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Diseases of urinary system Zhang Hongying How to learn systemic pathology (pathology of urinary system) ? basic science (anatomy, histology, physiology, immunology) general pathology systemic pathology clinic practice *correlations between structural and functional changes (clinicopathologic correlations) Functions of the kidneys nEliminating metabolic waste products nRegulating fluid and electrolyte balance nInfluencing acid-base balance nSecreting hormones (prostaglandins, erythropoietin, 1,25- dihydroxycholecalciferol, renin) The nephron The main functional unit of the kidney Normal glomerulus Outline nGlomerulonephritis nTubulointerstitial nephritis nDiseases of the lower urinary tract nTumors of urinary system Etiology of glomerular diseases nPrimary(majority):disease process appears to start within the glomerulus nSecondary:disease process is secondary to systemic disease (SLE, vascular diseases, diabetes mellitus) nHereditary:congenital diseases ( Fabrys disease) Primary glomerulonephritis nkidney is the only or predominant organ Mechanism of glomerular injury nCirculating immune complex nephritis nimmune complex nephritis in situ nCell-mediated immunity nOther mechanisms Granular pattern Immunofluorescence microscopy Linear pattern Immunofluorescence microscopy Mechanism of glomerular injury nCirculating immune complex nephritis nimmune complex nephritis in situ nCell-mediated immunity nOther mechanisms Steps in diagnoses of glomerular lesions nClinical presentation nRenal biopsy Clinical manifestations nHematuria (red blood cells in urine) n Proteinuria (protein lost in urine) nOliguria or anuria (no urine flow) nEdema nHypertension nUrinary casts (cell casts, granular casts, hyaline casts) nAzotemia (waste products accumulation) nUremia (with autotoxication symptom) Clinical presentations nAcute nephritic syndrome: acute onset, blood pressure ，hematuria, proteinuria, edema, azotemia nRapidly progressive nephritic syndrome: abrupt or insidious onset of hematuria, proteinuria, anemia and rapidly progressing renal failure Glomerulonephritis Clinical presentations nNephrotic syndrome: heavy proteinuria, hypoalbuminemia, sever edema, hyperlipidemia and lipiduria nChronic nephritic syndrome: slowly developing renal failure accompanied by proteinuria, hematuria hypertension and uremia Key facts renal failure nTwo renal syndromes of total renal failure ( acute and chronic renal failure) nTwo main syndromes of partial renal failure (nephritic syndrome and nephrotic syndrome) nChronic renal failure is irreversible, as it is caused by permanent destruction of nephrons nAcute renal failure sometimes recovers when the damaging stimulus resolves The renal biopsy nHistological examination nImmunofluorescence examination nElectron microscopic examination The renal biopsy nHistological examination nImmunofluorescence examination nElectron microscopic examination Primary glomerulonephritis nAcute diffuse proliferative glomerulonephritis nCrescentic glomerulonephritis nMembranous glomerulonephritis nLipoid nephrosis nFocal segmental glomerulosclerosis nMembranoproliferative glomerulonephritis nIgA nephropathy nChronic glomerulonephritis Diffuse proliferative glomerulonephritis nDiffuse, global, acute inflammation of glomeruli is caused by the deposition of immune complexes in glomeruli, stimulated by a preceding infection nChildren are more commonly affected Etiology nPoststreptococcal (most common): onset is 1 -2 weeks after a primary infection with - hemolytic streptococci of group A nNon-streptococcal(less common): a range of bacterial, viral and protozoal infections Morphology Gross examination Swollen with scattered petechia Histologic features nEndothelial cells proliferation nMesangial cells proliferation nNeutrophils infiltration Normal glomerulus Post-streptococcal glomerulonephritis Immunofluorescence Granular deposits Subepithelial “lumps” immune complexes deposition Electron microscopy Clinical features nSystemic symptoms nAcute nephritic syndrome Acute onset Oliguri a Edema Hypertension Hematuria Azotemia Injures of the capillary wall GFR decrease Fluid retention Fluid retention, renin increase waste products increase KEY FACTS: Diffuse proliferative glomerulonephritis nCaused by immune complexes in glomerulus, often after streptococcal infection nCauses nephritic syndrome, with proliferation of endothelium and mesangium and recruitment of neutrophilis nMost cases recover, but a minority may rapidly progress to renal failure or slowly develop chronic