Week 4: Pathology of tubulointerstitial and Cystic renal diseases Flashcards Preview

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Flashcards in Week 4: Pathology of tubulointerstitial and Cystic renal diseases Deck (27):
1

Define tubulointerstitial disease. Two principal causes?

-Definition: group of disorders characterized by abnormalities of renal tubules and the interstitial. Can spare the glomueruli.
1. inflammatory mediated injury
-infectious
-non infectious: interstitial nephritis
2. Ischemic or toxic injury
-acute tubular necrosis

2

Define pyelonephritis. Etiology? Pathogenesis?

-Definition: an acute suppurative bacterial infection of the kidney. Associated with UTIs.
ETIOLOGY
-bacterial infections caused by colonization of distal urethra and introits by coliform bacteria with adhesions on pili or upward spread by instruments
-most common: E.coli, other gram negative rods from fecal flora-Proteus, Klebsiella, Enterobacter, Strep faecalia, Pseudomonas aeruginosa
PATHOGENESIS
-most common mechanism: ascending infection from bladder
-promoted by obstruction, vesicoureteral reflux, or other reason for urinary stasis
-spread by blood less common

3

Predisposing conditions for pyelonephritis.

-urinary tract obstruction
-instrumentation, e.g. catheters
-vesicoureteral reflex
-pregnancy
-females between 1-40, in males, incidence rises with age
-diabetes mellitus-due to impaired leukocyte fxn, frequent micturition (fainting during or after urination), nephrogenic bladder
-immunosuppression and immunodeficiency

4

Pathology of pyelonephritis

-hallmark: patchy interstitial suppurative inflammation and focal tubular necrosis
-gross: Large kidney, bulging cut surface, yellow streaks of pus, inflamed calyces and pelvis
-micro: PMNs in interstitium and tubules with focal micro-abscesses

5

Clinical course of pyelonephritis.

-acute onset of chills, fever, dysuria, frequent and urgency with costovertebral angle tenderness and pain
-lab findings: pyruia, WBC casts, pathogenic organisms 10^3 to 10^5 organisms/mL

6

Complications of acute pyelonephritis

-septicemia and gram negative shock
-preterm labor
-papillary necrosis: mainly with DM
-pyonephrosis-pus fills in collecting systems of obstructed hydronephrotic kidney
-perinephric abscess: extension of the inflammatory process outside the renal capsule

7

Define chronic pyelonephritis.

-chronic tubulointerstitial inflammation and renal scarring with involvement of the calyces and pelvis
-other factors involved in pathogenesis in addition to bacterial infection (e.g. reflux and obstruction)
-chronic tubulointerstitial inflammation with scarring of the renal parenchyma and distortion of the renal calyces and pelvice

8

Etiology of chronic pyelonephritis

1. chronic obstruction: acute renal infection and inflammation superimposed upon diffuse or localized obstructive lesions-->parenchymal atrophy and extensive scarring
2. Reflux nephropathy: superimposition of urinary infection on congenital vesicoureteral reflex-->scarring

9

Pathology of chronic pyelonephritis

-gross: irregular discrete corticomedullary scars (pitted), overlaying dilated blunted calyx (fibrosis and shrunken parenchyma)
-micro: patchy atrophic tubules and hypertrophic surviving tubules, dilated with colloid casts (thyroidization), chronic interstitial inflammation, fibrosis

10

Clinical course of chronic pyelonephritis.

-insidious course, not recognized until onset of renal failure with lassitude, headaches, weight loss, nausea, polyuria, nocturne. HTN and its complications
-Lab findings: bacteriruia >10^5, low grade pyruria, proteinuria, elevated BUN and Cr (evidence of renal failure)

11

Define xanthogranulomatous pyelonephritis

-distant variant of chronic pyelonephritis
-foamy macrophages mixed with plasma cells, lymphocytes, neutrophils, giant cells
-associated with stag horn calculus with superimposed proteus infection
-yellow orange nodules mistaken for renal carcinoma

12

Causes of drug induced nephropathy (non infectious interstitial nephritis)

--delayed hypersensitivity reaction due to happen like effect. Onset ~2 weeks after exposure to drug
-penicillins and rifampin
-thiazides and cimetidine

13

Pathology of drug induced nephropathy

-interstitial edema with lots of eosinophils and neutrophils.
-may be granulomas (methicillin).
-Tubular necrosis and regeneration are present and glomeruli are normal

14

Clinical course of drug induced nephropathy

-15 days after exposure, prevents with rash, fever, eosinophilia, hematuria, mild proteinuria
-50% have Cr elevation
-acute renal failure may develop

15

Describe radiation nephritis.

