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Renal 11 Glomerneph.

Leonard: Glomerulonephropathies

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