Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&

Information&
• Renal&Failure&
o Acute&or&chronic,&can&results&from&any&glomerular&or&tubule7interstitial&lesions&
o Always&associated&with&azotemia&(renal&origin)&
o Results&in&uremia&in&advanced&stages;&uremia:&clinical&syndrome&+&azotemia&
• Uremia&
o Azotemia,&acidosis&(accumulation&of&sulfates,&phosphates&and&organic&acids),&hyperkalemia,&abnormal&control&of&fluid&volume&(can’t&concentrate&urine,&later&on&can’t&dilute&it,&Na+&and&water&retention&cause&
CHF),&hypocalcemia&(can’t&make&active&form&of&vitamin&D&!&renal&osteodystrophy),&anemia&(decrease&Epo&secretion),&HTN&(increased&renin&production)&
o Can&include&anorexia,&nausea,&vomiting,&neurologic&disorders,&bleeding,&urinary&pigments&accumulate&in&the&skin,&fibrinous&pericarditis&&
• Pre7Renal&Azotemia&
o Due&to&decreased&blood&flow&to&the&kidney&(blood&loss,&decreased&CO,&hypovolemia&from&massive&burns,&peripheral&pooling&of&blood&from&gram&negative&sepsis)&
o Increased&tubular&reabsorption&of&sodium&and&water&!&oliguria&!&concentrated&urine,&decreased&Na+&excretion&&
o BUN:Cr&>20:1&(due&to&decreased&GFR&and&increased&tubular&reabsorption&of&urea)&
• Post7Renal&Azotemia&
o Mechanical&blockage&of&urinary&flow&
GLOMERULAR&DISEASE&
• Glomerular&structure&
o Glomerulus&+&Bowman’s&capsule&!&captures&and&funnels&the&filtrate&into&the&tubule&
o Layers:&blood&in&the&capillary&lumen&!&endothelium&(fenestrated)&!&BM&(lamina&rare&interna&!&lamina&densa&!&lamina&rara&externa)&with&Type&IV&collagen&!&visceral&epithelium&(podocytes&embedded&
into&lamina&rare&externa,&spaced&out&to&form&slit&diaphragms&in&between)&!&urinary&space&!&parietal&epithelium&(lines&the&cavity,&collects&the&urine/filtrate)&
o Mesangial&cells&between&the&capillaries,&secrete&matrix&
• Patient&presentation&
o Asymptomatic&hematuria&or&proteinuria&!&subtle&or&mild&glomerular&abnormality&
o Nephritic&syndrome:&hematuria&(grossly&visible,&RBCs&in&urine),&HTN&(mild&to&moderate),&variable&proteinuria,&oliguria&and&azotemia&
o RPGN:&nephritic&syndrome&with&rapid&decline&in&GFR&
o Nephrotic&syndrome:&>3.5&g/day&proteinuria,&hypoalbuminemia,&hyperlipidemia,&lipiduria&and&severe&generalized&edema&&
" Membranous&glomerulopathy,&minimal&change&disease,&FSGS,&MPGN,&IgA&nephropathy&&
o Chronic&renal&failure:&azotemia&!&uremia&(clinical&signs&and&symptoms)&
• Pathogenesis&(things&that&initiate&glomerular&injury)&
o Antibody&mediated&injury&–&IC&in&situ&deposition&(either&fixed&intrinsic&tissue&Ags&or&planted&Ags)&OR&circulating&IC&deposition&&
o Cell7mediated&immune&injury&(T&cells)&
o Alternative&complement&pathway&activation&(MPGN&Type&II)&
• Deposit&locations&
o Subepithelial&humps:&outside&of&the&BM&under&the&podocytes&(acute&GN)&
o Epimembranous&deposits:&outside&the&BM&but&between&the&podocyte&foot&processes&(membranous&glomerulopathy,&Heymann&GN)&
o Subendothelial&deposits:&between&the&BM&and&the&endothelial&layer&(Lupus&Nephritis,&MPGN)&
o Mesangial&deposits:&in&the&mesangium&(IgA&nephropathy)&
o BM&
Post7infectious& • & • Exogenous&IC& • Ab&forms&after& • Granular&IgG&and&C3&in& • Nephritic&syndrome& • Kids:&&
glomerulonephritis& • GABHS&Types&1,&4,&and&12& infection&!&forms&IC& GBM&and&mesangium& (*Prototype*)& o 95%&recover&with&
!&deposits&in& • Sub7epithelial&humps& • 174&weeks&after&a& conservative&
glomerulus&& • “Lumpy7bumpy”& strep&infection& therapy&within&6&
