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32 Cards in this Set
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Basic Histopathological Changes of Glomerulonephritis
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Cellular Proliferation
Leukocytic Infiltration "Membranous" Change Hyalinization Thrombosis, Necrosis, Deposition of Fibrin |
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Minimal Change Disease
(lipoid nephrosis) |
NEPHROTIC
Loss of charge barrier --> leakage of protein No hematuria, NORMAL filtration function Occurs more often in children than in adults Histology is NORMAL Effacement (FUSION) of podocytes Almost all patients respond to steroids (kids > adults) Steroid-resistant respond to cyclophosphamide Associated with Hodgkin's disease and NSAID use Etiology is unknown |
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Focal Segmental Glomerular Sclerosis
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NEPHROTIC
Some may have microscopic hematuria Loss of renal function (ALL eventually require transplant) Deep, juxtmedullary glomeruli are affected first POOR or NO RESPONSE TO STEROIDS IF may show IgM and C3 trapped in the mesangium Epithelial cell vacuolization, Variable podocyte fusion Focal sclerosis is also associated with urinary reflux, and IV drug abuse Etiology is unknown Effects blacks > whites |
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Membranous Nephropathy
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NEPHROTIC (most common cause in adults)
Etiology is IN SITU IC (IgG) DEPOSITION (Sub-epithelial) Primary -- most common Secondary -- assoc. w/ cancer, drugs, HEP B, SLE, infections Adults of any age (peak 4th-5th decade); RARE in kids Female > Male Histology -- diffuse CAPILLARY WALL THICKENING Silver stain - spikes of GBM between deposits IF makes IgG (C3) deposits look linear, BUT THEY'RE NOT Responds to withdrawal of treatment of infection that caused the disease Stage I and 50% of Stage II respond to steroids |
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Different stages of Membranous Nephropathy
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Stage I -- GBM has normal thickness
Stage II -- GBM spikes between deposits Stage III -- Intramembranous dposits Stage IV -- Deposits disappearing (losing electron density) Stages II, III, IV show GBM thickening |
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Diabetic Glomerulosclerosis
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NEPHROTIC
Hyperglycemina appears to be the major cause Non-enzymatic glycosylation: LYSINE residues Cross-linking of matrix and GBM proteins Earliest manifestation is MICROALBUMINURIA ESRD in 30% of juvenile diabetics Two types: Diffuse glomerulosclerosis Nodular glomerulosclerosis (Kimmelstiel-Wilson) |
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Kimmelstiel-Wilson Disease
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NEPHROTIC
Found in pts. with microaneurysms of the retina (bad prognosis) ACELLULAR NODULES IN THE MESANGIUM PAS and Silver stain POSITIVE IF shows LINEAR highlighting of IgG (trapped) |
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Amyloidosis
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NEPHROTIC
Mesangial deposition of EC proteins; CONGO RED positive AL AMYLOID -- secondary to MONOCLONAL GAMMOPATHY (light chain deposition [LAMBDA]) AA AMYLOID -- assoc. w/ inflammatory diseases (RA, infections, Tb, osteomyo, Hodgkin's) Green bi-refringence when stained (under polarized light) HYPOcellular glomeruli ESRD is inevitable |
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Acute Proliferative GN
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NEPHRITIC
Pts. (usually kids) present after Strep infection (TYPES 12, 4, 1) Hematuria, Oliguria, EDEMA, Hypertension Believed to be IC mediated (kidneys are STERILE) C3 levels are initially low, ASO titer is elevated HYPERcellular glomeruli EM shows electron dense deposits (humps) Overall cure rate is 75% (kids ~100%; adults not so good) Slow progression to chronic GN |
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Rapidly Progressive GN Types
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RPGN with anti-GBM Ab
RPGN with Immune Complexes "Pauci-Immune" RPGN |
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Rapidly Progressive GN
(A.K.A. Crescentic GN) |
Associated with acute renal failure
Diagnosis based on finding of epithelial crescent FIBRIN is often deposited in Bowman's space Therapy is with CYCLOPHOSPHAMIDE |
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RPGN with anti-GBM Ab
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Less than 5% of RPGN
Serum anti-GBM Ab (negative test does NOT rule out) IF shows LINEAR deposits of IgG NO electron dense deposits Treatment is supportive |
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RPGN with Immune Complexes
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May be a sequel to post-infectious GN
