine, Technion-Israel Institute of Technology, 7 Michal St., Haifa, 34362, Israel

    Lihi Eder MD, Devy Zisman MD, Raffael Wolf MD & Haim Bitterman MD

Authors
  1. Lihi Eder MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Devy Zisman MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Raffael Wolf MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Haim Bitterman MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Lihi Eder MD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Eder, L., Zisman, D., Wolf, R. et al. Pulmonary Hypertension and Amyloidosis—an Uncommon Association: A Case Report and Review of the Literature. J GEN INTERN MED 22, 416–419 (2007). https://doi.org/10.1007/s11606-006-0052-9

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11606-006-0052-9

Key words

Search

Navigation