nephritic pathology

Question 1

Acute nephritic syndrome hallmarks

Answer 1

Increased glomerular capillary permeability (hematuria and proteinuria) and decreased glomerular filtration rate (Na and H20 retention, azotemia and hyperkalemia)

Question 2

What is chronic renal failure

Answer 2

nephron loss leads to decreased GFR, uremia

Question 3

Define focal vs diffuse

Answer 3

Focal affects 50%

Question 4

Define segmental vs global

Answer 4

Segmental affects part of a glomerulus whereas global affects the whole glomerulus

Question 5

What is thin basement membrane disease

Answer 5

AKA benign familial hematuria. Mutations in genes for collagen IV. Diagnosed based on EM

Question 6

Alports Disease triad, cause/genetics, presentation

Answer 6

•Triad of nephritis, deafness, and ocular lesions. Due to X-linked mutation in alpha-5 chain of collagen IV. Cant form normal basement membranes. Diagnosed by basket-weave pattern on electron microscopy. Usually progresses to end stage renal disease

Question 7

Types of proliferative glomerulonephritis

Answer 7

Endocapillary (occlusion of capillary loops), Mesangial, Epithelial – crescents (reaction to severe injury to, glomerular capillaries), Inflammatory cells

Question 8

IgA nephropathy presentation, morphology, pathogen, therapy and prognosis

Answer 8

Usually asymptomatic hematuria, can be nephritic or nephrotic; often coincides with URI or GI infection, liver disease. Usually focal mesangial (can be MPGN). Mesangial IgA and complement are pathogen. Therapy is steroids and some progress to ESRD

Question 9

Henoch-Schonlein purpura presentation and cause

Answer 9

Systemic IgA vasculitis due to deposition of IgA immune complexes. May involve kidneys, skin, joints, GI tract. Renal biopsy looks like IgA nephropathy. Usually occurs in kids <10 and is often after URI. Presents with purpuric rash on arms and legs

Question 10

Post infectious glomerulonephropathy clinical, morphology

Answer 10

Usually poststreptococcal infections (other bacterial etiologies possible), acute nephritic syndrome. Causes a diffuse, proliferative, exudative glomerulonephropathy

Question 11

Post-infectious GN pathology,serology, therapy and prognossi

Answer 11

Exogenous immune complexes result in subepithelial deposits, granular glomerular basemement membrane on IF with a starry sky appearance. Serology shows elevated anti-streptolysin O. Therapy is supportive ad 99% of children recover completely while 60% of adults recover completely

Question 12

Crescentic glomerulonephritis cause and presentation

Answer 12

Crescents: histologic sign of severe acute glomerular disease. Caused by fibrinoid necrosis of capillaries. Presents as rapidly progressing glomerulonephropathy. Glomeruli usually heal with a scaar
Crescents: histologic sign of severe acute glomerular disease. Caused by fibrinoid necrosis of capillaries. Presents as rapidly progressing glomerulonephropathy. Glomeruli usually heal with a scaar

Question 13

Which focal segmental necrotizing and cresentic glomerulonephritis have linear IF staining

Answer 13

Goodpastures and anti-GBM

Question 14

Which focal segmental necrotizing and cresentic glomerulonephritis have granular IF staining

Answer 14

IgA, SLE, endocarditis, idiopathic

Question 15

Which focal segmental necrotizing and cresentic glomerulonephritis have NO IF staining

Answer 15

Wegeners, microscopic PAN, Churg-Strauss, idiopathic

Question 16

List serum studies for goodpastures, lupus, vasculitis and endocarditis

Answer 16

Goodpastures: anti-GBM Abs. Lupus: ANA, anti-dsDNA, complement depletion (C3 & C4). Vasculitis: ANCA (ex. MPO – cationic protein that can fix complement). Endocarditis: blood cultures

Question 17

Anti-glomerular basement membrane disease cause and presentation

Answer 17

Caused by IgG against collagen IV in basement membrane. If reanl only, anti-GBM disease. If renal and lung, goodpastures. Presents as rapidly progressive glomerulonephritis and hemoptysis. Linear appearance on IF

Question 18

Crescentic glomerulonephritis clinical, morphology, pathogen, therapy and prognosis

Answer 18

Usually RPGN with crescents on morphology. Caused by immune complexes, anti-GBM, systemic disease (pauci-immune vasculitis, SLE). Therapy is steroids, cytotoxics, plasmaphoresis and prognosis is poor.

Question 19

Lupus nephritis cause, pathology, presentation

Answer 19

Caused by anti-ANA and anti-dsDNA immune complex deposition. IF shows granular immune-complex pattern with positive IgG, IgA, IgM, C1q, and C3. Presents as nephritic, nephrotic, RPGN, or mixed nephritic/nephrotic

Question 20

WHO classification of Renal lupus

Answer 20

Class I: minimal changes by light microscopy. Class II: Mesangial glomerulitis (A: Normal by light microscopy. Mesangial deposits by EM and/or IF. B: mild/ moderate mesangial hypercellularity/sclerosis). Class III: Focal, segmental glomerulonephritis. Class IV: Diffuse glomerulonephritis. Class V: membranous nephropathy

Question 21

Treatment of lupus nephritis

Answer 21

steroids, cytotoxics