Amyloidosis

Question 1

What is amyloidosis?

Answer 1

Amyloidosis is the extracellular accumulation of protein fibrils, which then interfere with specific organ functions.

Question 2

<p>How would one look for amyloid deposition in tissues?</p>

Answer 2

<p>You can see amyloid by staining with Congo red under polarized light and then looking for green-yellow birefringence. ***find pic?</p>

Question 3

What are some diseases that cause amyloid deposition?

Answer 3

Amyloid deposition occurs with many diseases, including infectious diseases (TB, lebrosy, and chronic osteomyelitis), familial Mediterranean fever, IBD, Behcet disease, and SLE.

Question 4

What is the most serious manifestation of amyloid deposition?

Answer 4

Renal disease is the most prevalent and serious manifestation of amyloid deposition.

Question 5

How do patients with renal amyloidosis typically present?

Answer 5

Patients present with proteinuria, with rapid progression to nephrotic-range proteinuria, and then eventually renal failure.

Question 6

How is amyloidosis diagnosed?

Answer 6

Diagnosis requires evidence of amyloid deposition in tissue. There are no specific lab tests which are diagnostic, but many patients with have markedly elevated ESR, CRP, and serum amyloid A levels.

Question 7

How is amyloidosis managed?

Answer 7

Prevention is much more effective than treatment. As such, aggressive therapy for diseases that predispose to amyloidosis is important.