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Flashcards in glomerulonephritis Deck (34):
1

Define glomerulonephritis

A group of diseases associated with inflammatory injury of the glomeruli indicated by Infiltration of leukocytes, Deposition of immune proteins and Disturbed function of the affected tissue

2

Which cell types can be involved in glomerulonephritis

Mesangium, podocytes, capillaries/endothelium, parietal epithelial cells

3

Nephritic syndrome definition

• Reduction in GFR (an elevated serum creatinine)
• Active urine sediment (RBC's, WBC's, and RBC casts)
• Proteinuria (usually sub-nephrotic)
• Edema
• Hypertension
• Reduction in GFR (an elevated serum creatinine)
• Active urine sediment (RBC's, WBC's, and RBC casts)
• Proteinuria (usually sub-nephrotic)
• Edema
• Hypertension
• Reduction in GFR (an elevated serum creatinine)
• Active urine sediment (RBC's, WBC's, and RBC casts)
• Proteinuria (usually sub-nephrotic)
• Edema
• Hypertension

4

What causes proteinuria in nephritic syndrome

direct damage to the glomerular capillary wall induced by immunologic mechanisms leading to an increase in protein filtration. <3g/day b/c damage is focal (as opposed to nephrotic syndrome where entire capillary is affected)

5

What causes reduced GFR in nephritic syndrome

acute, inflammatory process within the glomerulus resulting in glomerular vasoconstriction and/or occlusion or thrombosis of some glomerular capillary loops

6

What causes active urine sediment in nephritic syndrome

The excretion of red cells, white cells and red cell casts reflects glomerular inflammation and disruption of the GBM

7

What causes hypertension in nephritic syndrome

consequence of salt and water retention.

8

What causes edema in nephritic syndrome

increase in tubular reabsorption of salt and water due to reduced, glomerular perfusion with well preserved tubular function leading to expansion of extracellular fluid volume

9

Does glomerulonephritis require a kidney biopsy for diagnosis?

YES

10

Syndromes associated with aggressive disease

Rapidly progressive glomerulonephritis (RPGN), Crescentic glomerulonephritis, Pulmonary-Renal syndrome

11

Rapidly Progressive Glomerulonephritis (RPGN) description, associations and therapy

Serum creatinine doubles or GFR falls by 50% within a few days to 3 months. Associated with Anti-GBM disease, ANCA associated vasculitis, Lupus. Therapy is aggressive: cytotoxic drugs and plasma exchange
Serum creatinine doubles or GFR falls by 50% within a few days to 3 months. Associated with Anti-GBM disease, ANCA associated vasculitis, Lupus. Therapy is aggressive: cytotoxic drugs and plasma exchange
Serum creatinine doubles or GFR falls by 50% within a few days to 3 months. Associated with Anti-GBM disease, ANCA associated vasculitis, Lupus. Therapy is aggressive: cytotoxic drugs and plasma exchange

12

Crescentic Glomerulonephritis description, associations and therapy

Crescents seen on biopsy. Caused by rupture of capillary wall. Usually associated with Anti-GBM disease, ANCA associated vasculitis and Lupus. Therapy is aggressive: cytotoxic drugs and plasma exchange

13

Pulmonary-renal syndrome description, associations and therapy

Glomerulonephritis with pulmonary capillaritis. Presents with nephritic syndrome and hemoptysis. Associated with Anti-GBM disease, ANCA associated vasculitis, Lupus. Therapy is aggressive: cytotoxic drugs and plasma exchange

14

List mechanisms for glomerulonephritis pathophysiology and associated diseases

1) Deposited immune-complexes: Lupus, MPGN 2) Antibodies specific for renal antigens: Anti-GBM disease, lupus? 3) Other causes of inflammation within glomeruli: Vasculitis (ANCA associated disease), Hemolytic uremic syndrome, C3 glomerulopathy

15

Describe locations of immune complex deposition within glomeruli

Mesangial, subendothelial, subepithelial, intra-membranous

16

Describe causes of immune complex formation

Antibodies to glomerular antigens, Antibodies to extra-glomerular antigens (Infections, Cancer, Drugs, foreign substances), Idiopathic (i.e. antibodies to unknown antigens)

17

How do immune complexes cause damage to glomerulus?

Complement is activated which activates PMNs that release proteases/oxidants causing capillary wall damage, which then leads to fibrin and crescent development. Also, macrophages can be directly activated by deposits.

