Nephritic Syndromes Flashcards
What is azotemia?
Kidney damage and incr BUN and creatinine
Describe Nephritic syndrome in one word.
decr GFR
Four outcomes of decr GFR
1.** Slight protenuria** (decr. plasma oncotic pressure)—->EDEMA
2. RBC Cast (describe as Coca-cola urine or smoky)
3. Decr. Blood supply to kidneys–>azotemia
4. Faliure to filter Na+ and Cl- –>Incr. Plasma Hydrostatic pressure–>EDEMA+ Hypertension
Presentation in Nephritic syndrome?
-<3.5g/day of protein, Dark urine, swelling (periorbital or lower limbs), Fatigue (uremia).
Diagnosis
Dark urine,slight protenuria, fatigue and on auscultation you hear pericardial rub and EKG shows ST segment elevation everywhere..
Nephritic syndrome pesenting with** pericarditis **due to uremia (irridates pericardium)–>progress to pericardial effusion.
Where is cast formed in the anatomy of nephron?
collecting tubules because urine is mostly concentrated.
Is Hematuria a unike diagnosis of Nephritic syndromes?
NO.
-Urethral, ureter,bladder,penile ruptures can all cause bleeding but NOT cast.
Name the 3 divisions of your Nephritic syndromes?
1.Type 3 Hypersensitivity(Acute Post-strep Glomerulonephritis/IgA Nephropathy/Diffuse Proliferative Glomerulonephritis)
2.Multiple cause(Membranous Proliferative Glomerulonephritis/Rapidly Progressive Glomerulonephritis/Pauci Immune Vasculitis)
**3.Collagen related **(Alport syndrome)
Diffrent types of Group A Strep skin infections?
pharyngitis,cellulitis,impetigo(“honey-crusted”lesions),erysipelas
Diagnosis
Honey-coomb crusted lesion on face in a 6yr old, 1 week later has dark urine with azotemia, 24Hr hour urine test shows 2.5g /day
PSGN
Can you get PSGN and Rheumatic Fever at same time?
NO, either **M protein **is Nephrogenic or rheumatogenic.
JONES criteria uesed for?
Dukes citeria?
-RF
-Endocartitis
What do you see?
PSGN
IF,LM,EM
-IF:subEpithelial IgG,C3 deposits IgM(Granular pattern)”starry sky”
-LM:Hypercellular glomeruli**
-EM: **subEpithelial Humps **(usually diagnostic)
Why PSGN and RF takes at least 1-2 to develop?
Your body has to create immune response towards M protein.
PSGN has decr C3?
Yes, when immune complex deposits subepi it will activate continously complement system,
Diagnosis
DNAse and streptolysin O antibodies on serum with pharyngitis or impetigo
Group A Strep
Diagnosis
SubEpithelial Humps
prognosis of Children and Adult in PSGN?
-children (95% reslove)
-Adults(may progress to renal insufficency), may also be causd by Stap Aureus.
RBC cast
-Achantosis (Di-morphic RBC)–>squeezing througt nephron (looses shape)
-RBC cast
Two important virulence F for Group A strep
M protein, Exotoxin B (ass with the skin infections)
Diffrence between RF and PSGN
RF=Type 2 Hypersensitivity
PSGN=Type 3 Hypersensitivity
IgA nephropathy (Berger Dis.) characteristics
-Immune deposits in mesangium intraglomerular cell (NOT extraglomerular)–> stimulates cytokine (mediates damage)
-Ass with GI and Resp infections.
-IF: vey specific–>immune complex mesangium. Still considered granular pattern but very diffrent. Involves IgA deposition
-*LM; *mesangial proliferation.
-IF; mesangial deposition
-LM;mesangial proliferation
IgA nephropathy
Diagnosis
recurrent episodes of hematuria since childhood, episodes following Upper Resp Tract infect, diarrheal illnes, slow renal decline…
Iga Nephropathy (Berger dis.)
**Diffrence **timeline between IgA nephropathy and PSGN
PSGN (1-2 weeks)
IgA nephropathy (1-2 days)
Berger disease mechanism?
-Abnormal IgA antibodies produced,where your immune system makes IgG against them a promotes deposistion on your mesangial cells–>activates cytokine mediated damage.
Diagnosis
decreased glycosylation of O-linked glycans in Hinge region of Ig
IgA nephropathy (Berger disease)
IgA nephropathy ass confusion?Why?
