Leonard: Glomerulonephropathies
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| Glomerulopathies General considerations | Altered structure and Fxn
Generally acquired
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| Nature of disease involvement Primary | Intrinsic kidney disease
Often immune-mediated
May be unknown etiology
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| Nature of disease involvement Secondary | Extrarenal disease involvement
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| Nature of disease involvement Hereditary | Rarely
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| Clinical features | Nephritic
Nephrotic
Hematuria
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| Pathogenesis | Mechanisms of antibody-mediated damage
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| Morphologic Alterations Altered cells of the glomerulus | Proliferation:
-Endothelial
-Epithelial
-Mesangial
Effactment of foot processes
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| Morphologic Alterations Altered GBM or ECM | Thickened GBM or ECM
Thinned GBM
Deposition of immune complexes
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| Morphologic Alterations Inflammatory cell infiltrates | Macrophages and leukocytes
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| General pathologic features of nephrotic syndrome | Typically normal glomerular cellularity
Absence of inflammation
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| Minimal Change Disease (MCD) Clinical | Most common form of nephrotic syndrome in kids (~90%)
~15% in adults
Tends to be fairly "albumin selective" proteinuria
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| Minimal Change Disease (MCD) Morphology | LM: normal
IF: negative
EM: effacement of podocytes
no immune deposits
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| Minimal Change Disease (MCD) Prognosis | Most experience remission with corticosteroid Tx
Very good responce in kids
Some relapse upon withdrawal of corticosteroids
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| Minimal Change Disease (MCD) Important points | Child
proteinuria >3.5
LM & IF negative
EM: effacement of podocytes
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| Membranous Glomerulopathy Clinical | Most common cause of nephrotic syndrome in adults
Whites and Asians
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| Membranous Glomerulopathy Etiology | Idiopathic
Autoimmune?
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| Membranous Glomerulopathy Morphology | LM: thickened capillary walls
Silver stain can show spikes
IF: granular staining for IgG
Complement along capillary loops
EM: subepithelial immune deposits
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| Membranous Glomerulopathy Prognosis | ~25% progress to ESRD
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| Membranous Glomerulopathy Important Points | Adult: white, asian
S/Sx of nephrotic syndrome
IF: confluent granular staining (IgG C3)
EM: subepithelial deposits
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| Focal Segmental Glomerulosclerosis (FSGS) | Scarring of portion of some glomeruli
Heterogeneous group of dz
Primary and secondary (HIV)
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| Focal Segmental Glomerulosclerosis (FSGS) Clinical | Insidious onset of asymptomatic proteinuria with frequent progression to nephrotic syndrome
Most common cause in African Americans
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| Focal Segmental Glomerulosclerosis (FSGS) Morphologic | LM:↑ ECM, hyalinosis
Maybe confused with MCD on biopsy
IF: trapping Ig and C3 in sclerotic regions
(no immune complexes)
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| Focal Segmental Glomerulosclerosis (FSGS) Morphologic (EM) | Diffuse effacement of podocytes
Loss of podocytes and collapse of capillaries with ↑ ECM
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| Focal Segmental Glomerulosclerosis (FSGS) Prognosis | Depends on underlying cause
Corticosteroids and ACEI typically beneficial
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| Focal Segmental Glomerulosclerosis (FSGS) Important points | African-American Adult
May progress rapidly to ESRD
LM: may be normal
Focal and segmental changes
EM: foot process effacement
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| Diabetic Glomerulosclerosis (DGS) | Associated with small vessel disease throughout the body
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| Diabetic Glomerulosclerosis (DGS) Clinical | Seen in ~50% diabetic pts
Microalbuminuria
Progress to proteinuria and nephrotic syndrome
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| Diabetic Glomerulosclerosis (DGS) Etiology | Generalized increase in BM material synthesis in the microvasculature
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| Diabetic Glomerulosclerosis (DGS) Morphology | LM:
Diffuse thickening of BM region and proliferation and expansion of mesangium
**Kimmelstiel-Wilson nodules** PAS
No immune complexes
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| Diabetic Glomerulosclerosis (DGS) Prognosis | ~30% develope ESRD
Leading cause in USA
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| Diabetic Glomerulosclerosis (DGS) Important points | Adult
Hyperglycemia (diabetic features)
LM: nodular sclerosis of glomeruli, arteriolar sclerosis
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| Amyloid Nephropathy Clinical | Renal involvement typical in AA and AL forms of system amyloidosis
Nonselective proteinuria
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| Amyloid Nephropathy Etiology | AA amyloid: associated with chronic inflammatory process
AL amyloid: associated with plasma cell neoplasm derived from light chains
(Multiple myeloma)
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| Amyloid Nephropathy Morphologic | LM: Congo red stain: apple green birefringence with polarized light
No immune complex deposits
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| Amyloid Nephropathy Prognosis | Unless underlying process is addressed, many lead to renal failure
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| Amyloid Nephropathy Important points | Adult
Evidence of systemic amyloiosis
Multiple Myeloma
Congo red: brick red
Apple green bifringence with polarized light
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| Nephritic Syndrome | Inflammatory disease
Associated with:
hematuria, azotamia, HTN, variable subnephrotic proteinuria & edema
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| Consequences of glomerular inflammation | ↑ GBM permeability= proteinuria
Microscopic defects= hematuria
(RBC casts/dysmorphic RBCs)
↓ GFR
↓ Na filtration
Edema
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| Most common causes of nephritic syndrome | Primary GN:
IgA nephritis
Secondary GN:
Post-streptococcal GN
Secondary GN:
Lupus nephritis