18

Drugs used to treat glomerulonephritis

Immunosuppresives: Prednisone, cyclophosphamide, rituximab, IVIg

19

Which diseases is plasma exchange used and what does it do

Removes large plasma proteins such as auto-antibodies. Used in anti-GBM disease, severe ANCA associated vasculitis, and severe cryoglobulinemia

20

Post infectious glomerulonephritis etiology, pathology,

Abs + streptococcal Ags lodge in glomeruli and activate complement. Light microscopy: proliferative and exudative glomerulonephritis with infiltration of neutrophils and monocytes. Immunofluorescence (IF): granular deposits of IgG and C3 in the subendothelial, mesangial and subepithelial locations. EM: subendothelial and mesangial deposits, as well as the classic “subepithelial humps”.

21

Post infectious glomerulonephritis presentation, diagnosis, treatment

Edema, weight gain, hematuria, subnephrotic proteinuria, GFR decreased, severe hypertension. Elevated anti-streptolysin O, decreased C3. Usually get better without treatment

22

IgA nephropathy description/ presentation, associations, timeline and treatment

Mesangial IgA and mesangial expansion. Usually presents with hematuria +/- subnephrotic proteinuria. Associated with aberrant glycosylation of IgA1. Slowly progressive. Treated with prednisone, BP meds and ACEI
Mesangial IgA and mesangial expansion. Usually presents with hematuria +/- subnephrotic proteinuria. Associated with aberrant glycosylation of IgA1. Slowly progressive. Treated with prednisone, BP meds and ACEI

23

MPGN

Mesangial proliferation and thickening of GBM. C3 and IgG deposits in capillary walls and mesangium. Presents as nephritic syndrome, with hypertension. Many have Hep C. Poor prognosis.

24

Lupus nephritis causes

immune complex deposits: Mesangium (hematuria), subendothelial (nephritic syndrome and hematuria), and/or subepithelial (nephrotic)

25

Classifications of lupus nephritis

I-II: mesangial. III-IV: subendothelial. V: subepithelial. VI: scarring of >90% of glomeruli leading to renal failure.

26

lupus nephritis pathology and presentation

Immunofluorescence may demonstrate C3, IgG, IgM, IgA, and C1q all within the same kidney. These deposits appear as “lumps and bumps” Can present as nephritic or nephrotic

27

Anti-glomerular basement membrane disease causes and result

Caused by auto-antibodies to the NC1 domain of the alpha-3 chain of type IV collagen. Can be renal or pulmonary-renal. Often causes crescentic, necrotizing glomerulonephritis and RPGN

28

Goodpastures triad

pulmonary hemorrhage, iron deficiency anemia and GN associated with circulating antibody to GBM

29

Anti-glomerular basement membrane disease diagnosis and treatment

Check patients with nephritic syndrome, RPGN, or pulm-renal syndrome. Diagnosed by biopsy(linear IgG deposits) and detection of anti-GBM antibodies. Can see crescent formation. If untreated, progresses to end stage renal disease. Treated with cytotoxic drugs, corticosteroids and plasma exchange

30

ANCA associated vasculitis diagnosis

Check patients with nephritic syndrome, RPGN, or pulm-renal syndrome. Diagnosed by detection of ANCA (anti-neutrophil cytoplasmic antibodies) and biopsy

31

ANCA associated vasculitis disease examples, clinical findings and type of ANCA

Granulomatosis with polyangiitis (formerly called Wegener’s disease) causes nephritic syndrome with airway involvement- C-ANCA. Microscopic polyangiitis causes nephritic syndrome with airway involvement- P-ANCA. Eosinophilic granulomatosis with polyangiitis causes lung and skin vasculitis, asthma and eosinophilia- P-ANCA/C_ANCA. Renal limited vasculitis is P-ANCA
Granulomatosis with polyangiitis (formerly called Wegener’s disease) causes nephritic syndrome with airway involvement- C-ANCA. Microscopic polyangiitis causes nephritic syndrome with airway involvement- P-ANCA. Eosinophilic granulomatosis with polyangiitis causes lung and skin vasculitis, asthma and eosinophilia- P-ANCA/C_ANCA. Renal limited vasculitis is P-ANCA

32

Treatment of ANCA associated vasculitis

High mortality w/out treatment. Cytotoxic drugs, corticosteroids, Rituximab, plasma exchange

33

Cryoglobulinemia description, pathology, presentation

Cryoglobulins are antibodies that precipitate in the cold. In vivo they can form immune-complexes that precipitate in small vessels, causing vasculitis. Membrano-proliferative pattern of injury with subendothelial immune deposits. Associated with Hepatitis C and presents with proteinuria, hematuria, purpura

34

Henoch-Schonlein purpura description

in kids: (palpable purpura), arthritis, GI involvement (colic and bleeding) and glomerulonephritis. In adults: renal more severe, systemic less obvious. focal proliferative necrotizing glomerulonephritis, often with crescent formation, accompanied by mesangial and capillary wall deposits of IgA. Similar to IgA nephropathy