Kidney in Systemic Disease II Flashcards Preview

Renal Week 3 > Kidney in Systemic Disease II > Flashcards

Flashcards in Kidney in Systemic Disease II Deck (42):

What causes Sickle Cell Pain Crisis?
• what are some key lab values you would expect to see in this disease?

Sickle Cell Crisis:
Vasoocclusive Phenomenon


Key Labs (outside of peripheral smear):
Elevated Reticulocytes
Elevated Lactate Dehydrogenase and Bilirubin


Smear would show:
• Howell Jolly Bodies from Splenic Infarcts and Sickled cells


What percentage of Sickle Cell Patients will progress to end stage renal disease?

4-12% - these patients are hard to take care of because you have anemia on top of renal dysfunction


What type of Renal Pathology would you expect to see in someone with Sickle Cell Nephropathy at different points in disease progression:
• Early

• Late

• End Stage

• Focal areas of cortical injury, Hemorrhage and Necrosis with Glomerular Hypertrophy
TUBULAR atrophy and Papillary Infarcts
Focal Segmental Glomerulosclerosis

***Really you're just getting infarcts in the cortex (peritubular capillaries) that also progress through the vasa recta causing ischemia and decreased absorption, eventually tubular dysfunction leads to a decreased GFR.


T or F: what is shown here is Chronic Injury due to Sickle Cell.

FALSE, this is acute injury, all you see is CONGESTION of peritubular capillaries and ACUTE TUBULAR NECROSIS (notice nuclear fallout on both of these images)

note front and back are both Acute Sickle Cell Nephropathies


What immune complexes will stain positive in Sickle Cell Nephropathy?
• Key features on silver staining?

• NONE, sickle cell nephropathy is not associated with immune complex deposition


• Silver Staining Key Features - Duplication of the Basement membrane


How do you differentiate this silver staining from Membranoproliferative Glomerulonephritis type I based on the image below?
• Serological studies?

Shown here is Sickle Cell disease you can see sickeled cells inside the glomerular capillaries 


Serological Studies:
• Immunoglobulins/ Low C3 levels will be present in MPGN type I, this is not the case in sickle cell


Is the image shown below more indicative of acute or chronic sickle cell disease?

CHRONIC - you can see severe sclerosis of the glomeruli here


What are the key features seen in this EM from a sickle cell patient?

Podocyte Food Process Effacement
• Impacted cell also suggest an abnormal amount of clotting within the glomerulus


What cancer of the Kidney is EXCLUSIVELY associated with sickle cell patients?

• Prognosis?
• Location?


VERY POOR these pts. typically present with Metastatic cancer and die in 4-6 months


• Cancer is typically localized to the RIGHT kidney


What 3 chronic kidney diseases actually cause ENLARGMENT of the kidney?
• why is this unusual?

ENLARGMENT of the kidney:
• Renal Amyloidosis
• Diabetic Nephropathy
• HIV associated Nephropathy


What Disease is shown here?
• how does kidney disease progress in a patient who shows thiss?


Progression: Tubular and Interstitial Deposits may lead to TUBULAR atrophy and INTERSTITIAL fibrosis


What type of Amyloidosis should you suspect in someone who has Rheumatoid Arthritis and nephrotic syndrome?

AA amyloid, it associated with diseases of chronic inflammation


What type of amyloidosis should you suspect in a person who has polyneuropathy and family history of amyloidosis?

ATTR - transthyretin type of amyloidosis runs in families


What type of amyloid should you suspect in a patient with 'shoulder pads' and glossomegaly?

AL amyloid - deposition of restricted immuno light chains


What disease associations should you make with AL amyloid?

• Multiple Myeloma
• Lymphoma
• Waldenstrom's Macroglobulinemia


What disease associations should you make with AA amyloid?

Any disease that causes chronic Inflammation or Infection


Who gets ß2-microglobulin amyloidosis?
• typical sight of deposition?


• Tends to deposit in the Joints


After seeing apple green birefringence on Congo Red Staining of Biopsy, how can you confirm the type of amyloid you're dealing with?
• AA?
• AL?

• Immunohistochemistry is typically enough


• Need to show MONOCLONAL proliferation in the Bone or Kappa or Lamba restricted light chains in the tissue biopsy


• Need to do a DNA test


AL Amyloidosis Nephropathy:
• who are you most likely to see it in?
• Kidney size? HTN? Other problems?

• Mean age is 64 y/o - these pts. probably have cancer/pre-cancer


• Kidney is enlarged in this condition (b/c its an amyloidosis)


NO hypertension associated with this disease


 **Restrictive Cardiomyopathy and GI immotility may also be issues for these patients


What is the prognosis after getting diagnosed with PRIMARY AL amyloid?
• What are your treatment options?

Px: BAD, most pts. only live 10 months after diagnosis

Patients under 50:
• Melphalan and get a Stem Cell transplant

Patients over 50:
• Melphalan + Dexamethasone


Patients with what type of AL amyloidosis have the greatest chance of developing Multiple Myeloma?

• I Chain AL amyloidosis is most associated with this


What is shown here?

