05.20 - Kidney, Systemic 2 (Nichols, Hastings) - PP + Handout

Question 1

Most amyloiogenic proteins are made in

Answer 1

Hepatocytes or plasma cells

Question 1

Median age at dx of AL amyloidosis

Answer 1

64

Question 1

When to suspect Light Chain Disease

Answer 1

Nephrotic Syndrome or Rapidly progressive tubulointersitial nephritis, associated with cardiac diastolic dysfunction and monoclonal Ig in urine or serum

Question 2

Eprodisate

Answer 2

Limits deposition of AA fibrils

Question 2

What percent of patients with HgB SS will develop ESRD

Answer 2

4-12%

Question 3

Presentation of HIV Nephropathy

Answer 3

Progressive Azotemia, Significant Proteinuria, Minimal peripheral edema

Question 3

Glomular histology is sickle cell patients who present with chronic renal disease and proteinuria

Answer 3

Glomerulomegaly, Mesangial expanion, Segmental sclerosisi

Question 4

AA amyloidosis is due to

Answer 4

Chronic inflammatory diseases such as RA, IBD, etc.

Question 5

Light chain disease presents with

Answer 5

Proteinuria and Renal Failure

Question 5

Later Stages of Sickle cell nephropathy

Answer 5

Interstitial inflammation, edema, fibrosis, tubular atrophy, papillary infarcts

Question 6

In the kidney, mixed cryoglobulinemia most often causes what pattern of injury

Answer 6

Membranoproliferative

Question 7

Tubular and insterstitial deposits of amyloid may lead to

Answer 7

Tubular Atrophy and Interstitial fibrosis

Question 9

Interstitial fibrosis and Vascular Sclerosis with abundant brown pigment (Hb breakdown products)

Answer 9

Sickle Cell Nephropathy

Question 9

Concentrating ability in Sickle Nephropathy

Answer 9

Diminished

Question 9

Significant proteinuria but only minimal peripheral edema

Answer 9

HIV Nephropathy

Question 11

Advanced renal amyloidosis results in what kind of glomerulopathy

Answer 11

non-proliferative, non-inflammatory

Question 11

Kidney symptoms in AL amyloidosis

Answer 11

Nephrotic syndrome, Renal insufficiency w/ enlarged kidneys

Question 11

Where in kidney does sickling usually occur

Answer 11

Vasa Recta

Question 12

HIV Nephropathy Histology

Answer 12

Collapsing FSGS, with Microcystic Tubular Dilation, Intersitial inflammaiton and fibrosis

Question 13

Susceptibility gene for HIV Nephropathy

Answer 13

APOL1

Question 14

What distinguishes HIV-associated nephropathy from idiopathic collapsing FSGS

Answer 14

Accompanying prominent tubular dilation and interstitial inflammation

Question 14

GFR in sickle cell

Answer 14

Increased until 4th decade, then decreased

Question 15

Big kidneys on US

Answer 15

Diabetic Nephropathy, Amyloidosis

Question 16

Ig capable of binding IgG

Answer 16

RF

Question 18

Protein in AL amyloidosis

Answer 18

Ig light chain

Question 18

Median survival of AL amyloidosis

Answer 18

10 months

Question 18

Proteins in Cryoglobulinemia

Answer 18

Ig's, Complement, RF

Question 19

Most patients with AL just have

Answer 19

Monoclonal Gammopathy, instead of Multiple Myeloma

Question 20

Question to ask with Collapsing FSGS

Answer 20

HIV Test?

Question 21

AL amyloidosis can present with

Answer 21

Nephrotic syndrome, Restrictive CM, Diarrhea, and nonspecific

Question 21

Cryoglobulins can be polyclonal Ig's produced by

Answer 21

Chronic Inflammation

Question 22

Presence of proteins in serum, which precipitate w/ cooling serum below normal body temp

Answer 22

Cryoglobulinemia

Question 24

Early stages of sickle cell nephropathy

Answer 24

Glomerular Hypertrophy, hemosiderin deposits, focal areas of hemorrhage and necrosis

Question 25

IF of Light Chain Disease

Answer 25

Positive in glomerular capillary loop, mesangium, and tubules

Question 26

Mixed Cryoglobulinemia is associated with ___ in a very high % of cases

Answer 26

Hep C

Question 26

Coinfection of HIV and Hep C

Answer 26

Mixed Cryoglobulinemia

Question 27

Characteristic LM appearance of Light Chain Disease in Glomerulus

Answer 27

Nodular glomerulosclerosis, w/ expansion of MM

Question 28

Sickle cell disease can cause \_\_, although diabetes is much better known for it

