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77 Cards in this Set

  • Front
  • Back

Which renal syndrome is defined by hypercellularity between nephrotic and nephritic syndrome?

Nephritic syndrome

What is the most common glomerular nephritis in the world?

IgA nephropathy

In response to what type of acute disease is IgA produced typically?

Upper respiratory infections

What cells are over-proliferating in IgA nephropathy?



What do these cells in turn produce?

Mesangial cells



ECM

Which vasculitis is associated with IgA nephropathy?

Henoch-Schönlein Purpura

What is the clinical presentation of IgA nephropathy? (3)

- Hematuria 1-2 days after URI


- Asymptomatic hematuria or proteinuria


- Henoch-Schonlein Purpura

How does IgA Nephropathy look under light microscopy?



Immunofluorescence?



Electron microscopy?

LM - Variable



IF - Granular mesangial deposits of IgA and C3


(necessary for Dx)



EM - Mesangial deposits

Do capillaries show immunofluorescence when staining for IgA nephropathy?

No

What is the treatment for IgA Nephropathy?

No proven effective treatment

What supplement may be helpful for vasculitis?

Fish oil

What is the management for IgA nephropathy?

Long-term corticosteroids

What conditions indicate a poor prognosis for IgA nephropathy? (4)

- HTN


- Obesity


- Severe proteinuria


- High Creatinine


What 2 pathogens are associated with Infection-Associated Glomerulonephritis?

- Streptococcus


- Staphylococcus

What are 4 common sites of infection that can lead to Infection-Associated Glomerulonephritis?

- Lung


- Upper respiratory tract


- Skin


- Heart valves

What gender is more susceptible to Infection-Associated GN?

Males (x2)

What strain of strep is associated with Poststreptococcal GN (PSGN)?

Group A Strep pyogenes

How is incidence of PSGN reduced?

Antibiotics

Where do immune complexes affix to in PSGN? (2)

subendothelia and mesangium

What compliment is consumed by PSGN?

C3

What is the clinical presentation of PSGN? (3)

- HTN


- Edema


- Hematuria (coke urine)


(nephrotic syndrome possible)

What titers in blood will be elevated in PSGN?

- Antistreptolysin O


- Anti-DNAse B


- Antihyaluronidase

How is PSGN treated?

Treat the infection with antibiotic and treat nephritic syndrome with sodium restriction and loop diuretics

How does Poststreptococcal Glomerulonephritis look under light microscopy?



Immunofluorescence?



Electron microscopy?

LM - Hypercellular with PMNs, capillaries obliterated



IF - Stains spotty for IgG and C3



EM - Humps against GBM

What does crescentic presentation of the glomerulus mean?

Break in the GBM

What pathogens are responsible for Endocarditis-associated GN? (3)

- Staph aureus and epidermidis


- Strep viridans and pyogenes


- Enterococcus

In what subset of the population is endocarditis-associated GN most common?

I.V. drug users

What is the clinical presentation of endocarditis-associated GN? (7)

- Fever


- Arthralgias


- Anemia


- Purpura


- New heart murmur


- Nephritic syndrome


- Low C3 and C4

What is the treatment for endocarditis-associated GN?

I.V. antibiotics for 4-6 weeks unless crescentic

Describe membranous proliferative glomerulonephritis. (3)

Response to continuous chronic immunoglobulinemia


- Thick GBM


- Cell proliferation


- Tram-tracking

What is the most common chronic antigenemic disease?

Hepatitis C

Why do antigens get stuck in GBM?

Mesangial cells are trying to engulf immuno-complexes

What is the clinical presentation for Membranous Proliferative GN? (2)

- Mixture of nephritic and nephrotic syndrome


- HTN

What indicates Type I MPGN?

subendothelial deposits

What indicates Type II MPGN?

dense deposits in the GBM

Besides Hepatitis C, what are other chronic diseases that can lead to MPGN? (4)

- Hepatitis B


- Cryoglobulinemia


- SLE


- Lymphoma

How does Membranous Proliferative Glomerulonephritis look under light microscopy?



Immunofluorescence?



Electron microscopy?

LM - Global hypercellularity, clover-leafs, tram-tracking



IF - Course granular stain to C3 or smooth capillary borders to IgG



EM - Large subendothelial deposits

What causes tram-tracking in MPGN?

Cells are recruited to the GBM to help clear complexes leading to 2 layers on the GBM

In MPGN, what side of the GBM are the antigen-antibody complexes?

Blood side

What abnormal protein leads to Type II MPGN?