• Specific&Ag&is& immunofluorescence& (pharyngitis,& weeks&
unknown& • Hypercellular&glomeruli& impetigo)& o <1%&become&
due&to&leukocyte& • Kids,&aged&6710:& oliguric&!&RPGN&
infiltration&& malaise,&fever,& o <1%&progress&to&
• Diffuse&proliferative&GN& oliguria,&hematuria& chronic&GN&
• Foot&process&fusion& • Adults:&HTN,&edema,& • Adults:&&
elevated&BUN& o 60%&quick&recovery&
• Elevated&titers&for&1&or& o 40%&!&RPGN&
more&strep&Ags&
• Low&serum&
complement&
Disease& Characteristics/&
Information&
Non7streptococcal&acute& • Sporadic&cases&of&post7
GN& infectious&GN&
Rapidly&progressive& • Syndrome&associated&
glomerulonephritis& with&severe&glomerular&
(RPGN)& injury&
Goodpasture&Syndrome& • Most&common&disease&
associated&with&RPGN&
• Secondary&renal&
disease&
• High&prevalence&of&
HLA7DRB1&
Membranous& • 30%&of&nephrotic&
glomerulopathy& syndrome&in&adults&
• Highest&incidence&in&
teenagers&and&young&
adults&
• Seen&in&10%&of&SLE&pts&
• Sometimes&associated&
with&hep&B,&syphilis,&
malaria,&drugs&(gold&
salts,&PCN),&or&with&
malignancy&&
Minimal&change&disease& • AKA:&nil&disease,&lipoid&
nephrosis&
• Most&frequent&cause&of&
nephrotic&syndrome&in&
kids&
• Peak&incidence&age&276&
Etiology&&
• Bacterial:&staphylococcus&
endocarditis,&pneumococcal&
pneumoniae,&meningococcemia&
• Viral:&Hep&B&and&C,&mumps,&HIV,&
varicella,&infectious&
mononucleosis&
• Parasitic:&malaria,&
toxoplasmosis&
• Type&I&RPGN:&Anti7GBM&Ab,&
renal7limited,&20%&of&RPGN&
cases&
• Type&II&RPGN:&ICs,&granular&
staining&pattern&
• Type&III&RPGN:&Pauci7Immune,&
lack&of&anything&from&Type&I&or&
II,&ANCA7positive&!&Wegener’s&
• Anti7GBM&COL47A3&antigen&
• In&associated&with&systemic&
disease&&
• Unknown&antigens&(80%&of&
cases)&
• Unknown&cause&
• Higher&prevalence&in&certain&
HLA&types&
• Loss&of&glomerular&polyanion&
and&podocyte&injury&
Pathophysiology&& Morphology&&
• & • Granular&deposits&
characteristic&of&IC&
nephritis&
• Most&are&immune7 • Most&common:&crescentic&
mediated&& GN&due&to&proliferation&of&
parietal&epithelial&cells&+&
infiltration&of&
macrophages&and&
monocytes&
• ANCA7negative&forms&
with&ICs&or&Anti7GBM&Abs&
=&Type&II&
• Enlarged,&pale&kidneys&+&
petechial&hemorrhages&
• Distinct&ruptures&in&GBM&
!&“wrinkling”&of&GBM&
• Anti7GBM&Abs&react& • Extracapillary&
to&GBM&and&alveolar& proliferation&with&
septal&BM& crescents&
• Necrosis&
• Linear&IgG&and&C3&along&
BM&on&
immunofluorescence&
• Fibrin&in&crescents&
• No&deposits&
• GBM&disruption&
• Chronic&IC7mediated& • Glomeruli&normal&in&early&
disease&!& stages&
glomerular&wall&gets& • Diffuse&thickening&
leaky&!&protein& without&increased&
leaks&out& cellularity&+&spikes&
projecting&from&BM&into&
urinary&space&&
• “Spikes&and&domes”&
• Granular&IgG&deposits&
subepithelial&&
• Kidneys&normal&size&until&
advanced&stages&
• Immune& • Negative&for&staining&
dysfunction&!& • Normal&light&microscopy&
cytokine&release&!& (no&deposits,&GBM&intact)&
damage&to&visceral& • Foot&process&fusion&
epithelial&cells&!& (principle&lesion)&
foot&process&fusion& • Normal&or&slightly&
enlarged&kidney,&lipid7
laden&cortex&
• Lipid&in&proximal&tubules&
Clinical&Presentation& Treatment&and&Prognosis&
• & • &
• Hematuria& • Mild&forms&may&subside&
• RBC&casts&in&urine& • Early&intensive&
• Moderate&proteinuria& plasmapheresis,&
• Variable&HTN&and& steroids,&cytotoxic&
edema& agents&
• 3&major&DX&criteria:& • Type&II&doesn’t&respond&
acute&nephritis,& to&plasmapheresis&
