Internal Medicine

P1: SBT

0521779407-03 CUNY1086/Karliner 0 521 77940 7 June 7, 2007 19:6

Amebic Liver Abscess Amyloidosis 111

Prognosis

■Excellent prognosis w/ prompt diagnosis in absence of complica-

tions

■Delay in diagnosis can result in rupture & higher mortality: 20% mor-

tality w/ rupture into chest or peritoneum; 30–100% w/ rupture into

pericardium

Amyloidosis........................................

KEITH STOCKERL-GOLDSTEIN, MD

history & physical

multiple etiologies-associated with deposition of insoluble protein in

organs

primary amyloidosis (AL) – plasma cell dyscrasia; amyloid:

immunoglobulin light chains

secondary amyloidosis(AA) – chronic infections or inflammation;

amyloid A protein

familial amyloidosis(AT TR) – mutation in transthyretin

beta2-microglobulin amyloid (B2M)– in renal dialysis

History

■chronic dialysis (B2M)

■family history – family history of amyloidosis or progressive neu-

ropathy suggests ATTR

■chronic inflammatory processes: Crohn’s, RA, juvenile RA, ankylos-

ing spondylitis, familial Mediterranean fever (FMF)

■chronic infections including tuberculosis, osteomyelitis, bronchiec-

tasis, IV drug use

Signs & Symptoms

■variable depending on type of amyloid and organ involvement

■renal: proteinuria (AL and AA)

■cardiac: heart failure, conduction abnormalities, low voltage on EKG,

orthostatic hypotension; rare in AA

■GI: macroglossia, hepatomegaly, splenomegaly

■neurologic: carpal tunnel syndrome, peripheral neuropathies and

autonomic insufficiency

■integument: periorbital purpura, easy bruising, arthropathies

■less commonly: GI bleeding, diarrhea malabsorption, bleeding

diathesis, endocrine abnormalities