eMedicine Specialties > Rheumatology > Miscellaneous Inflammatory Arthritis

Amyloidosis, AA (Inflammatory): Follow-up

Author: Richa Dhawan, MD, Faculty, Center of Excellence for Arthritis and Rheumatology, Louisiana State University Health Science Center at Shreveport
Coauthor(s): Mohammed Mubashir Ahmed, MD, Associate Professor, Department of Medicine, Division of Rheumatology, University of Toledo College of Medicine; Eisha Mubashir, MD, Fellow in Rheumatology, Department of Medicine, Fellow, Center of Excellence for Arthritis and Rheumatology, Louisiana State University Health Sciences Center, Shreveport; Joel Buxbaum, MD, Professor, Department of Molecular and Experimental Medicine, The Scripps Research Institute
Contributor Information and Disclosures

Updated: Nov 21, 2008

Follow-up

Further Inpatient Care

  • Inpatient care may be necessary for intercurrent infections or deterioration in renal function, requiring acute dialysis or the initiation of chronic dialysis.

Further Outpatient Care

  • Monitor renal function to assess progress and the ultimate need for dialysis or transplantation.

Inpatient & Outpatient Medications

  • Use medications effective in the treatment of the primary inflammatory diseases to completely suppress the inflammatory process, if possible.
  • Colchicine may be administered concurrently with these agents, although no controlled studies indicate that it is effective in amyloid A (AA) amyloidosis, other than in cases associated with FMF.

Transfer

  • Diminishing renal function demands management by an experienced nephrologist, with particular emphasis placed on the need for dialysis and the availability of transplantation.

Deterrence/Prevention

  • The use of colchicine prophylaxis in FMF has been previously mentioned, as has the need for aggressive anti-inflammatory treatment for the predisposing inflammatory disorders (see Treatment).
  • The recent introduction of anti-inflammatory biological agents for the treatment of rheumatologic disorders may decrease the current rate of appearance of tissue AA deposition.

Complications

  • The major consequence of renal amyloidosis is complete renal failure. Because it occurs in the natural course of AA amyloidosis, it may not be considered a complication but certainly requires aggressive management, with transplantation or maintenance with dialysis.

Prognosis

  • The prognosis of the AA amyloidosis, regardless of the prognosis of the primary disease, has generally been associated with the degree of renal compromise present at the time of diagnosis, ie, poor prognosis is associated with a serum creatinine level greater than 2 mg/dL or a serum albumin level of less than 2.5 g/dL. Mean survival is 2-3 years.
  • More recent studies in which patients had access to renal replacement therapy suggest improved survival to more than 4 years. In the latter cases, infection was the major cause of death. With improved aggressive anti-infectious treatment, further enhanced survival likely is possible, even without specific treatment that allows resorption of the deposited fibrils or inhibits further deposition.
  • The idea has been suggested that, even with fibril resorption and no further deposition, residual tissue damage will persist or fibrillar material will redistribute, primarily to the kidney. At present, these speculations remain to be tested.

Patient Education

  • Inform patients about the natural course of AA amyloidosis and the fact that aggressive anti-inflammatory management could prevent ultimate organ failure.
  • Preparing the patient for either renal transplant or dialysis is the major educational goal. Clearly, the manner in which this is presented depends on the relationship between the physician and the patient and the physician's assessment of the patient's emotional needs.

Miscellaneous

Medicolegal Pitfalls

  • Misdiagnosis of amyloid A (AA) amyloidosis as AL amyloidosis and the institution of cytotoxic therapy appropriate for AL amyloidosis cause needless risk for the complications of chemotherapy. Competent immunohistologic diagnosis of biopsy samples is critical to avoid this pitfall.
  • Failure to diagnosis and appropriately treat a treatable primary cause of AA amyloidosis puts the patient at risk for continuing deposition of amyloid, when it could be reduced or eliminated by appropriate antibiotic, surgical, or aggressive anti-inflammatory therapy.

Special Concerns

  • The major special concern with AA amyloidosis is the accuracy of the diagnosis, and the managing physician must be aware that ongoing research in the therapy of the predisposing inflammatory disorders and amyloid itself may result in advances that should be implemented immediately in the therapeutic regimen.
  • Participation of patients with amyloidosis in clinical trials is critical to the evaluation of new therapeutic modalities.
 


More on Amyloidosis, AA (Inflammatory)

Overview: Amyloidosis, AA (Inflammatory)
Differential Diagnoses & Workup: Amyloidosis, AA (Inflammatory)
Treatment & Medication: Amyloidosis, AA (Inflammatory)
Follow-up: Amyloidosis, AA (Inflammatory)
References
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References

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Further Reading

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Keywords

amyloidosis, secondary amyloidosis, amyloid A amyloidosis, AA amyloidosis, inflammatory amyloidosis, systemic amyloidosis, inflammation-associated amyloidosis, tissue amyloid deposition, AA deposition, renal amyloidosis, amyloid renal disease, amyloid nephropathy, rheumatoid arthritis, RA, familial Mediterranean fever, FMF, serum amyloid A protein, SAA protein

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Contributor Information and Disclosures

Author

Richa Dhawan, MD, Faculty, Center of Excellence for Arthritis and Rheumatology, Louisiana State University Health Science Center at Shreveport
Richa Dhawan, MD is a member of the following medical societies: American Association of Physicians of Indian Origin, American College of Physicians-American Society of Internal Medicine, and American College of Rheumatology
Disclosure: Nothing to disclose.

Coauthor(s)

Mohammed Mubashir Ahmed, MD, Associate Professor, Department of Medicine, Division of Rheumatology, University of Toledo College of Medicine
Mohammed Mubashir Ahmed, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, and American Federation for Medical Research
Disclosure: Nothing to disclose.

Eisha Mubashir, MD, Fellow in Rheumatology, Department of Medicine, Fellow, Center of Excellence for Arthritis and Rheumatology, Louisiana State University Health Sciences Center, Shreveport
Disclosure: Nothing to disclose.

Joel Buxbaum, MD, Professor, Department of Molecular and Experimental Medicine, The Scripps Research Institute
Joel Buxbaum, MD is a member of the following medical societies: American Society for Clinical Investigation, American Society of Human Genetics, and Association of American Physicians
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: Nothing to disclose.

Managing Editor

Elliot Goldberg, MD, Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine
Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology
Disclosure: Nothing to disclose.

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

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; Merck, Amgen, Biogen, Zimmer, Wyeth, Johnson&Johnson, Stryker, Medtronic, Zimmer.Abbott,  Ownership interest Other; West Penn Allegheny Health System Consulting fee Consulting; Alpharma Honoraria Consulting; Proctor&Gamble Grant/research funds Independent contractor

 
 
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