Glomerular Disease 4 Flashcards
What age group typically has post infectious nephritis/post-streptococcal glomerulonephritis?
5-15 years
When does the GN associated with post-streptococcal infection usually occur?
1-4 weeks after streptococcal infection of the pharynx or skin
What type of HSR type is post-streptococcal GN?
type 3 HSR
What infection usually leads to post-streptococcal GN?
Group A B hemolytic strep infection or skin infection
What is the latent period before onset of GN causing PSGN?
2-4 weeks latent activity
Describe immune complex patter on IF of one with PSGN?
lumpy-bumpy pattern
Is characteristic deposit of complex in PSGN subepithelial or sub endothelial?
supithelial
What are the CF hinting you to believe that a child has PSGN?
Children
-preceding history of sore throat (1-2 weeks)
-acute onset of malaise, fever, oliguria, periorbital edema
-smoky or coca-cola colored urine
-HTN mild to moderate
Nephritic presentation remember
Adults
Atypical features
Acute onset of HTN, edema, azotemia
What may you see upon microscopy of one with PSGN?
In severe cases - epithelial crescent +ve
Hypercellularity and neutrophilic infiltration
Increased cells within the glomerulus
Obliterated capillaries- end epithelial proliferation
Describe characteristic EM of one with PSGN.
sub epithelial deposits commonly seen as humps
Upon IF of one with PSGN what can be seen?
IgG and C3 deposits along BM (sub epithelial) and mesangium
What are some characteristic lab findings for one with PSGN?
Urine- creanated cells: red cell casts (possible cola colored urine)
ASO elevation (anti strep ab titre)
C3 levels decreased (for 1st 2 weeks
BUN may be elevated
Prognosis of PSGN in kids?
good
Prognosis of PSGN in adults?
relatively bad compared to having the condition in kids
40% progress to RPGN or chronic GN
What are some bacterial infections that can lead to Postinfectious GN?
staphylococcal endocarditis, pneumococcal pneumonia and meningococcemia
What are some viral diseases that can lead to non streptococcal acute GN?
Hep B, Hep C, mumps, HIV infection, varicella and infectious mononucleosis
What are some parasitic infections that can lead to post infectious GN?
malaria, toxoplasmosis
Are histology and EM for those with Nonstreptococcal acute GN the same as those with PSGN?
yes
What is the most common type of GN “worldwide”?
IgA nephropathy
In Berger’s Disease IgA typically deposits in what area of the glomerulus?
mesangial regions
Can diagnosis of IgA be made on LM?
no
A Disease that presents with nephritic presentation that is a frequent cause of recurrent gross or microscopic hematuria?
Berger’s Disease
What conditions will Berger’s Disease recur in?
transplanted kidney
What are other primary GN which will recur in transplanted kidneys?
FSGS, IgA nephropathy, MPGN type 2
Explain pathogenesis behind Berger’s Disease>
Genetic or acquire abnormality of immune regulation > Increased mucosal IgA synthesis ( in response to respiratory (pharyngitis) or GI exposure (acute gastroenteritis) to environmental agents) e.g viruses, bacteria(URI), food proteins)
IgA1 immune complexes trapped in mesangium activate alternative complement pathway and initiate glomerular injury
What is a GI disease that IgA nephropathy commonly presents with?
gluten enteropathy (celiac disease)
What is a condition that IgA nephropathy has some common presentation with?
HSP
Disease of what organ can bring about secondary IgA nephropathy?
liver disease
Histology of glomeruli in one with Berger’s disease may have what characteristics?
normal
mesangioproliferative glomerulonephritis
focal proliferative glomerulonephritis
rarely crescentic GN
What conditions is there an increased risk of progression of Berger’s disease?
Onset in old age, heavy proteinuria, HTN, and extent of glomerulosclerosis on biopsy
Features of HSP.
Purpuric skin lesions that can involve arms but mostly involves buttocks and legs
abdominal pain and vomiting
Intestinal bleeding
renal abnormalities
preceding viral infection (URI, pharyngitis)
non migratory arthralgia
IgA nephropathy
Can a presentation of HSP in adults include gluten sensitive enteropathy and dermatitis herpetiformis?
yes
T/F. IgA nephropathy and Henoch-Schonlein pursuer are spectra of the same disease.
True
What is a nephritic syndrome characterized by rapid and progressive losss of renal function?
RPGN
Prognosis of RPGN?
death from renal failure within weeks month is untreated
Histology of RPGN.
crescentic GN
Diffuse crescents formed by proliferation of parietal cells, monocytes and lymphocytes
Fibrin contribute to crescent formation
What is the best treatment for RPGN?
early diagnosis and use of cytotoxic drugs to disrupt crescents are the key to halt disease progression
Describe gross morphology characteristic of crescentic GN.
enlarged, pale kidneys with petechial hemorrhages on cortical surface
How does crescentic GN look upon LM?
proliferation of parietal epithelial cells admixed with macrophages and fibers filling Bowman’s/urinary space
What stain shows locations of crescents in crescentic GN?
PAS stain shows location of crescent
Be able to classify different causes for Crescentic GN based on if. Create a slide.
Linear deposits along the GBM are characteristic to what nephritic syndrome?
anti GBM/ Good Pasture’s
What type of deposits are characteristic of Pauci Immune?
no deposits
Post infectious causes of crescentic GN have what pattern?
granular
C/F of Goodpasture’s Syndrome.
hemoptysis and RPGN
Treatment for Goodpastures?
plasmapheresis and immunosupression
HLA DRB1 is associated with what nephritis syndrome?
Goodpastures ag
Can good pastures syndrome develop in a patient with Alport’s?
RPGN Immune Complex Deposition that has granular deposits on IF are due to what complications?
complication of post infectious, IgA nephropathy, MPGN, SLE
What are the Labs to check for SLE?
ANA/dsDNA+ve
Prescription for RPGN?
treat underlying condition
Does Wegner’s granulamatosis have a +ve or Negative IF?
negative
LM of kidney in one with Wegner’s?
neurosis of glomeruli
Labs to check of Wegner’s Granulomatosis?
C-ANCA +ve
Clinical features of Wegner’s granulomatosis?
saddle nose; cavitary lung lesion
-hemoptysis
- necrotizing granulomas
A person with C-ANCA +ve labs will have ab directed agains what?
proteinase 3 of neutorophils
What type of string pattern is characteristic of C ANCA?
cytoplasmic
Ab are directed agains what in p-ANCA +ve labs?
myeloperoxidase of neutrophils
p-ANCA + conditions?
microscopic polyangitis, Churg Strauss, PAN
What is the pathology triggering crescent formation in RPGN?
disruption of BM
fibrin leaks into Bowman’s space triggering crescent formation
Be able to reproduce Chart on comparison and Contrast between Alport’s Syndrome and Thin BM disease.
What are some characteristic features upon EM of Alpert’s?
thickening alternating with attenuation (thinning)
pronounced BM splitting (black thin arrows)
