Summary of Kidney in Systemic Disease II

Question 1

Sickle cell nephropathy
• Prevalence in Sickle cell pts.
• Style of damage?

Answer 1

4 to 12% of patients with hgb SS disease will develop end-stage renal disease (ESRD)

Associated with glomerular enlargement and focal segmental glomerulosclerosis

Question 2

Amyloidosis
• Strongest association you should make? 
• Location?
• Proliferation?
• Inflammation?

Answer 2

• this is a NON-INFLAMMATORY renal disease that is located in EXTRACELLULAR SPACES and does NOT involve cell proliferation
• Ironically CHRONIC INFLAMMATION is the most common cause of this disease

Question 3

Light Chain Disease
• Amyloid?
• Staining?
• Disease association?

Answer 3

• nonamyloid monoclonal immunoglobulin deposition disease
• Congo red negative
• Involves precipitation of immunoglobulin chains without the elongation seen in amyloid
• Usually associated with multiple myeloma

Question 4

HIV-associated nephropathy
• Edema?
• Type of Renal Damage?

Answer 4

• Progressive azotemia, significant proteinuria and MINIMAL PERIPHERAL EDEMA (weird for a protein loss)
• Collapsing focal segmental glomerulosclerosis with pronounced MICROCYSTIC tubular dilation, interstitial inflammation, and fibrosis

Nephrotic w/o looking nephrotic
Collapsing doesn’t mean they have to be HIV+

Question 5

Cryoglobulinemia
• Disease Association?
• Pattern of Injury?
• Location of injury?

Answer 5

• Associated with Hepatitis C in more than 90% of cases
• Deposition of immune complexes containing rheumatoid factor (RF), IgG, HCV RNA, and complement on endothelial surfaces
• Causes a membranoproliferative pattern of injury