Chapters
- 00:00:15The advice for the nephrologist to diagnose AL amyloidosis
- 00:00:19earlier is severe nephrotic syndrome or massive proteinuria,
- 00:00:27again, in the absence of diabetes, hypertension, rheumatologic conditions,
- 00:00:34specifically if nephrotic syndrome is associated with massive albuminuria
- 00:00:40of greater than 5 grams, greater than 10 grams.
- 00:00:44Patients also have other organ system involvement with congestive heart failure,
- 00:00:49autonomic neuropathy leading to postural hypotension, or low blood pressures.
- 00:00:55Again, this constellation of these syndromes and these clinical features
- 00:00:59should lead a nephrologist to suspect a systemic disease
- 00:01:05and maybe a renal biopsy should be performed sooner
- 00:01:09rather than later to diagnose AL amyloidosis.
- 00:01:14Furthermore, 10% of patients with renal AL amyloidosis may not have
- 00:01:20nephrotic syndrome, may not have albuminuria, or albumin spillage in the urine.
- 00:01:26They could present just with worsening of renal function.
- 00:01:30And in those cases, as well, renal biopsy would make the diagnosis
- 00:01:35of AL amyloidosis with amyloid deposits
- 00:01:38in the tubulointerstitial region rather than the glomeruli.
- 00:01:48A lot, you know, cardiologists and nephrologists,
- 00:01:50along with hematologists and neurologists
- 00:01:52work very closely together, especially at the
- 00:01:56Boston University Amyloidosis Center,
- 00:01:58because the key to diagnosing AL cardiac amyloidosis is
- 00:02:03is to recognize that these organs are being involved simultaneously.
- 00:02:07First of all, realize that this is somebody who has worsening heart failure
- 00:02:11with thick heart walls, who also has protein in their urine,
- 00:02:15and all at once the differential shifts from
- 00:02:19run-of-the-mill heart failure to something that is more,
- 00:02:23that is rarer, like a systemic AL amyloidosis.
- 00:02:27And so that happens all the time, and it involves a collaboration
- 00:02:30between the nephrologist, the kidney specialist,
- 00:02:33and the heart specialist to put their heads together and realize that
- 00:02:36systemic AL amyloidosis is the common theme here.