eMedicine Specialties > Rheumatology > Metabolic and Bone Disease

Amyloidosis, Transthyretin-Related: Follow-up

Author: Jefferson R Roberts, MD, Rheumatology Fellow, Walter Reed Army Medical Center, Washington, DC
Coauthor(s): Robert John Oglesby, MD, Chief of Rheumatology Service, Department of Medicine, Walter Reed Army Medical Center; Associate Professor of Medicine, Uniformed Services University of the Health Sciences
Contributor Information and Disclosures

Updated: Sep 15, 2009

Follow-up

Complications

  • Complications reflect the organ system(s) involved. The most severe complication of systemic ATTR is extensive cardiac deposition, with consequent congestive heart failure and/or arrhythmias, and is the cause of death in many patients with ATTR.
  • Severe complications include a variety of gastrointestinal disorders (malabsorption, nausea, vomiting, diarrhea, constipation). Neuropathy can lead to paresis, sexual dysfunction, and sphincter dysfunction.

Prognosis

  • The prognosis depends on the presence and identity of a TTR variant and organ(s) involved. Patients with early-onset of variant-sequence TTR may die within a few years of diagnosis. Older patients with slowly progressive disease can live for decades after the onset of symptoms and may never develop life-threatening disease.7
  • Unlike in AL, symptomatic cardiac involvement does not necessarily portend a poor prognosis. Median survival in cardiac AL is about 6 months, but median survival is several years in older patients with cardiac ATTR, even when a TTR variant is present.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose definitively the specific type of amyloidosis, with consequent incorrect treatment
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthors Seetha U Monrad, MD; Mariana J Kaplan, MD; and Daniel R Jacobson, MD, to the development and writing of this article.



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Treatment & Medication: Amyloidosis, Transthyretin-Related
Follow-up: Amyloidosis, Transthyretin-Related
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References
Further Reading
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References

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Keywords

transthyretin-related amyloidosis, senile cardiac amyloidosis, senile systemic amyloidosis, familial amyloidotic polyneuropathy, transthyretin-type familial amyloid cardiomyopathy

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Contributor Information and Disclosures

Author

Jefferson R Roberts, MD, Rheumatology Fellow, Walter Reed Army Medical Center, Washington, DC
Jefferson R Roberts, MD is a member of the following medical societies: American College of Physicians and American College of Rheumatology
Disclosure: Nothing to disclose.

Coauthor(s)

Robert John Oglesby, MD, Chief of Rheumatology Service, Department of Medicine, Walter Reed Army Medical Center; Associate Professor of Medicine, Uniformed Services University of the Health Sciences
Robert John Oglesby, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, and Arthritis Foundation
Disclosure: Nothing to disclose.

Medical Editor

Robert E Wolf, MD, PhD, Professor Emeritus, Department of Medicine, Louisiana State University Health Sciences Center at Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Administration Medical Center of Shreveport
Robert E Wolf, MD, PhD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation, and Society for Leukocyte Biology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center
Lawrence H Brent, MD is a member of the following medical societies: American Association of Immunologists, American College of Physicians, and American College of Rheumatology
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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