Amyloidosis primary and secondary

Question 1

renal clinical manifestations of amyloidosis

Answer 1

heavy proteinuria and nephrotic syndrome

peripheral edema

Question 2

cardiac clinical manifestations of amyloidosis

Answer 2

restrictive cardiomyopathy
conduction defects and low voltage

see increased ventricular mass and may be misdiagnosed as LVH on Echo. But this person will have decreased QRS voltage which doesn’t cause LVH

Question 3

CNS and neurological clinical manifestations of amyloidosis

Answer 3

peripheral and autonomic neuropathy and stroke

Question 4

GI clinical manifestations of amyloidosis

Answer 4

hepatosplenomegaly
dysmotility
malabsorption
GI bleeding due to vascular fragility

Question 5

Pulmonary clinical manifestations of amyloidosis

Answer 5

pulmonary nodules and tracheobronchial infiltration and pleural effusions

Question 6

MSK clinical manifestations of amyloidosis

Answer 6

enlarged tongue, should pad enlargement

Question 7

SKIN clinical manifestations of amyloidosis

Answer 7

thickened skin, subcutaneous nodules and plaques
ecchymoses and periorbital purpura

remember the bluish skin of the “geno” lady

Question 8

Heme clinical manifestations of amyloidosis

Answer 8

anemia, and thrombocytopenia

Question 9

pts present with

Answer 9

lower extremity edema, dyspnea, orthostatic hypotension and conduction delays and see heavy proteinuria and glomerular deposits

Question 10

pathology stain to confirm amyloidosis

Answer 10

congo red stain and apple green birefringence.

Question 11

treatment of amyloidosis

Answer 11

<1 year survival with patients who have cardiac amyloidosis .

Question 12

heavy proteinuria, peripheral edema and low albumin and see new onset heart failure with low voltage QRS complexes

Answer 12

see nephrotic syndrome, heart failure with restrictive cardiomyopathy - think amyloidosis

Question 13

20% (most common cause) of all nephrotic syndromes in pts >60 yrs is caused by

Answer 13

amyloidosis

Question 14

what causes amyloidosis?

Answer 14

see extracellular fibrillar deposition of low molecular weight proteins

Question 15

amyloid light chain also known as

Answer 15

(AL or primary amyloidosis)

Question 16

primary amyloidosis or light chain amyloidosis is caused by

Answer 16

deposition of monoclonal immunoglobulin light chains and is associated with other plasma cell dyscrasias like MM

Question 17

Amyloid A is also known as

Answer 17

AA or secondary amyloidosis

Question 18

AA or secondary amyloidosis is caused by

Answer 18

from elevated serum amyloid A which is seen in chronic inflammatory conditions like rheumatoid arthritis

Question 19

fatigue and weight loss is seen more in this type of amyloidosis

Answer 19

seen in AL or primary amyloidosis (light chain)

Question 20

diagnosis of amyloidosis

Answer 20

detection of monoclonal protein in serum and confirmed by biopsy stain of Congo Red staining with apple green birefringence

Question 21

treatment of amyloidosis is by

Answer 21

treating underlying condition and supportive care

1 year median survival with cardiac amyloidosis

Question 22

primary amyloidosis is a

Answer 22

rare systemic disorder that occurs due to insoluble proteins that deposit into the body organs causing abnormal building and causes organ dysfunction and death.

Organs that are involved are: heart, liver, kidney, GI and peripheral autonomic nervous system.

Question 23

when does primary amyloidosis present

Answer 23

70yrs old
seen more in men than women
see profound fatigue and weight loss and peripheral edema

= could be due to nephrotic syndrome, cardiac involvement.