Gen Path Exam 3 - Autoimmune Disease - Amyloidosis Flashcards
Diverse family of rare, chronic infiltrative
localized or multisystem disorders characterized by the presence of extracellular deposits of insoluble fibrillar proteins
Amyloidosis
What is each variety of amyloidosis identified by?
Immunochemical nature of amyloid protein fibrils
What are the syndromes present at presentation from most to least common?
Nephrotic syndrome/renal failure
Carpal tunnel
CHF
Peripheral neuropathy
Orthostatic hypotension
Amyloidosis is an important cause of what 3 things?
Nephrotic syndrome
Renal insufficiency
Progressive renal failure
Not a single substance, but a group of substances that share a common physical structure that can be formed by a number of different proteins
Amyloid
What does amyloid always have?
Beta-pleated sheet configuration
Amyloidosis results from abnormal _________ of proteins, which are deposited as _________ in extracellular tissues and disrupt normal function
folding; fibrils
What is the soluble circulating protein found in the serum of pts with amyloidosis?
Serum Amyloid P (SAP)
Where is Serum Amyloid P (SAP) deposited?
Tissues
What is Serum Amyloid P (SAP) deposited in the tissues as?
Insoluble beta-pleated sheets
What does 95% of amyloid material consist of?
Fibril proteins
What does the other 5% of amyloid material consist of?
P component
Glycoproteins
What do amyloid deposits cause?
Tissue injury
What do amyloid deposits impair? How?
Normal function by causing pressure on cells/tissue
Do amyloid deposits in tissues evoke an inflammatory response?
NO!