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KIDNEY DISEASES Developmental disorders Glomerular diseases Tubulo-interstitial diseases Urinary stones Obstructive uropathy Tumors. LUPUS GLOMERULONEPHRITIS GN-Common feature of SLE Immune complex-deposition -in situ formation Associated with deposits in -blood vessels

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slide1

KIDNEY DISEASES

Developmental disorders

Glomerular diseases

Tubulo-interstitial diseases

Urinary stones

Obstructive uropathy

Tumors

slide2

LUPUS GLOMERULONEPHRITIS

GN-Common feature of SLE

Immune complex-deposition

-in situ formation

Associated with deposits in

-blood vessels

-tubular BM

-interstitium

Complementinflammation

slide3

LUPUS GLOMERULONEPHRITIS

I: No lesion

II: Mesangial

III: Focal proliferative

IV: Diffuse proliferative

V: Membranous

VI: Chronic

slide4

PATHOLOGY OF SLE GN

  • Highly variable
  • Immune complexes vary in/size/location
          • Subendothelial
          • Intramembranous
          • Subepithelial
          • Mesangial
  • Proliferation of Mes, End, Ep cells; PMN and MACROPHAGES
  • Fibrinoid necrosis
  • IF-”full house” (ML Ig)
slide5

KIDNEY ANATOMIC COMPARTMENTS

Glomerulus

Tubules

Blood vessels

Interstitium

Collecting system

(Callices & Pelvis)

slide6

COMPONENTS OF THE GLOMERULUS

  • Capillary basement membrane
  • Mesangium
  • Bowman capsule
  • Cells
  • Endothelial
  • Epithelial
  • Mesangial
slide7

THE JUXTAGLOMERULAR APPARATUS

Afferent arteriole

Macula densa – ascending loop of Henle

Lacis cells - Renin

slide8

CONGENITAL RENAL ANOMALIES

Agenesis – Potter syndrome

Ectopia

Fusion

Dysplasia

Polycystic kidney disease

slide9

POLYCYSTIC KIDNEY DISEASE

Autosomal dominant (adult) (1:1,000)

Autosomal recessive (infantile (1:30,000)

Medullary cystic disease complex (1:10,000)

Medullary sponge kidney

Acquired cystic renal disease

slide10

AUTOSOMAL DOMINANT

POLYCYSTIC KIDNEY DISEASE

Common kidney disease (1:1,000)

10% of all transplant/dialysis patients

ADPKD-1 gene (polycystin) mutation 85%

Bilaterally enlarged kidneys (>3,000g)

Symptoms appear in adult life

Renal failure 5-10 years thereafter

slide11

ADPKD

Associated Conditions

Liver cysts (30%)

Splenic cysts (10%)

Pancreatic cysts (5%)

Cerebral aneurysms (20%)

Diverticulosis coli

slide12

GLOMERULAR DISEASES

Asymptomatic hematuria/proteinuria

Nephrotic syndrome

Nephritic syndrome

Rapidly progressive glomerulonephritis

Chronic nephritis

slide13

KIDNEY DISEASES

(MNEMONIC “ANNURIC”)

A Asymptomatic hematuria/proteinuria

N Nephrotic syndrome

N Nephritic syndrome

U Urolithiasis

R Rapidly progressive glomerulonephritis

I Interstitial and tubular diseases

C Chronic renal disease

slide14

CHRONIC RENAL FAILURE

(UREMIA)

General symptoms – weakness, fatigue

Cardiovascular – hypertension, pericarditis

G.I. – nausea, vomiting, diarrhea

CNS – lethargy, confusion, coma

Muscles – twitching, weakness

Bones – osteodystrophy

Metabolic – acidosis, PK, BUN, Cr.