-6-12 months after radiation
-micro: glomeruli hyalinization, severe interstitial fibrosis and tubular atrophy-->HTN, anemia, and proteinuria

16

Define acute tubular necrosis.

-characterized by destruction of tubular epithelial cells and clinically by suppression of renal function
-most common cause of acute renal failure
-when urine falls below 400mL in 24 hrs
-reversible
-necrosis from ischemia or toxicity

17

Pathogenesis of ischemic ATN

-medulla is susceptible to ischemia because it receives small portion of blood fluid
-ischemi-->vasoconstriction-->reduced GFR and oliguria
-clinical complication after shock produced by severe bacterial infection, large burns, mismatched blood transfusion reaction, massive crushing injuries

18

Pathology of ischemic ATN

-focal tubular necrosis at multiple irregular points
-rupture of BM sometimes
-hyaline and granular casts of Tamm-Horsfall protein (glycoprotein normally secreted by TALH in normal urine)
-pigment casts
-glomeruli spared

19

Etiology of nephrotoxic ATN

-sensitive to toxic injury due to vast electrically charged surface for reabsorption, active transport for ions and organic acids, ability to concentrate urine
-ethylene glycol
-drugs: amphotericin B, gentamicin
-metals
-radiocontrast media

20

Pathology of nephrotoxic ATN

-more diffuse with toxic compared to segmental with ischemic
-greatest injury usually in PT

21

Pathology of ADPKD

-gross: bilateral, large, polycystic kidneys
-renal cysts vary in size
-often contain colorless fluid/turbid hemorrhagic or gelatinous fluid
-micro: variably sized cysts lined by simple epithelium with normally differentiated intervening parenchyma

22

Etiology of ARPKD (autosomal recessive polycystic kidney disease or infantile polycystic kidney disease). Prognosis?

-mutation on chromosome 6p (PKHD1). gene encodes fibrocystin, function unknown
-failure to communicate between nephron and pelvicalyceal system
-babies usually don't survive long and older children have associated hepatic fibrosis

23

Pathology of ARPKD

-gross: bilaterally enlarged, multi cystic kidneys with normal contour. Uniform sized cysts
-micro: cysts lined by uniform cuboidal cells, rare glomeruli but no normal parenchyma

24

Describe medullary sponge kidney

-congenital disorder with unknown pathogenesis
-renal condition consisting of multiple cystic dilations of the medullary collecting ducts
-usually asymptomatic unless presenting with secondary complication, usually in 40s-60s.
-the dilations give sponge like appearance to renal medulla

25

Describe uremic medullary cystic disease. Pathology?

-usually originates in childhood but found in adolescents and adults
-progressive renal failure and cyst formation in medullary and cortico-medullary regions of the kidney
-gross: small contracted kidneys, granular surfaces, cysts are present near corticomedullary junction
-micro: cotical tubular atrophy with interstitial fibrosis, cysts lined by flattened or cuboidal cells and border by inflammation or fibrosis

26

Clinical features of uremic medullary cystic disease

-polyuria, polydipsia -due to defect in concentration ability
-sodium wasting
-tubular acidosis also prominent
-progression terminal renal failure in 5-10 years
-assoc. with hepatic fibrosis, retinal dysplasia, hyperuricemia, and gouty arthritis

27

Describe dialysis cystic disease. Etiology, pathology, clinical course

-definition: cortical or medullary cysts occurring in patients with end-stage renal disease who have undergone prolonged dialysis
-etiology: may be result of fibrosis than dialysis
-gross: cysts containing clear fluid and some times calcium oxalate crystals
-micrl: cysts lined by flattened epithelium, but foci of papillary hyperplasia of the tubular lumen are common
-increased incidence of renal adenoma and renal cell carcinoma arising within the wall of cysts