renal failure Main types of glomerulonephritis nAcute proliferative(poststreptococcal, postinfectious)glomerulonephritis nRapidly progressive (cresentic) glomerulonephritis nChronic glomerulonephritis Rapidly progressive glomerulonephrits (RPGN; crescentic glomerulonephritis) nRPGN is a manifestation of severe glomerular injury characterized by the formation of crescent- shaped masses within the Bowmans space nRPGN occurs in a small percentage of patients with poststreptococcal glomerulonephritis, but can also be associated with many other forms of glomerular damage Pathogenesis nType I RPGN (12%): anti-GBM antibodies ( special type: Goodpasture syndrome) nType II RPGN (44%): immune complex-mediated disorder nType III RPGN (44%): pauci-immune complexes Crescentic glomerulonephritis Histological features nThe characteristic is the presense of crescents in most of the glomeruli (50%) nThe crescents lining Bowmans capsule are composed of a mixture of epithelial cells and macrophages proliferating Basement membrane destruction Cresentic glomerulonephritis Cresentic glomerulonephritis Cresentic glomerulonephritis Crescentic glomerulonephritisCrescent Cresentic glomerulonephritis Goodpasture syndrome Clinical features nRPGN is characterized by rapid and progressive loss of renal function associated with sever oliguria even anuria nPrognosis is poor Main patterns of glomerulonephritis nAcute proliferative(poststreptococcal, postinfectious)glomerulonephritis nRapidly progressive (cresentic) glomerulonephritis nChronic glomerulonephritis Chronic glomerulonephritis nend stage kidney nIt may be caused by many diseases Morphology nMacroscopically, affected kidneys are small and there is graularity of external surface (symmetrically contracted) nMicroscopically, there is fibrosis, hyalinization of glomeruli, tubular atrophy and interstitial fibrosis Chronic glomerulonephritis Chronic renal failure End stage kidney Clinical features nChronic glomerulonephritis develops insidiously nChronic glomerulonephritis is characterized by chronic nephritic syndrome Hypertension Proteinuria Anemia Azotemia Uremia Edema Uremic fibrinous pericarditis Required reading Suggested reading Case study nA 13-year-old girl who is brought by her parents because she had become lethargic and her face appeared swollen, particularly around her eyes. On enquiry, she had recently recovered from a flu-like illness. As part of routine examination her urine is tested with a dip-stick and is found that to contain both protein and blood. Case study nShe is also noted to have a mildly raised blood pressure. The patient is referred to hospital where she is found to have urea and creatinine together with a reduced urine output. Renal biopsy is performed. Histology nThe histology report reveals hypercellularity of glomeruli with neutrophils in capillary lumina. Granular deposition of IgG, C1q and C3 are seen in the glomerular basement membrane and mesangium. Electron microscopy confirms the presence of electron dense deposits in the basement membrane, predominantly in a subepithelial location. Diseases of urinary system nGlomerulonephritis nTubulointerstitial nephritis nDiseases of the lower urinary tract nTumors of urinary system Tubulointerstitial nephritis nTubulointerstitial nephritis refers to a group of inflammatory diseases of the kidneys that primarily involve the interstitum and tubules Tubulointerstitial nephritis nPyelonephritis nAcute tubular necrosis nInterstitial nephritis Pyelonephritis nAffecting tubules nAffecting interstitum nAffecting renal pelvis nOne of the most common diseases of the kidney Pyelonephritis nAcute pyelonephritis nChronic pyelonephritis Acute pyelonephritis nIncidence parallels that of obstructive uropathy nCaused by bacterial infection nCommon suppurative inflammation of the kidney and the renal pelvis Acute pyelonephritis nChildhood nPregnancy nElderly nInfancy: malesfemales nPuberty to middle age: femalesmales nPost-40 years: malesfemales Three age peaksThree age peaks Gender differencesGender differences Routes of bacterial infection nAscending infection from the lower urinary tract(most common): enteric gram-negative rods ( Escherichia coli) nBloodstream spread in bacteremic or septicemic states: staphylococci Route of infection Gross feature nSuppurative inflammation nCortical abscesses nMedullary abscesses Acute Acute pyelonephritispyelonephritis Acute Acute pyelonephritispyelonephritis Histological feature nInfiltration of tubules by neutrophils nAbscess formation nInterestitial edema Acute pyelonephritis