6 Glomerular disease Flashcards
Nephrotic syndrome
- Proteinuria >3.5g/day
- Hypoalbuminemia
- Edema from loss of plasma oncotic pressure
- Hyperlipidemia
(lipiduria and hypocoaguability from C and S loss)
3 different kinds of immune kidney disease
1) Immune complex deposition between endothelum and BM as well as deposition in BM and subepithelium.
2) Antibodies to BM or to glomerular antigens
3) Cytokines created by inflammatory cells
Nephritic
- Mild proteinuria
- Hematuria- RBCs, RBC casts, and dysmorphic RBCs
- HTN
- Edema
Acute Glomerulonephritis causes
- IgA Nephropathy
- post infectious GN
- Anti GBM disease/ Goodpasteure’s
- Small vessel Vasculitis
- Lupis Nephritis
- Membranoproliferative glomerulonephritis
IgA Nephropathy Clinical presentation
Nephrotic and Nephritic
1) Hematuria (frequently with URI)
* 50-60% episodic gross hematuria
* 30% persistant microhematuria
* 10% acute GN or nephrotic syndrome
2) Proteinuria
3) Dysuria and loin pain
4) HTN in advanced patients
IgA Nephropathy Histo
- Mesangial hypercellularity
- IF: mesangial IgA deposition
- EM: Electron dense deposits
IgA nephropathy course and management
- 40% will develop CKD
- Fish oil may low progression
- ACEi for BP control
- Corticosteroids may be helpful for progressive disease
Henoch-Schoenlein Purpura Presenation
- Small vessel vasculitis
- IgA Deposition in skin and many organs
- Lower body nonblanching purpura
- Arthralgias
- ABD pain, vomiting, melena, hematochzia
- hematuria, proteinuria, Rare RF
Post- infectious GN
Nephritic
- HTN, azotemia, oliguria, cola/tea colored urine
- low c3
- anti streptolysin O
- RBC casts
- Mild proteinuria
- follows GAS- 7-14d post pharyngitis, 14-28 post skin infection
Post-infectious GN histo
- Enlarged hypercellular glomeruli
- diffuse proliferation of mesangial, endocapillary, and neutrophils
- sometimes crescenteric
- IF: granular capillary wall and IgG and C3 in mesangium
- EM: Mesangial and sub epithelial HUMP LIKE DEPOSITS
Post strep GN treatment and course
95% children- conservative management
*1% KF
60% of adults recover promptly
Rapidly progressing progressive GN Causes
Nephritic syndrome
- Crescenteric
- Rapid progression to RF
1) Anti GBM- Goodpastures
2) Immune complex GN
* Lupis
* Post- infectious
* Cryoglobulinemia
3) Anca Assocaited GN (pauci immune)
Rapid progressive GN Histo
Segmental necrosis early in the disease
Cellular crescent
Anti GBM/Goodpasteure’s Signs/causes
Nephritic
- M>F
- Hemoptysis
- Pulmonary infiltrates
- GN
Caused by circulating anti GBM
* a3 chain of collagen IV
Goodpastures diagnosis/treatment
- anti GBM antibody test
- Linear IgG and C3 on IF
- crescenteric
Treatment
- Plasmapheresis
- prednison
- Cytoxan
Pauci-immune GN
Nephritic
* Idopathic, Microscopic polyangitis, Wegeners Granulomatosis, Churg-strauss syndrome
Histo
- little immune deposition on IF
- Crescenteric
- ealy segmental necrosis
Wegener’s Granulomatosis
Nephritic
- c-ANCA pos in 80%
- UR smptoms - sinusitis, nasal lesions, hemoptysis
- purpura
- Nephritis
Biopsy-
Pauci-immune crescenteric GN
Nephrotic Syndrome
Primary
- Membranous nephropathy
- Focal Segmental Glomerulosclerosis
- Minimal Change Disease (80% of kiddos with nephrotic)
Secondary:
- Diabetes, SLE, Amyloidosis
- HIV, Hep B and C, Syphilis
- NSAIDs, gold, penicillamine
Nephrotic syndrome treatments
- ACE or ARB to reduce proteinuria
- Statin to decrease lipids
- Diuretics and salt restriction
Minimal change disease course and presentation
Nephrotic
- 2-6 yo.
- 5% ESRD progression
- spontaneous remissionscan occur
- Steroid treatment induces remission but 75% relapse
- less relapses after puberty
Minimal change disease causes in adults
NSAIDs
Neoplasms HODGKINS
Syphilis/HIV
Minimal change histo
Appears normal on LM
IF negative or IgM positive
EM: PODOCYTE FOOT PROCESS EFFACEMENT
Minimal change treat
Corticosteroids (may need longer treatments in adults)
Membranous Nephropathy
Nephrotic
- Heavy proteinuria
- later Azotemia and HTN
- Hypercoagulability and renal vein thrombosis.
*Most common caucasian adult nephrotic disease
20% cause by secondary causes
*HBV, SLE, Neoplasm- lumg, colon, stomach, breast, non hodgkins, drugs
Membranous nephropathy histo
LM: GBM spikes and diffuse thickening,
IF: IgG granular deposits on GBM (Granular BM staining)
EM: Subepithelial dposits
Membranous neuropathy course
1/3 spontaneous remission
1/3 partial stable remission
1/3 slow progression
Treat: if no poor prognositc factors
- Sometimes ACEi or ARBs
- Steroids/immunosuppressants
FSGS (foccal sgmental glomerulosclerosis) overview
Nephrotic (most common AA idopathic nephrosis) Agressive: *HTN/HEmaturia *Renal dysfunction * ESRD 5-20 years later
FSGS Types (3)
Primary (Acute onset nephrotic syndrome)
Secondary (slow increasing proteinuria and renal insufficiency)
Hereditary ( Slight diaphragm mutations)
FSGS causes
- HIV
- NSAIDs/Heroin
- Massive obesity
- Previous glomerular disease
- loss of functional renal mass
FSGS histo
LM: focal and segmental glomerular sclerosis:
*Capillary Collapse, Hyaline and lipid deposition (adhesion to bowmans capsule
IF: Negative or IgM and C3 in mesangium or scars
E: Podocyte effacement and
FSGS Treatment
- ACEi to decrease proteinuria
- corticosteroids may induce remission
- immunosuppresives for relapsing and Steroid refractory
- 50% ESRD at 10 yrs.
Mixed nephrotic nephritic
- Membranoproliferative GN
* SLE
MPGN (membranoproliferative GN)
Nephrotic or nephritic
- Proteinuria and Hematuria
- HTN in 33% patients
- LOW C3
Secondary MPGN causes
SLE
Cryoglobulinemia
HCV, HBV, endocarditis, abscesses
Neoplasm
MPGN histo
- LM: Hypercellular glomeruli, endocapillary proliferation, LOBULAR GLOMERULI
- IF: Granular C3 deposition
- EM: Subendothelial deposits
SLE
- Can cause many different renal diseases- Commonly diffuse and proliferative GN
- Lupis with HTN is frequently GN
SLE nephritis Histo
LM: lotts of immune cells
EM: Subendothelial deposits
SLE Nephritis treatment
*BP control
*Lipid control
* Extrarenal treatments
ClassIV: 25% renal failure by 5-10 yrs
