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Cirulis MM, Emerson LL, Bull DA, et al. Pulmonary arterial hypertension in primary amyloidosis. Pulm Circ. 2016;6(2):244-8doi: 10.1086/686172.
Cirulis, M. M., Emerson, L. L., Bull, D. A., Hatton, N., Nativi-Nicolai, J., Hildebrandt, G. C., & Ryan, J. J. (2016). Pulmonary arterial hypertension in primary amyloidosis. Pulmonary circulation, 6(2), 244-8. https://doi.org/10.1086/686172
Cirulis, Meghan M, et al. "Pulmonary arterial hypertension in primary amyloidosis." Pulmonary circulation vol. 6,2 (2016): 244-8. doi: https://doi.org/10.1086/686172
Cirulis MM, Emerson LL, Bull DA, Hatton N, Nativi-Nicolai J, Hildebrandt GC, Ryan JJ. Pulmonary arterial hypertension in primary amyloidosis. Pulm Circ. 2016 Jun;6(2):244-8. doi: 10.1086/686172. PMID: 27252852; PMCID: PMC4869930.
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Pulm Circ. 2016 Jun;6(2):244-8. doi: 10.1086/686172.

Pulmonary arterial hypertension in primary amyloidosis.

Pulmonary circulation

Meghan M Cirulis, Lyska L Emerson, David A Bull, Nathan Hatton, Jose Nativi-Nicolai, Gerhard C Hildebrandt, John J Ryan

Affiliations

  1. Division of Cardiovascular Medicine, Department of Medicine, University of Utah, Salt Lake City, Utah, USA; Internal Medicine-Pediatrics Residency Program, Department of Medicine, University of Utah School of Medicine, Salt Lake City, Utah, USA.
  2. Division of Anatomic Pathology, Department of Pathology, University of Utah, Salt Lake City, Utah, USA.
  3. Division of Cardiothoracic Surgery, Department of Surgery, University of Utah, Salt Lake City, Utah, USA.
  4. Division of Pulmonary Medicine, Department of Medicine, University of Utah, Salt Lake City, Utah, USA.
  5. Division of Cardiovascular Medicine, Department of Medicine, University of Utah, Salt Lake City, Utah, USA.
  6. Division of Hematology and Hematologic Malignancies, Department of Medicine, University of Utah, Huntsman Cancer Institute, Salt Lake City, Utah, USA.

PMID: 27252852 PMCID: PMC4869930 DOI: 10.1086/686172

Abstract

Amyloidosis involves extravascular deposition of fibrillar proteins within tissues and organs. Primary light chain amyloidosis represents the most common form of systemic amyloidosis involving deposition of monoclonal immunoglobulin light chains. Although pulmonary amyloid deposition is common in primary amyloidosis, clinically significant pulmonary amyloidosis is uncommon, and elevated pulmonary artery pressures are rarely observed in the absence of other underlying etiologies for pulmonary hypertension, such as elevated filling pressures secondary to cardiac amyloid. In this case report, we present a patient with primary light chain amyloidosis and pulmonary arterial hypertension in the setting of pulmonary vascular and right ventricular myocardial amyloid deposition.

Keywords: Congo red stain; medial hypertrophy; multiple myeloma; pulmonary vascular disease

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