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Renal 11 Glomerneph.
Leonard: Glomerulonephropathies
| Question | Answer |
|---|---|
| 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
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