Nephritic syndrome 12/21 Flashcards
LM - enlarged and hypercellular glomeruli, IF - granular, EM - subepitheliam humps. What disease?
Acute PSG.
Pathoma. Nephritic syndrome. glomerular disorders characterized by …. 2
glomerular inflammation and bleeding
Nephritic syndrome. 4 points in pathoma
Limited proteinuria < 3,5g/day
Oliguria and azotemia
Salt retention with periorbital edema and hypertension
RBC casts and dysmorphic RBCs in urine
Pathoma. Biopsy nephritic
hypercellular, inflamed glomeruli
Pathoma. Nephritic. What molecules deposited and what is activated
Immune complex deposition activates complement;
C5a attracts neutrophils, which mediate damage.
In acute PSG what is deposits in EM?
Subepithelial.
What organs infection cause Acute PSG?
Pharynx and skin.
What are 2 serologican changes in acute PSG?
Increased ASO, decreased C3.
What 3 materials form deposits in acute PSG? Where are those deposits?
IgG, IgM, C3. Deposits are along GBM and mesangium.
When is seen acute PSG after A streptococcal infection?
2-4 weeks after infection.
Crescents consist of ……
Fibrin, macrophages, monocytes, glomerulal parietal cells, plasma proteins (C3b)
Rapidly progressive glomerulonephritis: Linear IF. What is mechanism?
Antibodies againts GBM and alveolar basement membrane, e.i. IV type collagen.
Rapidly progressive glomerulonephritis: Negative IF. What is mechanism?
In disease there are no Ig/C3 depositions.
Rapidly progressive glomerulonephritis: Granular IF. What is mechanism?
Immune depositions. IgG, C3, in acute PSG also IgM.
What is the type of hypersinsitivity in Goodpasture syndrome?
II type.
What is the treatment of Goodpasture syndrome?
Plasmapheresis.
What clinical appearance is in Goodpasture syndrome?
Hematuria and hemoptysis.
What ANCA is in Wegener granulomatosis?
c-ANCA/PR3-ANCA
What ANCA is in Microscopic polyangiitis?
p-ANCA/MP-ANCA
What means Pauci immune?
No Ig/C3 depositions (negative IF).
In what diesease are negative IF?
Wegener, microscopic polyangiitis, Churg-Strauss.
What ANCA is in Churg-Strauss?
p-ANCA/MP-ANCA
How to distinguish Churg-Strauss and Microscopic polyangiitis?
In Churg-Strauss granulomatous inflammation, eosinophilia, asthma.
How to distinguish Wegener and Microscopic polyangiitis?
In Wegener upper respiratory infections: sinusitis, rhinitis. Cough, nose deformation. Affected lungs, kidney, skin. Polyangiitis. Necrozing granulomatous inflammation. c-ANCA.
What type of inflammation is in Wegener and Churg-Strauss?
Necrozing granulomatous infalmmation.
Most often caused kidney disease in SLE?
Diffusive proliferative glomerulonephritis.
What 2 diseases can present as nephritic-nephrotic?
Diffusive and membrano proliferative glomerulonephritis.
LM - wire looping of capillaries, IF - granular, EM - subendothelial depositions (sometimes intramembranous). What disease? Deposits are consisted of ……..
Diffusive proliferative glomerulnephritis. IgG based often with C3
What diesease presents with wire looping capillaries?
Diffusive proliferative glomerulonephritis.
Where are deposits in diffusive proliferative glomerulonephritis?
Subendothelial granular.
Most common nephropathy worldwide?
IgA nephropathy (Berger disease).
How often apears hematuria in Berger disease?
Episodic.
What diseases appers concurrently with Berger disease?
Respiratory and GI infections.
What Ig are in Berger disease?
IgA
What Ig vasculitis is in Berger disease?
IgA vasculitis (Henoch - Schonlein purpura).
LM - mesangial proliferation, IF - mesangium deposits, EM - mesangial deposits. What disease?
IgA nephropathy.
In what disease mesangium is the main affected tissue?
IgA nephropathy.
Why Berger disease is associated with 2 other mucosal infections?