Endocrine - parathyroids

slide15

NEPHROTIC SYNDROME

Proteinuria (“nephrotic range” >3.5g/24h)

Hypoalbumimenia

Edema

Hyperlipidemia

Lipiduria

slide16

CAUSES OF NEPHROTIC SYNDROME

DiseaseChildren(%) Adults(%)

Minimal change GN 75 20

Membanous GN 5 40

MPGN I 5 5

Other GN 5 20

slide17

NEPHRITIC SYNDROME

Hematuria

Proteinuria

Hypoalbuminemia

Oliguria (GFR, Cr, BUN)

Edema (salt and water retention)

Hypertension

slide18

DIAGNOSTIC FEATURES OF GLOMERULAR DISEASES

  • Light microscopy
    • Cellularity
    • Extracellular matrix
  • Special stains
  • (PAS, amyloid)
  • Immunofluorescence microscopy
  • (Linear, granular, mesangial, irregular deposits)
  • Electron microscopy
  • (deposits of immune complexes, BM changes, amyloid)
slide19

MINIMAL CHANGE GLOMERULOPATHY

Most common cause of nephrotic syndrome in children

E/P: unknown (?immune)

Path: normal by LM and IF

EM: fusion of foot processes

CL: Nephrotic sy – responds to steroids

Dd: FGS

slide20

FOCAL SEGMENTAL GLOMERULOSCLEROSIS

Def: 15% of all nephrotic syndromes; heterogenous group of diseases

E/P: unknown-circulatory glomerular injury (?)

HIV, I-V drug abuse

CHD, obesity, sickle-cell disease

Path: focal and segmental glomerular hyalinosis

“Collapsing” pattern (e.g. HIV)

Trapping of serum proteins (IF and EM)

Clin: Nephrotic syndromeESKD (5-20y)

If HIV relatedESKD (1 year)

slide21

MEMBRANOUS NEPHROPATHY

Def: Most common cause of nephrotic syndrome in adults (40%)

Immune complexBM thickening

E/P: Primary (unknown)

Secondary (SLE, HBV, drugs, cancer)

Path: Subepithelial deposits of immune complex

CL: Nephrotic syndrome

(25% recover, 50% persist, 25% progress)

slide22

DIABETIC GLOMERULOSCLEROSIS

Def: Diabetes caused BM thickening

Proteinuria renal failure (leading cause in US)

E/P: BM synthesis , nonenzymatic glycation (?)

Path: Diffuse global thickening of BM

Nodular sclerosis (K-W)

Arteriolosclerosis

Trapping of serum proteins

Clin: Proteinuria (in 50% diabetics)

ESKD (30% all in the US)

slide23

DIABETIC KIDNEY DISEASES

Glomerulosclerosis

Arteriolosclerosis  Hypertension

Pyelonephritis

Papillary necrosis

slide24

AMYLOIDOSIS

AA amyloid – systemic disease

AL amyloid – multiple myeloma

Deposits of amyloid – glomeruli (mesangial)

- arterioles

- tulular BM

Nonselective proteinuria – nephrotic sydrome (60%)

renal failure (with large kidneys!)

slide25

ACUTE POSTINFECTIOUS GN

Def: Acute nephritic syndrome 1-2 weeks after infection

E/P: immune response to A -hemolytic streptococci

(other infections Staph, malaria, HBV less common)

Path: Acute glomerulonephritis

Clin: Childhood nephritic syndrome

90% recover

9% presistent hematuria/proteirunria

1% chronic renal disease

slide26

PATHOLOGY OF ACUTE POSTINFECTIOUS GN

LM: hypercellularity (endo + MES + PMN)

IF: Irregular deposits of Ig, Compl.

EM: Deposits (mesang + BM)

“HUMPS”

slide27

MEMBRANOPROLIFERATIVE GLUMERULONEPHRITIS

  • Type I
  • Def: Immune mediated presistent GN
  • Mesangiocapillary cell proliferation and BM reduplication
  • E/P: Immune comples deposition in glomeruli
    • Primary (unknown cause)
    • Secondary (SBE, chronic infection, HCV, cryoglobulinemia, cancer)
  • Path: Lobular gromerulonephritis
  • Clin: Older children/young adults
  • Nephritic or nephrotic syndrome
  • Progressive – 50%  ESKD (10 years)
slide28

MEMBRONOPROLIFERATIVE GLOMERULONEPHRITIS

Type II

Def: Immune mediated persistent GN

Dense deposits in GBM

E/P: C3 nephritic factor (IgG)

