eMedicine Specialties > Dermatology > Internal Medicine

Amyloidosis, Primary Systemic: Follow-up

Author: Judit Nyirady, MD, MBA, Adjunct Assistant Professor, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: Apr 29, 2009

Follow-up

Complications

  • The presence of diffuse skin infiltrates and the loss of scalp hair can be distressing.
  • Macroglossia may be associated with painful dysphagia.
  • Cardiac involvement may cause angina, infarction, arrhythmias, or orthostatic hypotension.
  • Congestive heart failure or arrhythmias account for death in about 40% of the cases of primary systemic amyloidosis.
  • Blood vessel infiltration may lead to claudication of the legs or jaw.
  • Amyloid infiltration of the gastrointestinal tract may result in hemorrhage that can cause malabsorption or even death.21

Prognosis

  • The prognosis of primary systemic amyloidosis is generally poor.
  • Cardiac failure and renal failure are the major causes of death.
  • The median survival in most reported cases is as long as 14.7 months.
  • The prognosis depends on the response to therapy and the extent of disease.22
  • The presence of congestive heart failure is associated with a median survival of 7.7 months.

Miscellaneous

Medicolegal Pitfalls

  • Because the symptoms of primary systemic amyloidosis are often nonspecific, the diagnosis is often delayed.
  • Misdiagnosis may result in medical and legal liability.
 


More on Amyloidosis, Primary Systemic

Overview: Amyloidosis, Primary Systemic
Differential Diagnoses & Workup: Amyloidosis, Primary Systemic
Treatment & Medication: Amyloidosis, Primary Systemic
Follow-up: Amyloidosis, Primary Systemic
References
Further Reading
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References

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Keywords

primary systemic amyloidosis, amyloidosis, PSA, amyloidosis associated with multiple myeloma, secondary systemic amyloidosis

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Contributor Information and Disclosures

Author

Judit Nyirady, MD, MBA, Adjunct Assistant Professor, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Judit Nyirady, MD, MBA is a member of the following medical societies: American Academy of Dermatology, European Academy of Dermatology and Venereology, International Society of Dermatology, Society for Investigative Dermatology, and Women's Dermatologic Society
Disclosure: Novartis Pharmaceuticals Corporation Salary Employment

Coauthor(s)

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Takeji Nishikawa, MD, Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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