Clinical feature nSudden onset nPain in the back nEvidence of systemic infection( chills, fever, malaise) nIndications of bladder and urethral irritation( dysuria, urgency, frequency of micturition) Urinary findings nPyuria ( white blood cells and pus cells in urine) nBacteriuria (bacteria in urine) Complications and sequelae nResolution nHealing with scarring nChronicity nPyonephrosis nRenal papillary necrosis nPerinephric abscess nDeath in uremia Papillary necrosis Pyelonephritis nAcute pyelonephritis nChronic pyelonephritis Chronic pyelonephritis nChronic pyelonephritis is defined as a morphologic entity in which prodominantly interstitial inflammation and scarring of the renal parenchyma is associated with grossly visible scarring and deformity of the pelvicalyceal system Chronic pyelonephritis nObstructive chronic pyelonephritis nReflux-associated chronic pyelonephritis (vesicoureteral reflux) Gross feature nKidneys are not equally damaged nIrregular areas of scarring nMarked calyceal deformities Gross feature not equally damaged Irregular areas of scarring Marked calyceal deformities Chronic pyelonephritis Chronic pyelonephritis Histological feature nChronic inflammatory cells infiltration, occasionally neutrophils nUneven interstitial fibrosis nDilation or contraction of tubules nGlomeruli show periglomerular fibrosis Chronic pyelonephritis “Thyroidization” Dilation of tubules Clinical feature nGradual onset of renal insufficiency nTubular dysfunction (polyuria) nUrinary tract infection nUremia Diagnosis nIntravenous pyelogram nUrine culture Requirement nList the complications of acute pyelonephritis nName the two types of pathogenesis for chronic pyelonephritis Outline nGlomerulonephritis nTubulointerstitial nephritis nDiseases of the lower urinary tract nTumors of urinary system nInfection nObstruction nStone formation nTumor formation nDevelopmental abnormalities Diseases of the lower urinary tract Urinary outflow obstruction nRenal stones nHydronephrosis Renal stones nUrolithiasis is calculus formation at any level in the urinary collecting system n most common sites: pelvicalyceal system and bladder. Main predisposing factors nIncreased concentration of solute in urine nReduced solubility of solute in urine Etiology nAcquired nInherited Renal calculus Renal staghorn calculus S Staghorn calculus Hydronephrosis Abscess Fluoroscopy Ureteral calculus Clinical feature nWithout symptoms nRenal colic with nausea , vomiting and hematuria nDual ache in the loins nRecurrent urinary tract infection Urinary outflow obstruction nRenal stones nHydronephrosis Hydronephrosis nHydronephrosis refers to dilation of the renal pelvis and calyces, with accompanying atrophy of the parenchyma, caused by obstruction to the outflow of urine nRenal pelvis nPelviureteric junction nUreter nBladder nUrethra HydronephrosisHydronephrosis Obstruction of the levelsObstruction of the levels Morphology nUnilateral :caused by obstruction at the level of the ureter, pelviureteric junction or renal pelvis nBilateral: caused by obstruction of the level of the bladder or urethra Hydronephrosis Normal urinary tract Intravenous pyelogram (IVP) Hydronephrosis Intravenous pyelogram (IVP) Hydronephrosis ( cut surface) Clinical feature nClinical features depend on the cause and site of the lesion Effects of hydronephrosis nObstruction is removed: renal function returns to normal nPersistence of obstruction: atrophy of renal tubules with glomerular hyalinization and fibrosis Outline nGlomerulonephritis nTubulointerstitial nephritis nDiseases of the lower urinary tract nTumors of urinary system Tumors of urinary system nTumors of the kidney nTumors of the bladder nTumors of the ureter nTumors of the urethra Angiomyolipoma Angiomyolipoma MRI Angiomyolipoma Renal cell carcinoma Renal cell carcinoma invade into the renal vein MRIRenal cell carcinoma Renal cell carcinoma (clear cell type) Key facts renal cell carcinoma nMale: female incidence is 3:1 nIncidence is greatest in those over 50 years, and increases with age nCommon presenting symptoms include painless hematuria, loin pain, loin mass nOccasional presenting symptoms include bone metastasis,brain metastasis, polycythemia Key facts renal cell carcinoma nLocal spread through renal capsule into perinephritic fat nLymphatic spread to para-aortic and other nodes nBlood stream spread to lungs, bone, brain, liver nPrognosis depends on stage at presentation Wilms tumor (Nephroblastoma) Common malignant tumors of childhood Abdominal enlargement Genetic defects on chromosome 11 Wilms tumor Metastatic