The advice for the nephrologist to diagnose AL amyloidosis
earlier is severe nephrotic syndrome or massive proteinuria,
again, in the absence of diabetes, hypertension, rheumatologic conditions,
specifically if nephrotic syndrome is associated with massive albuminuria
of greater than 5 grams, greater than 10 grams.
Patients also have other organ system involvement with congestive heart failure,
autonomic neuropathy leading to postural hypotension, or low blood pressures.
Again, this constellation of these syndromes and these clinical features
should lead a nephrologist to suspect a systemic disease
and maybe a renal biopsy should be performed sooner
rather than later to diagnose AL amyloidosis.
Furthermore, 10% of patients with renal AL amyloidosis may not have
nephrotic syndrome, may not have albuminuria, or albumin spillage in the urine.
They could present just with worsening of renal function.
And in those cases, as well, renal biopsy would make the diagnosis
of AL amyloidosis with amyloid deposits
in the tubulointerstitial region rather than the glomeruli.
A lot, you know, cardiologists and nephrologists,
along with hematologists and neurologists
work very closely together, especially at the
Boston University Amyloidosis Center,
because the key to diagnosing AL cardiac amyloidosis is
is to recognize that these organs are being involved simultaneously.
First of all, realize that this is somebody who has worsening heart failure
with thick heart walls, who also has protein in their urine,
and all at once the differential shifts from
run-of-the-mill heart failure to something that is more,
that is rarer, like a systemic AL amyloidosis.
And so that happens all the time, and it involves a collaboration
between the nephrologist, the kidney specialist,
and the heart specialist to put their heads together and realize that
systemic AL amyloidosis is the common theme here.
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