proteinuria,&acute&
renal&failure&&
&
• RPGN& • Plasmapheresis&
• Pulmonary& • Steroids&
hemorrhage&+&renal& • Cytotoxic&agents&
failure&
• Nephrotic&syndrome& • Corticosteroid&therapy&
(insidious&onset)& INEFFECTIVE&
• 15735%&mild&HTN& • Proteinuria&persists&in&
• RBC&casts&or& 60%&of&patients&
hematuria&is&rare& • 40%&get&renal&
• Suspect&it&when& insufficiency&&
nephrotic&syndrome&+& • 10%&progress&to&ESRD&
azotemia&(increased&
BUN&and&creatinine)&
• Nephrotic&syndrome& • Dramatic&response&to&
(*Prototype*)& corticosteroid&therapy&
• NO&HTN& • Excellent&long&term&
• Edema&& prognosis&&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
Focal&segmental& • Most&common&cause&of& • Idiopathic&–&10%&of&kids&with& • Degeneration&and& • Like&MCD:&no&visible&BMM& • Nephrotic&syndrome& • Doesn’t&respond&to&
glomerulosclerosis& nephrotic&syndrome&in& nephrotic&syndrome,&35%&of& focal&disruption&of& changes&but&segmental& • Non7nephrotic&and& corticosteroids&
(FSGS)& adults&in&the&US& adults&with&nephrotic& podocytes& sclerosis&of&scattered& non7selective& • 50%&get&ESRD&within&10&
• Higher&prevalence&in& syndrome& • Plasma&proteins& juxtamedullary&glomeruli& proteinuria& years&
Hispanic&and&African7 • HIV7associated&nephropathy&(57 trapped&in&foci&!& • Focal&sclerosis,&hyalinosis& • Lower&GFR&than&MCD& • 20%&of&patients&have&
Americans& 10%&of&HIV&patients)& hyalinosis,&sclerosis& • Focal&staining,&IgM&and&C3& • HTN& rapid&course&!&massive&
• Renal&ablation&FSGS& positive& proteinuria&and&renal&
• Adaptive&response&after&loss&of& • Loss&of&foot&processes& failure&in&2&years&
renal&function&(IgA& • Epithelial&de7nudation& (malignant&focal&
nephropathy&!&FSGS)& (GBM&is&naked)& sclerosis)&
• Uncommon&inherited&forms& • Collapsing&FSGS:&entire& • Not&a&good&prognosis&
glomerular&tuft&
retracts/collapses,&
podocytes&proliferate&
Membranoproliferative& • 10720%&of&nephrotic& • Idiopathic&(primary)& • ICs&in&glomerulus&!& • Subendothelial&deposits& • Nephrotic&and& • Slowly&progressive&but&
glomerulonephritis& syndrome&in&kids& • Secondary&to&systemic& complement& • C3&deposited&in&granular& nephritic&syndrome& un7remitting&course&
(MPGN)&Type&1& disorders&(most&common)& activation& pattern&& (combined)& • 50%&chronic&renal&
• Ag&is&unknown&& • Glomerular& • Microscopic& failure&in&10&years&
hypercellularity&mostly&in& hematuria&or&mild&
mesangium& proteinuria&
• Leukocyte&infiltration&& • Possible&HTN,&oliguria,&
• Lobular&appearance&of& edema&
glomeruli&due&to&increase&
matrix&
• “Tram7track”&appearance&
of&GBM&(split&BM)&
Dense7deposit&disease& • 10720%&of&nephrotic& • Primary&renal&disease&in&kids& • Activation&of& • Dense&deposits& • Hematuria& • Worse&prognosis&than&
(MPGN&Type&2)& syndrome&in&kids& • Not&as&common&as&MPGN&Type&I& alternative& subendothelial&& • Decrease&serum& type&I&
• 70%&of&patients&have& complement& • No&IgG&in&glomeruli&& complement& • Recurrence&even&in&a&
C3NeF&(binds&to&C3& • Glomerular& (persistent&C3& transplant&
convertase&so&it&can’t& hypercellularity&mostly&in& activation)& • 50%&chronic&renal&
be&degraded&!& mesangium& • 50%&have&nephritic& failure&in&10&years&
persistent&activation)& • Leukocyte&infiltration&& syndrome&
• Lobular&appearance&due&
to&increase&matrix&
• “Tram7track”&appearance&
of&GBM&(split&BM)&
IgA&nephropathy& • AKA:&Berger’s&disease& • Unknown&cause& • IgA&increased&in& • Focal&mesangial& • Recurrent&hematuria& • 15740%&is&slowly&