Answer 28

Renal Papillary Infarcts

Question 29

Amyloidosis seen in long-term hemodialysis

Answer 29

Beta-2 Microglobulin

Question 29

Pattern of injury in Cryoglobulinemia

Answer 29

Membranoproliferative

Question 30

Cryoglobulinemia

Answer 30

Presence of proteins in serum, which precipitate w/ cooling serum below normal body temp

Question 32

AA amyloid is derived from

Answer 32

SAA protein

Question 34

Mixed Cryoglobulinemia most often produces what types of symptoms

Answer 34

Constitutional and Nonspecific

Question 35

End Stage of Sickle Cell Nephropathy

Answer 35

Glomerular Enlargement and FSGS

Question 36

Cryoglobulinemia can be asymptomatic or result in

Answer 36

Immune-complex-mediated GN or Vasculitis

Question 38

Limits deposition of AA fibrils

Answer 38

Eprodisate

Question 39

Tx of AL Amyloidosis

Answer 39

Melphalan and Dexamethasone

Question 41

Ages for Amyloidosis

Answer 41

Late middle age and older

Question 42

Triopathy

Answer 42

Retinopathy, Neuropathy, Retinopathy in Diabetes

Question 44

Gross appearance of advanced amyloidosis

Answer 44

Enlarged, pale organs with waxy texture

Question 45

How to distinguish Diabetic Retinopathy from Light Chain Disease

Answer 45

IF shows only lambda or kappa positivity in light chain disease

Question 46

What is necessary for amyloidosis

Answer 46

Extracellular Fibriloogenic Environment: GAG, serum amyloid P, laminin, Collagen IV, apo E

Question 47

Light Chain Disease glomerular LM strongly resembles

Answer 47

Diabetic Nephropathy

Question 49

Beta 2 Amyloid occurs with

Answer 49

long-term hemodilaysis

Question 50

Beta 2 microglobulin is a component of

Answer 50

MHC class 1 molecules

Question 51

B cell lymphoproliferative disorder characterized by deposition of immune complexes containing RF, IgG, HCV RNA, and complement on endothelial surfaces, eliciting vascular inflammation

Answer 51

Mixed Cryoglobulinemia

Question 53

Most common renal manifestation of AA amyloidosis

Answer 53

Nephrotic syndrome

Question 55

How does Sickle Cell Nephropathy most commonly manifest?

Answer 55

Proteinuria

Question 56

Hereditary Amyloidosis

Answer 56

Replacement of single AA --\> Renders protein prone to fibril formation

Question 57

Pseudothrombi in capillary lumens in glomerulus

Answer 57

Cryoglobulinemia

Question 59

Monoclonal cryoglobulins typically produce signs and symptoms related to

Answer 59

Hyperviscosity and/or thrombosis

Question 60

Pathology of HIV Nephropathy

Answer 60

Collapsing Form of FSGS

Question 62

Most commonly dx type of amyloidosis

Answer 62

AL

Question 63

Main target organ of AA amyloidosis

Answer 63

Kidney

Question 64

\_\_ proteins can precipitate in tubules where epithelial cells coalesce into a syncytium around them

Answer 64

Bence Jones

Question 65

Glomerulomegaly, Mesangial Expansion, Tubular epithelial hemosiderin accumulation, Interstitial fibrosis and vascular sclerosis

Answer 65

Sickle Cell Nephropathy

Question 66

\_\_\_ is a unique feature of the collapsing FSGS associated with HIV

Answer 66

Microcystic Tubular Dilatation

Question 68

Light chain disease vs AL amyloid

Answer 68

deposits are congo red negative and have no fibrillar organization

Question 69

Tx of AA amyloidosis

Answer 69

Eprodisate

Question 70

Beta 2 amyloid tends to deposit

Answer 70

Synovium, joints, and tendon sheaths

Question 71

Membranoproliferative pattern of injury with deposition of immune complexes containing RF, IgG, Hep C virus RNA and complement factors on endothelial surfaces and in subendothelial and mesangial locations

Answer 71

Mixed Cryoglobulinemia

Question 72

HIV-associated nephropathy results from

Answer 72

Direct infection of renal epithelial cells in genetically susceptible host

Question 73

Podocyte proliferation

Answer 73

HIV Nephropathy

Question 74

Cryoglobulins can be monoclonal Ig's produced by

Answer 74

Multiple Myeloma