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Glomerular Disease Clinical Syndromes, May Present as Nephritic - Coggle…
Glomerular Disease Clinical Syndromes
Nephritic
Hematuria W/ RBC casts
Hypertension
Proteinuria
Azotemia
Acute Poststreptococcal Glomerulonephritis (APSGN)
Glomerular inflammation assoc with strep infection
Children, and has hypocomplementemia
Immune complex formation in subendothelial space
Nepritogenic strains (step pyrogrenic exotoxin B)
Hypercellular glomeruli!
Low C3 []
IgG
Membranoproliferative GN (MPGN)
Glomerilonephritis
Immuned-Mediated (Type 1)
Chronic infection (Hep B and C) Systemic autoimmune disorder, maligancny.
LM: lobular
IF: granular IgG and C3
GBM SPLITING
C3-Dominant (Type 2):
Dense Deposit Disease (DDD)
Subtype assoc. with alternate complement activation
Excessive activation
LM: lobular, GBM thickening with a tram-track
IF: Granular C3, mesangial staining)
EM: RGBM splitting and Ribbon like dense deposits in lamina densa
Nephrotic Syndrome
Proteinuria 3.5g/day
Hypoalbuminemia, lipidemia, edema
Minimal Change Disease (CHILDREN)
*Dramatic response to CORTICOSTERIOD Therapy
Prototypic Nephrotic Syndrome
Children with resp infection, allergy, immunization
Adults follow Hodgkins Lymphoma and NHL or NSAID's
Severe Selective proteinuria
Visceral epithelial cell (podocyte) damage
Effacement
Focal Segmental Glomerulosclerosis (FSGS): IgM and C3
Primary disease of unknown etiology OR secondary in association with other condtion
Secondary FSGS
Fa
HIV
Triad:
Collapsing FSGS
Cystic Tubule Dilation
Tubuloreticular Inclusions
Epithelial cell hyperthrophy(e.g., mitosis)
Drug-Induced
Adaptive (stress)
Scarring
Primary FSGS
Most common cause: DIABETES
A1 variant in African-Americans
Em: Effacement of podocyte and Podocyte detachment
Immune complexes NOT present
Membranous Nephropathy IgG and C3
Subepithelial immune deposits
Primary or secondary
Autoantibody to Phospholipase A2 Receptor (PLA2) or antibody to various cationic (planted) antigens
LM: Basement membrane
spikes
IF: granular
EM:
Subepithelial electron-dense deposits
Effacement of foot processes
Follows a resp infection
Chronic Glomerulonephritis
/Chronic Kidney Disease
Without an identifiable antecedent glomerulonephritis
egfr<60ml/min for 3 months
Stage 3: Moderate: 30-59mL/min
State 5: Renal Failure ,15mL/min (dialysis needed)
End-Stage: ,15mL/min
Rapidly Progressive Glomerulonephritis (RPGN) (CRESCENTS and Fibrinoid necrosis)
Nephritic presentation that is rapidly progressive
Renal failure days-weeks
Pauci-Immune
Assoc. Vasculitis
Antinuetriphilic Cytoplasmic Antibody Autoantibodies
(ANCA)
Microscopic Polyangiitis
Necrotizing small vessel vasculitis
Fragmentation of neutrophils (Leukocytoclastic vasculitis)
P-ANCA
Eosinophilic Granulomatosis W/. Polyangiitis
(Churg-Strauss Syndrome)
Assoc. eosinophilia and allergy
Pulmonary involvement
P-ANCA
Granulomatosis W/. Polyangiitis
(Wegeners Granulomatosis)
Necrotizing granulomas upper/lower res tract Lungs
Necrotizing vasculitis
C-ANCA
Immune Complex Mediated
Complication of any glomerular disease that is involved with immune complexes
Anti-GBM Antibody Mediated
Assoc. W., Hemmorhage
Assoc. w/. smoking; resp infection
50% will have pulmonary hemorrhage (goodpasture syndrome)
Autoantibody to type IV Collagen a3 chain (HLA-DRB1)
IF: LINEAR Ig
Lung; Alveolar hemorrhage
May present as
Proteinuria & Hematuria
Excretion >150mg to <3.5g/24hrs
3 RBCs
Insufficient for a diagnosis of nephrotic or nephritic
IgA Nephropathy (Berger Disease)
Episodic Recurrent Hematuria
w/o proteinuria
Post GI or Resp Infection
Assoc w/. celiac disease or Henoch-Schonlein purpura
Abnormal glycosylation of IgA and Activation of Alternate Complement pathway
Henoch-Schonelin Purpura
Reasambles IgA Nephropathy
Palpable purpura
children
Alport Syndrome (Hereditary Nephritis)
Hereditary glomerulonephritis related to
Mutation in collagen
Sensorineural hearing loss and vision problems
Ocular abnormalities (lens, retina, cornea)--> dependent on collagen
EM: GBM Thickening and thinning/splitting (
basketwave
appearance)
X-linked
Type IV collagen
Thin Basement Membrane Nephropathy (TBMN)-->
(Benign Familial Hematuria)
Associated with mutations in Collagen
Most common cause of persistent microhematuria
Normal Renal function
EM: Thinning of the GBM
Autosomal Dominant
May Present as
Nephritic