In Berger - IgA. In mucosal infections - increased IgA secretion.
What gene is affected in Alport syndrome?
COL4A5
What structure is affected by mutation in particular gene in Alport syndrome?
IV collagen, which is founded in basal membranes.
,,Can’t see, can’t pee, can’t hear a bee” - what disease?
Alport syndrome.
What sex chromosome is related with Alport syndrome?
X
EM - ,,basket weave”. What disease?
Alport syndrome.
What changes are in GBM in Alport syndrome?
Thinning and splitting of GBM,
Clinical appearance in Alport syndrome?
Eye problems - ocular disturbance (rethinopathy, lens dislocation), glomerunephritis, isolated hematuria, sensory hearing loss.
What is affected in Membranoproliferative GN?
Mesangium and GBM.
EM - Subendothelial, IF - granular. + mesangial IC. What disease?
Membranoproliferative GN
Membranoproliferative GN type I is associated with ……….
HBV, HCV.
Membranoproliferative GN type II is associated with ……….
C3 nephritic factor (IgG autoantibody).
What is dense deposit disease?
Membranoproliferative GN type II.
Mechanism in Membranoproliferative GN type II.
Increased C3 nephritic factor –> stabilized C3 convertase –> persistent complement activation –> decreased C3).
In what disease there is persistent complement activation? What structure is associated?
In membranoproliferative GN type II. C3 nephritic factor stabilizes C3 convertase.
Intramembranous deposits presents in …….. (2).
Membranoproliferative GN type II and may present in Diffusive proliferative GN.
Mesangial ingrowth –> GBM splitting –> tram track. What disease?
Membranoproliferative GN type I and II.
GBM splitting in Membranoproliferative GN is caused by ………
Mesangial ingrowth.
What stains are needed to indicate tram track?
PAS or silver.
What is tram track?
Doubling of GBM.
PSGN. IF - due to what depositions?
IgG, IgM, C3 - depositions along GBD -> starry sky
PSGN. Where are depositions?
Subepithelial deposits or ,,humps” along the GBM
PSGN. Clinical?
Hematuria (coca-cola urine)
Salt retention -> periorbital edema and hypertention
Oliguria
Malaise
PSGN - urine?
Dysmorphic RBCs, proteins (<3,5g/d), +/- RBC casts
UW. PSGN. What lab is decreased?
C3 and may be C4
UW. PSGN. What patway is activated in complement?
alternative –> therefore dec. C3.
UW. PSGN. Wat titers are increased?
Anti-DNAse and AHase (anti-hyaluronidase)
UW. PSGN. Wat titers are increased due to preceeding pharingitis?
ASO and anti-NAD (anti-nicotinamice-adenine-dinucleotidase)
UW. PSGN. Total complement?
Decreased.
UW. PSGN. Anemia?
Not seen
UW. PSGN. What prognosis?
in children - excellent (95 proc.)
Age is most important poor prognostic factor (conversely).
Increased age –> poor prognosis.
Progress to RPGN -> end stage renal failure.
UW. complement levels in IgA nephropathy?
Normal - likely due to weak complement-fixing activity of IgA copared to IgG and IgM
UW. PSGN vs IgA nephropathy.
PSGN - granular subepithelial depositions consisted with IgG, IgM, C3, decr complement + symptoms few weeks after infection.
IgA nephropathy - IgA depositions in mesangium, normal complement, occurs feww days after mucosal infection, and resolves after several days
UW. IgA nephropathy biopsy/IF?
Mesangial hypercellularity with mesangial IgA deposits
UW. IgA nephropathy with extrarenal symptoms –> what disease?
Extrarenal:
GI symptoms (abdominal pain) and bleeding, arthralgias, purpuric skin lesions (papable purpura on buttocks and legs)
IgA urinalysis?
hematuria
+ since its nephritic -> minimal protein
UW. how is called hematuria that occurs in IgA after infection?
synpharyngic hematuria
the most common cause/mechanism of nephritic syndrome?
immune complex depositions
Alport abbreviation?
cant see, cant pee, cant hear a bee
