nephritic syndrome Flashcards
basic principles of nephritic syndrome
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.
what does the biopsy of a nephritic kidney show?
there will be hypercellularity and inflammation of the glomerulus.
how is the damage of nephritic syndrome mediated?
there is immune-complex deposition which activates complement. C5a attracts neutrophils which mediate the damage.
post-streptococcal glomerulonephritis PSGN characteristics
nephritic syndrome that arises after group A beta-hemolytic streptococcal infection of the skin (impetigo) or pharynx (strep throat). this occurs with certain strains.
how does PSGN present?
2-3 weeks after infection as hematuria (cola-colored urine), oliguria, hypertension, and periorbital edema. usually in children, but can occur in adults.
what will PSGN look like on histology
hypercellular, inflamed glomeruli. there will be immune complex deposition with granular stain.
what will PSGN look like on EM?
sub endothelial humps on EM.
rapidly progressive glomerulonephritis RPGN characteristics
nephritic syndrome that progresses to renal failure in weeks to months. CRESCENTS in bowman space.
what are the crescents composed of in rapidly progressive glomerulonephritis
they are composed of fibrin and macrophages.
IgA nephropathy or burgers disease.
IgA immune complex deposition in the mesangium of the glomeruli. this is the most common nephropathy.
how does IgA nephropathy present?
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.
where is the IgA deposited in IgA nephropathy
in the mesangium.
what is the prognosis of IgA nephropathy?
it can progress to renal failure.
what is the classic presentation of bergers?
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.
alport syndrome characteristics
inherited defect in collagen, most commonly X-linked. results in thinning and splitting of the glomerular basement membrane.
how does aport syndrome present?
presents as hematuria, sensory hearing loss, and ocular disturbances.
what are the types of rapidly progressive GN?
this is dependent on the staining of the IF. there is linear, granular and pauci-immune.
linearly staining rapidly progressive GN
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.
common presentation of good pastures?
hematuria and hemoptysis classically seen in young adult males.
what two tissues does good-pastures disease affect?
the lung and the kidney. basement membrane antibody.
granular staining rapidly progressive GN?
this is typical of immune complex deposition and can indicate PSGN or diffuse proliferative GN.
what is diffuse proliferative GN and what disease is typical to cause this?
this is diffuse antibody-antigen deposition usually sub endothelial and is the most common type of renal disease in lupus.
negative IF or pauci-immune rapidly progressive GN
knee-jerk should be ANCA! this is characteristic of wagerers granulomatosis, microscopic polyangiitis, and charge-strauss.
wegeners granulomatosis ANCA?
associated with c-ANCA.
