What is the role of surgery in the treatment of AA (inflammatory) amyloidosis?

Updated: Jan 08, 2021
  • Author: Jefferson R Roberts, MD; Chief Editor: Herbert S Diamond, MD  more...
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Castleman disease, a rare group of lymphoproliferative disorders in which IL-6 is often the pathologic driver, can be complicated by AA amyloidosis. Surgical resection is effective for cases that involve a single region of enlarged lymph nodes (unicentric Castleman disease). In certain cases where surgery is not feasible or curative, the anti–IL-6 agent siltuximab may be effective. [18]

Although kidney transplantation is widely used for treating renal amyloidosis secondary to familial Mediterranean fever (FMF), some data suggest that patients who have amyloidosis do not have as favorable a prognosis as patients transplanted for other forms of renal failure. [51] Nonetheless, there have been reports of improving results and transplantation is a reasonable option. [52]

Recurrence of amyloidosis in the allograft, gastrointestinal intolerance, and fatal infections remain as major complications during the post-transplant period. Severe sepsis is the cause of 60% to 100% of all deaths with a functioning graft in kidney recipients with AA amyloidosis. [53]

In a multicentric retrospective survey to assess the graft and patient survival in 59 renal transplant recipients with AA amyloidosis, the recurrence rate of AA amyloidosis nephropathy was estimated at 14%. There was significant decrease in the 5-year and 10-year survival of patients in the AA amyloidosis group compared with the control group. [54]  

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