Other forms of IC GN cal also present as this, with crescents |
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"Pauci-Immune" RPGN
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NO GBM Abs or ICs
Can occur as GN OR as changes in vasculitis (Ex. microangiitis, Wegener's) Pts. frequently have POSITIVE ANCA |
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P-ANCA Ab
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Peri-nuclear staining
In patients with Pauci-immune GN (with or without vasculitis) |
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C-ANCA Ab
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Cytoplasmic staining
In patients with Wegener's Patients alo have sinusitis, often with nose bleed/hemoptysis |
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Membranoproliferative GN
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Nephritic OR Nephrotic
Both kids and adults Selective depletion in C3, 5, 6, 7 Some pts. have C3 NeF -- IgG AUTO-Ab to C3b INTERPOSITION of mesangial cells --> splitting of the GBM |
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IgA Nephropathy
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NEPHRITIC ("HEMATURIA is the rule")
Histo -- mesangial enlargement IF shows abundant IgA in the mesangium Prognosis is usually good No specific treatment - control hypertension |
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Henoch-Schoenlein Allergic Purpura
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Children and young adults are affected
Focal GN (presents as hematuria) PURPURA; ARTHRITIS; ACUTE ABDOMINAL PAIN Prognosis is fair to good |
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Cryoglubulinemic GN
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NEPHRITIC
Proteins that deposit are cryoglobulins Most pts. hematuria, proteinuria, HYPOcomplementemia Two broad groups: Those associated with ICs Those associated with monoclonal gammopathy |
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Essential Mixed Cryoglobulinemic GN
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Most common form
Composed of monoclonal IgM MAJOR ASSOCIATION WITH HEP C Palpable purpura on lower extremities Leg ulcers, arthralgias, hepatosplenomegaly |
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Monoclonal Cryoglobulinemic GN
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Assoc. w/ underlying LYMPHOPROLIFERATIVE disorders
IF shows glomerular deposit only having one of the light chains (lambda) Histology is like MPGN |
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Thin GBM Disease
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Patients are usually women
Hematuria only Etiology is unknown Segments of the GBM are abnormally thin, all else is normal NO treatment necessary All patients do well |
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GN of SLE
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Nephritic or Nephrotic
Almost 100% of pts. have anti-nuclear Abs Ag-Ab complex disease Serum Complement C3 is depressed Different classifications resemble different GNs |
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Mesangial Lupus Nephritis
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Resembles IgA Nephropathy
Presents with hematuria Usually respond well to steroid therapy (good prognosis) NOTE: can progress to more serious forms of lupus GN |
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Membrane Lupus GN
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Resembles Membranous GN
Severe Proteinuria |
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Diffuse Membranoproliferative Lupus GN
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Known as Type IV (very serious)
BOTH hematuria and proteinuria, freq. assoc. w/ ARF IV CYCLOPHOSPHAMIDE is given in a bolus Electron dense deposits are adjacent to GBM Some SLE complexes have a finger print pattern on them |
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Wire Loop
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Significant thickening of the BM in capillary loops
Associated with Lupus GN type IV |
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Hematoxylin Body
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Round, glass-like basophilic deposit adjacent to necrosis
Represent naked cell nuclei that have reacted with ANA |
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Nephrotic GNs
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Minimal Change
FSGN Membranous Diabetic Amyloidosis MPGN (can be nephritic also) SLE (can be nephritic also) |
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Nephritic GNs
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Acute Proliferative
IgA Nephropathy Cryoglobulinemic Henoch-Schonlein Allergic Purpura Vasculitis MPGN (can be nephrotic also) SLE (can be nephrotic also) |
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Renal Diseases assoc. w/ ARF
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RPGN
ATN Interstitial Nephritis |