Stablizes the activated C3 convertase

Path: Similar by LM to MPGN-I

EM-shows linear dense deposits in GBM

slide29

RENAL BIOPSY IN SLE

To determine

1. Category (class)

2. Activity

3. Extent

4. Chronicity

Note: Class IV-worst prognosis

Class V-no response to steroids

slide30

IgA NEPHROPATHY (BERGER)

Most common form of GN

Young adults (15-30 years)

Pathology: IgA deposits in mesangium

-varied severity

Clin: Protean manifestations

40% asymptomatic microscopic hematuria

40% bouts of macro hematuria

10% nephrotic syndrome

10% renal failure

slide31

ANTI-GBM ANTIBODY GN

RPGN mediated by antibody

Antibody to collagen IV

Linear IF

Fibrinoid necrosis of GBM

Crescentic GN

Goodpasture syndrome

slide32

CRESCENTIC GLOMERULONEPHRITIS

  • Anti-GBM glomerulonephritis
  • (inc. Goodpasture syndrome)
  • 2. ANCA granulonephritis
  • (inc. Wegener granulomatosis, micr. polyangitis, Churg-Strauss syndrome)
  • 3. Immune complex glomerulonephritis
  • (inc. Henoch-Schonlein purpura, cryoglobulinemia)
  • 4. Non-immune
slide33

ANCA GLOMERULONEPHRITIS

Necrotizing and crescentic

ANCA (PMN myeloperoxidase, proteinase 3)

No immune deposits in glomeruli

Respond to immunosuppresion (25% pregress)

Associated with small vessel polyangiitis (75%)

Pulmonary-renal vasculitic syndromes

slide34

SMALL VESSEL VASCULITIS

AFFECTING KIDNEYS

Henoch-Schonlein purpura-children, IgA

Cryoglobulinemic vasculitis-HCV, MPGN-I

Wegener GR.-upper slower respiratory tract

Churg-Strauss-eosinophilia, asthma

Microscopic polyangiitis-multiple organs, lungs (-)

slide35

NEPHROSCLEROSIS

Benign – sustained mild hypertension

– hyaline arteriolosclerosis

– arterial fibrosis

– glomerular hyalinization, tubular atrophy

Malignant-BP>125 mm/Hg, retinal hemorrhage, papilledema, renal dysfunction

– fibrinoid necrosis of arterioles

– myxoid intimal expansion of arteries

– microthrombi

slide36

THROMBOTIC MICROANGIOPATHY

Morphologic finding in several diseases

Assoc: microangiopathic hemolytic anemia

E/P: HUS

TTP

Malignant nephrosclerosis

Systemic sclerosis

slide37

RENAL INFARCTS

  • Thromboemboli
          • Mural thrombi
      • (M.I., atrial fibrillation)
          • Endocarditis
          • Aortic thrombi (atherosclerosis)
  • Cholesterol emboli
slide38

RENAL CORTICAL NECROSIS

  • Cortical ischemia: thrombi, vasospasm
  • Complication of shock
          • Abruptio placentae
          • Endotoxic shock
          • Hypotensive shock
  • Cortex pale – medulla spared
  • Massive tubular necrosis
slide39

CAUSES OF ACUTE TUBULAR NECROSIS

  • Ischemia (hypoperfusion)
    • Hypovolemic shock (e.g. bleeding)
    • Sepsis
    • Burns
    • Prolonged surgical operations
  • Nephrotoxins
    • Toxic chemicals (e.g. CCl4)
    • Heavy metals (e.g. Hg)
    • Drugs (e.g. cisplatin)
  • Heme proteins
    • Myoglobin (e.g. rhabdomyolysis)
    • Hemoglobin (e.g. hemolysis)
slide40

PATHOLOGY OF ACUTE TUBULAR NECROSIS

Cortex> medulla

Necrotic tubular cells slough off

Intratubular cast in medulla

Regeneration of tubules occurs fast

slide41

CLINICAL ASPECTS OF ATN

  • The most common cause of acute renal failure
  • “Dirty” brown casts in urine
  • Oliguria anuria polyuria
    • Azotemia
    • Acidosis, K
    • Fluid retention
  • Recovery 1-2 weeks
slide42

ACUTE PYELONEPHRITIS

Bacterial infection (E. coli 80%)