Bence Jones proteins can precipitate in the urine or in the tubules and the tubular epithelial cells can coalesce into a syncytium around them:


Shown Below is a Bone Marrow Biopsy of a Patient with amyloidosis


What is the main target organ for AA amyloidosis?
• What is the newest treatment option?
• what typically causes death in these patients?

Main Target Organ:
1st = Kidney
2nd = GI

(compare to AL that also targets the hrt)


Newest Tx:
Eprodisate - Prevents interaction between GAGs needed to form amyloid sheets (does not improve survival)


• Typically related to infection from dialysis


What is this patients' biggest risk factor for experiencing this injury?

This is a ß2 microglobulin defect that deposits in BONE leading to destruction

DIALYSIS patients are the people most often seen with this


Below you can see an H and E of this disease


What is Light Chain Deposition Disease?
•what is it associated with?
• Amyloid Staining? explain.

Light Chain Deposition Disease:
• Associated with: MULTIPLE MYELOMA


This DOES NOT complex to form chains that are characteristic of amyloid this means it also WILL NOT STAIN WITH THE CONGO RED DYE


How might someone with light chain syndrome present?

•Proteinuria and renal failure

•May have non-nephrotic range proteinuria with a tubulointerstitial syndrome

•May present with rapidly declining renal function

•May have hepatomegaly

•Cardiomegaly, CHF, conduction disturbances

•Peripheral neuropathy, GI disturbances, pulmonary nodules, arthropathy, sicca syndrome


What are the Key Features of the Image seen here?
• what should be in your differential for this image?

NODULAR Glomerulosclerosis
Deposition along the outside of the Tubular Membrane
Expansion of the Mesangial Matrix


• Light Chain Deposition Disease
• Diabetic Nephropathy


Shown here is a Kappa light chain stain.
• Key features?
• Dx?

1. Kappa Restriction suggests pathology

2. Can see NODULAR Glomerulosclerotic appearance


Dx: Light Chain Disease


What should raise suspicion of a patient having Light Chain Disease?

•Increased index of suspicion with nephrotic syndrome or rapidly progressive tubulointerstitial nephritis, cardiac diastolic dysfunction, and presence of monoclonal immunoglobulin in the urine or serum


HIV-associated Nephropathy
• Type of Neuropathy? What distinguishes it from others of this type?
• Who does this occur in most often?



COLLAPSING Focal Segmental Glomerulosclerosis mostly occurs in AFRICAN AMERICANS

**MYCROCYSTIC DILATED TUBULES with PODOCYTE PROLIFERATION and Interstitial inflammation distinguish this from other FSGS types


Besides HIVAN, what else can cause Microcystic Tubular Dilation and podocyte proliferation in FSGS?

Bisphosphonate Pamidronate


What viral Genes are believed to be key in causing HIVAN?
• genes that make African Americans Susceptible?

Viral Genes:
Nef and Vpr - cause DE-differentiation of podocytes


African American Susceptibility:
APOL1 related


What should you be thinking when you see the following clinical features?

•Black race

•Advanced HIV disease

•Heavy proteinuria

•Rapid decline in kidney function

HIVAN - behind sickle cell, this is the most common cause of End Stage Renal Disease in young African Americans


What cause glomerular collapse in HIVAN?

FSGS leads to collapse because of PODOCYTE (visceral epithelial) overgrowth


What is shown here?
• KEYYYY terms?

Collapsing FSGS from HIVAN

• This shows the MICROCYTIC**** tubular interstitial appearance (lots of little holes to the left)

• On the far right in the middle you can see collapsed glomeruli


What triad is associated with Cryoglobulinemia?

• markers found in serum?
• Disease association?
 • Persistence of the disease?

Palpable Purpura
• Asthenia
• Arthralgia

 Rh factor and Cryoglobulins are key

Disease Association:
90% of cases are associated with Hepatitis C

Described as Waxing and Waning




• Immune Complex Disease?
• Kidney Pathology?
• Presence of Proteinuria or Hematuria?
• Px?

•Renal disease usually due to immune complex disease

•Was the cause of biopsy proven glomerulonephritis

Membranoproliferative glomerulonephritis is the typical renal biopsy finding

•Isolated proteinuria or hematuria occurs much more frequently than nephrotic or nephritic syndromes or acute renal failure.

•Most patients declare a chronic or rapidly progressive disease course within three to five years of diagnosis


Where does Cryglobulinemia tend to affect the kidney?

Intralobular Arteries and Afferent Arterioles


See Below: deeply acidophilic material in the vessel probably is a mixture of thrombus, fibrinoid necrosis, and aggregated cryoglobulins. The vessel wall and adjacent tissue are infiltrated by leukocytes with leukocytoclasia.



In acute cases, the deposits produce the appearance of thrombi or wire loops comparable to what is seen in lupus glomerulonephritis.


What do you expect to see on immunofluorescence of Cryoglobulinemia?
• what immuno stain should you probably use?

GLOBULAR APPEARANCE of Cryogloblulins most often is C3


What key Feature on this EM lets you know that its Cryoglobulinemia?

Fibrillary Appearance in the SUBEPITHELIUM