nephritic syndrome

Question 1

basic principles of nephritic syndrome

Answer 1

glomerular disorders characterized by loss of blood and glomerular inflammation. there is limited proteinuria. oliguria and azotemia. there will salt retention with periorbital edema and hypertension. RBC casts and dysmorphic RBCs in the urine.

Question 2

what does the biopsy of a nephritic kidney show?

Answer 2

there will be hypercellularity and inflammation of the glomerulus.

Question 3

how is the damage of nephritic syndrome mediated?

Answer 3

there is immune-complex deposition which activates complement. C5a attracts neutrophils which mediate the damage.

Question 4

post-streptococcal glomerulonephritis PSGN characteristics

Answer 4

nephritic syndrome that arises after group A beta-hemolytic streptococcal infection of the skin (impetigo) or pharynx (strep throat). this occurs with certain strains.

Question 5

how does PSGN present?

Answer 5

2-3 weeks after infection as hematuria (cola-colored urine), oliguria, hypertension, and periorbital edema. usually in children, but can occur in adults.

Question 6

what will PSGN look like on histology

Answer 6

hypercellular, inflamed glomeruli. there will be immune complex deposition with granular stain.

Question 7

what will PSGN look like on EM?

Answer 7

sub endothelial humps on EM.

Question 8

rapidly progressive glomerulonephritis RPGN characteristics

Answer 8

nephritic syndrome that progresses to renal failure in weeks to months. CRESCENTS in bowman space.

Question 9

what are the crescents composed of in rapidly progressive glomerulonephritis

Answer 9

they are composed of fibrin and macrophages.

Question 10

IgA nephropathy or burgers disease.

Answer 10

IgA immune complex deposition in the mesangium of the glomeruli. this is the most common nephropathy.

Question 11

how does IgA nephropathy present?

Answer 11

in childhood as episodic gross or microscopic hematuria with RBC casts, usually followed by mucosal infections such as gastroenteritis. the IgA production is produced during infection.

Question 12

where is the IgA deposited in IgA nephropathy

Answer 12

in the mesangium.

Question 13

what is the prognosis of IgA nephropathy?

Answer 13

it can progress to renal failure.

Question 14

what is the classic presentation of bergers?

Answer 14

hematuria that begins a day or two after an upper respiratory tract infection. (as opposed to PSGN which occurs weeks after). less commonly, but still very likely it will occur after a gastrointestinal or urinary tract infection. renal function usually stays true, but it can produce renal failure. important to keep in mind: henoch-shonlein purpura.

Question 15

alport syndrome characteristics

Answer 15

inherited defect in collagen, most commonly X-linked. results in thinning and splitting of the glomerular basement membrane.

Question 16

how does aport syndrome present?

Answer 16

presents as hematuria, sensory hearing loss, and ocular disturbances.

Question 17

what are the types of rapidly progressive GN?

Answer 17

this is dependent on the staining of the IF. there is linear, granular and pauci-immune.

Question 18

linearly staining rapidly progressive GN

Answer 18

this is typical of good-pastures syndrome and characterized by basement membrane antibodies. this is typically an antibody to collagen in glomerular and alveolar basement membranes.

Question 19

common presentation of good pastures?

Answer 19

hematuria and hemoptysis classically seen in young adult males.

Question 20

what two tissues does good-pastures disease affect?

Answer 20

the lung and the kidney. basement membrane antibody.

Question 21

granular staining rapidly progressive GN?

Answer 21

this is typical of immune complex deposition and can indicate PSGN or diffuse proliferative GN.

Question 22

what is diffuse proliferative GN and what disease is typical to cause this?

Answer 22

this is diffuse antibody-antigen deposition usually sub endothelial and is the most common type of renal disease in lupus.

Question 23

negative IF or pauci-immune rapidly progressive GN

Answer 23

knee-jerk should be ANCA! this is characteristic of wagerers granulomatosis, microscopic polyangiitis, and charge-strauss.

Question 24

wegeners granulomatosis ANCA?

Answer 24

associated with c-ANCA.

Question 25

microscopic polyangiitis ANCA?

Answer 25

this is p-ANCA

Question 26

churg-strauss ANCA?

Answer 26

this is p-ANCA

Question 27

how do we distinguish churg-strauss from MPA?

Answer 27

there will be asthma, eosinophilia, and granulomatous inflammation in CS.