Diagnostic Considerations

Clinical/laboratory clues to cardiac involvement with amyloidosis include the following^[3]:

Persistently elevated cardiac troponin levels, or multiple presentations with elevated cardiac troponin that are not adequately explained by coronary angiography results
Heart failure combined with findings in other organ that are characteristic of amyloidosis (eg, macroglossia, nephrotic syndrome, elevated alkaline phosphatase, dysphagia)
Unexplained heart block, atrial arrhythmias, or ventricular arrhythmias in a patient with increased ventricular wall thickness

In patients with suspected cardiac amyloidosis, normal results on serum/urine protein electrophoresis with immunofixation (SPEI/UPEI) and a normal serum kappa:lambda free light chain (FLC) ratio make systemic (AL) amyloidosis highly unlikely; these patients should be evaluated for transthyretin-related amyloidosis. Patients with an abnormal SPEI/UPEI or FLC ratio should undergo biopsy for identification and subtyping of amyloid deposits, which will identify AL amyloidosis and exclude monoclonal gammopathy of undetermined significance (MGUS) and myeloma.^[3]See Workup.

Differential Diagnoses

Workup

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Media Gallery

Amyloidosis. Amorphous eosinophilic interstitial amyloid observed on a kidney biopsy specimen.
Amyloidosis. Congo red staining of a cardiac biopsy specimen containing amyloid, viewed under polarized light

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Author

Judit H Nyirady, MD, MBA Adjunct Assistant Professor, Department of Dermatology, Rutgers New Jersey Medical School

Judit H Nyirady, MD, MBA is a member of the following medical societies: American Academy of Dermatology, European Academy of Dermatology and Venereology, Royal College of Physicians of Edinburgh, Women's Dermatologic Society

Disclosure: Receiving salary from LEO Pharma Inc. for employment. for: LEO Pharma Inc.

Coauthor(s)

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

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, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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.

Primary Systemic Amyloidosis Differential Diagnoses

Diagnostic Considerations

Differential Diagnoses

References

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