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| Acute Post-Infectious GN Clinical | One of most common pediatric
Dx based on rise in serum titers of Ab against strep
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| Acute Post-Infectious GN Etiology | Most often group a strep
Type III hypersensitivity rxn
2-4 weeks post-infxn
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| Acute Post-Infectious GN Morphology | LM: proliferative GN
IF: "lumpy-bumpy" IgG, C3 around capillaries & mesangium
EM: sub-epithelial humps
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| Acute Post-Infectious GN Prognosis | Majority improve to baseline within months
No need to intervene
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| Acute Post-Infectious GN Important points | Child
Hx of pharyngitis 2-4 wks prior
Hematuria, oliguria, HTN, edema, proteinuria, azotemia
LM: ↑ cellularity
EM: subepithelial humps
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| Membranoproliferative GN (MPGN) | Proliferation of glomerular cells
Alterations in GBM
Infiltration by WBCs
Primary (type I&II)
Secondary
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| Type I MPGN Clinical | Can occur at any age
Often in older children and younger adults
More prevalent in underdeveloped countries with chronic infxn
Low C3
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| Type I MPGN Etiology | Immune complexes in mesangium and subendothelial cap. walls
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| Type I MPGN Morphology | Mesangial hypercellularity
Glomerular crescents
Silver stain showing "tram tracking"
EM: subendothelial and mesangial dense deposits
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| Type I MPGN Prognosis | Tx of underlying disease
~50% progress to ESRD
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| Type II MPGN Clinical | Rare, more pronounced hypocomplementemia
Worse prognosis
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| Type II MPGN Etiology | Extensive complement in GBM
NOT immune complex
Most pts have IgG autoantibodies
(C3 nephritic factor)
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| Type II MPGN Morphology | LM: less pronounced hypercelularity
IF&EM: "ribbon-like" zone of increase density
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| Type II MPGN Prognosis | Lack of effective Tx
Some pts develop crescents and picture of rapodly progressive GN
~50% develop CKD in 10 yrs
High recurrance within transplanted kidney
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| Membranoproliferative GN Important Points | Adolescent/young adult
S/Sx of nephritic
Chronic infxn, low C3
LM: tram-tracking
IF: ribbon-like
EM: subendthelial/mesangial depots
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| Lupus Nephritis | Autoimmune
Nephritis is one of the more common features (70%)
Immune complexes in variety of places
Tx= immunosuppression
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| Lupus Nephritis Important Points | Woman/African-American
Nephritic syndrome
SLE
ANA+ w/anti-dsDNA ab's
LM: wire-loop thickening of GFB
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| IgA Nephropathy CLinical | Most common form of GN in the world
Young men
40% asymptomatic microhematuria
10% nephrotic syndrome
Slowly progressive course
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| IgA Nephropathy Etiology | IgA-dominant immune complexes
Symptoms initiated or exacerbation with respiratory or GI infxn
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| IgA Nephropathy Morphology | LM: diffuse mesangial prolideration
Crescent formation is rare
IF: mesangial staining for IgA
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| IgA Nephropathy Important Points | Young man
Hematuria, proteinuria, oliguria, azotemia
Hx of resp or GI infection
LM: mesangial prolif.
IF: IgA staining
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| Anti-GBM GN | Goodpasture's Disease
-associated with pumonary hemorrhage
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| Anti-GBM GN Clinical | Rare but aggressive
10-20% of RPGN
Serum anti-GBM abs
-Tx high dose immunosuppressants
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| Anti-GBM GN Etiology | 2-3 days after URIs
Autoantibodies against type IV collagen
Antigenetic epitope may be present in pulmonary alveolar capillary BM
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| Anti-GBM GN Morphology | LM:crescents
IF: diffuse linear staining of GBM and IgG
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| Rapidly Progressive GN (Crescentic GN) Clinical | No specific etiology
Severe injury with rapid and progressive renal dysfunction
Severe oliguria
Signs of nephritic syndrome
Ultimate fatality w/o intervention
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| Rapidly Progressive GN Morphology | Presence of cresents in most glomeruli
Proliferation of parietal epithelial cells
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| Rapidly Progressive GN Prognosis | Not good
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| Hereditary Nephridites | Present with hematuria
Two types:
Thin basement membrane dz
Alport syndrome
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| Hereditary Nephridites Thin basement membrane disease | Most common of the hereditary
Asymptomatic
Mutations coding Type IV collagen
EM: uniform thinning of GBM
Generally benign
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| Hereditary Nephridites Alport syndrome Clinical | Hematuria with progression to CRF
Nerve deafness, eye disorders
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| Hereditary Nephridites Alport syndrome Etiology | Majority are X-linked
-Females limited to benign hematuria
-Defective assembly of Type IV collagen
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| Hereditary Nephridites Alport syndrome Morphology | EM: alternating thinning and thickening of GBM
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| Hereditary Nephridites Thin BM disease Important Points | Young person
Asymptomatic hematuria
EM: Uniform thinning of GBM
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| Hereditary Nephridites Alport syndrome Important Points | Male:
Hematuria with pregression to renal impairment
Nerve deafness, cataracts
Fracturing of GBM
Female: Hematuria only
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| Chronic Glomerulosclerosis (CGN) Clinical | Progressive RF
Oliguria
Uremia
Anemia
HTN with cardiac and CNS effects
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| Chronic Glomerulosclerosis (CGN) Etiologies | Those who survive acute phase of RPGN develop CGN and CRF
DM (30%)
HTN
Primary glomerular dz
Systemic autoimmine dz
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| Chronic glomerulosclerosis (CGN) Morphologies | Shrunken kidney with diffuse granular cortical surface
Cortex thinned
LM: arteriolar sclerosis, tubular atrophy, renal osteodystrophy
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