-PSGN
-IgA vasculitis (Henoc Purpura)
-IgA vasculitis can cause Berger disease, but you see palbable purpura rash in lower extremity and buttocks, arthriti.
-IgA nephropathy only causes nephropathy.
Diffuse Proliferative Glomerulonephritis (DPGN) highly Ass with….
SLE (Lupus Nephritis)
Type of deposition
**SubEndothelial deposits. **
-“Wire Looping”–hypercellular
DPGN,meaning…
Diffuse means >50% og glomeruli are affected.
Rapidly Progressive Glomerulonephritis (RPGN) Types (3)
1.Type 1: Idiopathic or Good Pasture syndrome–>Type 2 Hypersensitivity
2.Type 2 (PSGN or SLE Nephritis)–>Type 3 Hypersensitivity
3.Type 3 (Paucini immune vasculitis)–>NO immune complex formation
What ass does Exotoxin B of Group A strep has?
Skin infections.
IgA nephropathy is ass with GI and Respiratory secretions,Why?
-When you get infections of this sites, your immune system secrtes more abnormal IgA.
Does IgA nephropathy decr C3?
NO
What deposits does IgA nephropathy involve?
IgA
Prognosis of RPGN (Rapidly progressing Glomerulonephritis)
-Poor prognosis, RUPTURE of basement membrane–>rapid decline of kidney function.
- **Fibrinoid necrois. **
Diagnosis
Anti-GBM antibodies towards Type 4 collagen in the alpha-3-chain.
1.Type of Hypersensitivity?
2.Presentation
3.Treatment
-Good Pasture syndrome (Type 1 RPGN)
1.Type 2 Hypersensitivity
2.Hematuria (kidney damage) and Hemoptysis (alveolar damage)
3.Plasmapheresis
Composition?
-“Crescent-moon shape”(mainly made up of FIBRIN)
-Seen in **ALL **Rapidly progressing Glomerulonephritis.
-Fibrin (fibrinoid necrosis)+plasma proteins (C3)+monocyte+macrophages+Bowman space parietal cells
-The **expansion of Bowman space is due to parietal cell proliferation **
What type of deposit do you see in Type 2 Rapidly Progressive Glomerulonephritis?
**Sub-Endothelial **
IM:Granular pattern.
IF:Linear pattern (deposits in only GBM)
-Good Pastures
Types of Paucini immune vasculitis?
1.Eosinphilic Granulomatosis
2.Microscopic Granulomatosis
3.Granulomatosis with Polyangitis (Wegner)
Diagnosis
Hemoptysis,Hematuria, nasal septal perforation,otitis media, mastotitis,
-C-ANCA/PR3-ANCA
-Non-casseating Granulomas
Granulomatosis Polyangitis
What does all Pauccini immune vasculitis have in common?
-All relate ANCAS’s
-All are** IF NEGATIVE **(NO immune complex is present)
-crecentic moon shape
Diagnosis
Hematuria,Hemoptysis
-MPO-ANCA/P-ANCA
-NO granulomas
-Ass with penincillin
Microscopic Polyangitis (NO URT association)
How do we diagnose Pauccini syndrome since they all are IF negative?
-Look for ANCA involvmenet.
Celle responsible for Crescent moon shape in RPGN
Bowna’s parietal cells
DIAGNOSIS
Ass with ANCA
Pauccini immune vasculitis
Diagnosis
“Can’t see, can’t pee, can’t hear a bee”
***Alport syndrome
-Eye problems (retinopathy, ant lenticonus), sensineural deafness, glomerulonephritis.
-Irregular thickening of basement membrane.
-Alport syndrome mutation ?
-People mayorly affected
X linked Dominant of Type 4 collagen.
-Affects mayorly very youn MALE
Diagnosis
-Alport syndrome
-EM:Irregular thin areas and thicker areas “basket weave”
Patient with some collagen disorder, describe lesion.
Alport syndrome (retinopathy)
IgA nephropathy is damage mediated by what?
Cytokine
Where does IgA Nephroptahy deposist?
mesangial cells.
Differential diagnosis of hematuria girl following URT infection 1 week ago?
-IgA nephropathy and PSGN.
Dipstick of 2g per day?and other examples
2+
3g=3+
4-20g=4+