Ascending > hematogenous

Lower UTI precedes renal infection

Fever, flank pain, neutrophilia

Leukocyte casts in urine

Healing - recurrence chronic pyelonephritis

slide43

PATHOLOGY OF ACUTE PYELONEPHRITIS

Unilateral> bilateral

Focal

PMNs in tubules interstitium

Abscesses with tissue destruction

slide44

PYELONEPHRITIS

PREDISPOSING CONDITIONS

Urinary stones

Hydronephrosis

Cystitis

Prostatic hyperplasia

Tumors

Pregnancy

Vesico-ureteric reflux

External ureteric compression (e.g. fibrosis)

slide45

PREDISPOSITION TO PYELONEPHRITIS

(MNEMONIC URINE)

U Urolithiasis

R Reflux (vesico-ureteric)

I Infections of lower UT

N Neoplasms (e.g. ureteric, vesical, prostatic)

E External compression (e.g.) pregnancy

retroperitoneal fibrosis

slide46

CHRONIC PYELONEPHRITIS

  • PATHOLOGY
  • Destruction of renal tissue and fibrosis
    • Cortical scars
    • Loss of papillae
    • Ectasia of calices
    • Hydronephrosis
  • Irregularly shrunken small kidney
  • Histology: Chronic inflammatory infiltrates
          • Tubular atrophy with casts
        • (“thyroidization”)
slide47

DRUG INDUCED RENAL DISEASES

  • Acute tubular necrosis(toxic)
  • Acute tubulointerstitial nephritis (allergic)
  • Analgesic nepropathy (phenacetin dose-related)
slide48

METABOLIC TUBULAR INJURY

Multiple myeloma – light chain casts

Hyperuricemia (gout) – urate nephropathy

Hypercalcemia – uric acid stones

Hypercalciuria – nephrocalcinosis

– calcium stones

slide49

CAUSES OF HYPERCALCEMIA – HYPERCALCIURIA

  • Primary (increased intestinal absorption of Ca)
    • Idiopathic (most common)
    • Milk-alkali syndrome
    • Vitamin D excess
    • Sarcoidosis
  • Secondary (release of Ca from bones)
    • Renal osteodystrophy
    • Hyperparathyroidism
    • Osteolytic metastases (e.g. breast cancer)
    • Paraneoplastic syndromes (PTrP)
slide50

RENAL STONES (NEPHRODITHIASIS)

  • Calcium oxalate or phosphate (75%)
  • Uric acid (15%)
  • Struvite (magnesium ammonium phosphate)
  • and calcium phosphate (8%)
  • 4. Cystine (1%)
slide51

RENAL TUMORS

Benign – oncocytoma, angiomyolipoma, fibroma (rare!)

Renal cell carcinoma (most common – adults)

Wilms tumor (childhood)

Transitional cell carcinoma of renal pelvis

slide52

RENAL CELL CARCINOMA

Most common renal tumor

Peak age – 60y M:F = 2:1

Incidence increasing world wide

Risk factors: Tobacco; genetics (vHL-gene, familial cases)

slide53

RENAL CELL CARCINOMA PATHOLOGY

Yellow orange on cut surface

Partially encapsulated

Extends into renal vein

Histology: tubular clear cell (77%)

papillary (15%)

granular, chromophobe, sarcomatoid (5%)

slide54

RENAL CELL CARCINOMA

CLINICAL ASPECTS

Classical triad (hematuria, flank pain, mass) (<10%)

Hematuria (50%) most common symptom

Metastases-hematogenous and local abdominal

Paraneoplastic syndromes (PTH, Epo, amyloid)

5 year survival = 40%

slide55

WILMS TUMOR

Childhood tumor (peak age 2 years) 98%< 10 years

Most common abdominal solid tumor in childhood

Sporadic (90%)

Familial syndromic (5%)

(WAGR syndrome – WT1 tumor suppressor gene Beckwith – Wiedemann syndrome – WT2)

Familial nonsyndromic (5%)

Unilateral (90%)

Bilateral more common in familial cases (20%)

slide56

WILMS TUMOR

CLINICAL – PATHOLOGIC FEATURES

Lobulated tumors mass – usually encapsulated

Histology: mixture of immature cells metanephic, stromal, tubular

Chemotherapy + surgery = 5 years = 90%

Children< 2 years better prognosis