Gertz et al listed criteria for determining if a patient with primary systemic amyloidosis have responded to therapy. The authors are from the Mayo Clinic.
Conditions evaluated:
(1) renal insufficiency
(2) nephrotic syndrome level proteinuria (>= 3 grams protein per 24 hour)
(3) liver involvement
(4) cardiac involvement
(5) serum monoclonal protein
(6) urine monoclonal protein excretion
Conditions
Indications of Response
renal insufficiency
complete return of serum creatinine to normal
nephrotic syndrome level proteinuria
>= 50% reduction in proteinuria without an increase in serum creatinine
liver involvement
no longer palpable below the right costal margin; complete return of alkaline phosphatase to normal
cardiac involvement
resolution of symptoms of CHF; echocardiographic evidence of response
serum monoclonal protein
complete disappearance
urine monoclonal protein excretion
complete disappearance
Reduction in serum monoclonal protein levels without evidence of improved function in involved organs was not considered a response.
A > 50% reduction in the serum monoclonal protein level without a complete return to normal may indicate a partial response if there is evidence of improved organ function.
The requirement for stable renal function with nephrotic level proteinuria is to make sure the decrease is not due to functional deterioration.
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