You might be wondering how they ended up in my transplant clinic. The answer is secondary (AA) amyloidosis primarily involving the kidney. However it only occurs in a minority of patients (approximately 15 percent in the United States).
Managing patients with TRAPS consists mainly of Etanercept, a fusion protein consisting of two copies of the 75kDa TNF receptor (TNFR2) bound to the Fc portion of human IgG. Etanercept can be highly effective in many patients with TRAPS, though not all respond and the response is sometimes partial.
Finally, the major question remains, do those patients need to be on Etanercept after transplantation, considering that they are already immunosuppressed with our maintenance regimen? Well, it’s not clear in the literature…. In our cases, the brother was maintained on Etanercept and he didn’t have any flare of the disease with stable kidney function and no recurrence of amyloidosis. The sister however, was not on Etanercept and she had recurrence of AA amyloidosis in the transplanted kidney. My approach will be to keep those patients on Etanercept and have low threshold to reduce maintenance immunosuppression.