C3 nephritic factor (C3Nef)


(which normally stabilizes C3-Convertase so its never deactivated)

How does Type II MPGN differ from Type I under EM?

Type II MPGN EM has thick GBM

What is the treatment for Type I MPGN?

Treat underlying disease and long-term corticosteroids for nephrotic syndrome

What is the treatment for Type II MPGN?

- Factor H replacement


- Eculizumab

What gender is more likely to have Lupus nephritis?

Females (x10)

What races are more susceptible to Lupus nephritis?

non-whites

What parts of the immune system are involved in lupus nephritis?

All parts, humoral, cell-mediated, compliment

What compliment factors are low in lupus nephritis?

C3 and C4

What is the clinical presentation of lupus nephritis? (8)

- Malaise


- Fever


- Weight loss


- Rash


- Alopecia


- Arthralgia


- Serositis


- Miscarriages

What are renal manifestations of Lupus Nephritis? (5)

- Proteinuria


- Hematuria


- RPGN


- Tubular damage


- HTN


Describe the 6 classes of light microscopy pathology for Lupus Nephritis?

Class I: normal


Class II: mesangial hypercellularity


Class III: focal endocapillary proliferation


Class IV: diffuse endocapillary proliferation


Class V: membranous lesion


Class VI: scarred-out glomerulus

What is unique about immunofluorescence of lupus nephritis compared to other GN diseases? (2)

- Many types of antigens


- Nephron staining

What is the inductive treatment for lupus nephritis? (2)

- High-dose I.V. corticosteroids and cyclophosphamide



- High-dose I.V. corticosteroids and mycophenolate mofetil

What is the maintenance treatment for lupus nephritis?

Low-dose corticosteroids or cyclosporine for at least 2 years

What is Rapidly Progressive Glomerulonephritis (RPGN)?

Nephritic syndrome with rapidly declining kidney function

What is the hallmark of RPGN pathology? (2)

necrosis and glomerular crescents

How many types of types of RPGN are there?

4

What defines Type I RPGN?

What is another term for it?

- Autoantibodies to alpha-3 chain of collagen IV in GBM and lung alveoli



- Goodpasture's syndrome

What is the clinical presentation of type I RPGN? (5)

- Nephritic syndrome


- Anti-GBM antibodies


- Progressive oliguria


- Hemoptysis


- Significant iron deficient anemia

Which glomerular diseases can lead to type II RPGN? (5)

- Lupus nephritis


- IgA nephropathy


- Endocarditis-associated GN


- Hep C-associated GN


- Post-strep GN

What kind of deposits cause necrosis in type II RPGN?

Immune-complexes

What makes type III RPGN unique among the other types?

No immune deposits

What antibody denotes type III RPGN?

antineutrophil cytoplasmic antibodies (ANCA)

What is the cause of type III RPGN?

Small-vessel vasculitis

What are two types of small-vessel vasculitis that may lead to type III RPGN?

- Microscopic polyangiitis



- Granulomatosis polyangiitis

Against what antigen are the ANCAs in microscopic polyangiitis?

myeloperoxidase (MPO)

Against what antigen are the ANCAs in Granulomatosis with polyangiitis?

proteinase 3 (PR3)

Which subtype of ANCA stains for microscopic polyangiitis?





Which for granulomatosis with polyangiitis?

MPA: p-ANCA





GPA: c-ANCA

What is the most common crescentic GN?

- Type III RPGN

What are symptoms of type III RPGN? (5)

- Fever


- Malaise


- Arthralgia


- Anorexia


- Weight-loss

What signs are seen in the ENT of a patient with type III RPGN? (5)

- nasal crusting


- sinusitis


- otitis media


- persistent rhinorrhea


- oronasal ulcers

What is seen on skin of patients with type III RPGN?

palpable purpura

Which type of RPGN has linear deposits visible on IF?



Granular?



No deposits?

Type I = linear deposits



Type II = granular deposits



Type III = no deposits

In anti-GBM disease, what is visible under light microscopy?



Immunofluorescence?



Electron microscopy?

LM - Diffuse crescentic and necrotizing GN



IF - Global linear GBM to IgG



EM - no electron deposits

What molecule stains in IF for type III RPGN?

Fibrin

What creatinine level signifies that recovery from RPGN is unlikely?

> 5.0 mg/dL creatinine

Which type of RPGN has the fastest progression of all the types?

Type III

What is the treatment for RPGN?

I.V. immunosuppressants and then plasmapheresis

What two drugs can be used for maintenance therapy after clearing RPGN? (2)

- Azathioprine


- Methotrexate