• Renal7limited& serum&!&deposit&in& proliferative&GN& (gross&or&microscopic)&& progressive&over&20&
• Most&common&type&of& mesangium&!& • Increased&matrix& • Mild&proteinuria& years&to&chronic&renal&
GN&& alternative& • Mesangial&widening& • 5710%&have&HTN&and& failure&
• More&common&in& complement& • IgA&+&IgG,&IgM&and&C3&in& acute&nephritic&
patients&with&gluten& activation&!& the&mesangium& syndrome&
enteropathy&and&in& glomerular&injury& • Mesangial&and&para7 • 172%&have&RPGN&!&
liver&disease& mesangial&dense&deposits& acute&renal&failure&
• Most&common&in&older& • &
kids,&young&adults&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
Henoch7Shonlein& • IgA&nephropathy&that&is& • & • & • Renal&manifestations& • Skin&lesions&on& • Kids&have&excellent&
Purpura&(HSP)& NOT&renal&limited& same&as&Berger’s& extensor&surfaces&& prognosis&
• Most&common&in&age&37 • Abdominal&pain,& • Worse&prognosis&with&
8& vomiting,&bleeding& crescents&or&nephrotic&
• Can&happen&in&adults& • Non7migratory& syndrome&
(more&severe)& arthralgia&
• 33%&renal&
abnormalities&–&IgA&
nephropathy&&
Alport&Syndrome& • Hereditary&nephritis&& • 85%&are&X7linked& • Defective&assembly& • GBM&has&irregular&foci&of& • Chronic&renal&failure& • Renal&failure&between&
• More&common&in&males& • Autosomal&recessive&and& of&GBM&& thickening&alternating& • Nerve&deafness& ages&20750&in&men&
(1&in&5000)& autosomal&dominant&forms& with&thinning&& • Eye&disorders&(lens& • Worse&prognosis&if&
• Symptomatic&between& exist& • Split&BM& dislocation,&posterior& homozygous&&
ages&5720& • Abnormal&alpha73,&4,&or&5& • Moth7eaten/frayed& cataracts,&corneal& • ESRD&progression&not&
chains&in&Type&IV&collagen&& appearance&of&GBM& dystrophy)& common&&
• Gross&or&microscopic&
hematuria&
• Mild&proteinuria&
possible&
Thin&BM&Lesion& • Most&common&cause&of& • Mutation&in&alpha73&or&4&chains& • & • Diffuse&thinning&of&GBM& • Normal&renal&function& • Good&prognosis&unless&
benign&familial& of&type&IV&collagen& homozygous&&
hematuria& • Most&patients&heterozygous&&
Chronic& • The&pool&of&ESRD&with& • Post7strep&GN&172%& • & • Hyalinized&glomeruli& • Chronic&renal&failure& • Relentlessly&progressive&
glomerulonephritis& many&contributing& • RPGN&90%& • Granular&or&negative&on& • Changes&in&dialysis&& at&variable&rates&
streams& • Membrane&glomerulopathy&307 immuno7fluorescence& • Uremic&complications&& • Renal&transplant&
50%& required&
• MPGN&50%&
• IgA&nephropathy&30750%&
• FSGS&50780%&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
Lupus&Nephritis& • Class&IV&is&the&worst& • Renal&manifestations&of&SLE& • & • Minimal&or&no&change& • Minimal&or&no&change& • Minimal&or&no&change&
prognosis***& (<5%&of&SLE&patients)& (<5%&of&SLE&patients)& (<5%&of&SLE&patients)&
• Class&I&and&Class&II&SLE& • Class&I&and&Class&II&SLE& • Class&I&and&Class&II&SLE&
(10725%):&Mesangial& (10725%):&Mesangial& (10725%):&Mesangial&
Lupus&Nephritis& Lupus&Nephritis& Lupus&Nephritis&
o IF&mesangial&deposits& • Class&III&(20735%):& o Good&prognosis&
o Normal&glomeruli& Focal&Proliferative& • Class&III&(20735%):&
• Class&III&(20735%):&Focal& Lupus&Nephritis& Focal&Proliferative&
Proliferative&Lupus& o Microscopic& Lupus&Nephritis&
Nephritis& hematuria& o 5%&of&patients&
o Focal&necrotizing& • Class&IV&(35760%):& advance&to&ESRD&
lesions&in&glomeruli& Diffuse&Proliferative& within&5&years&
• Class&IV&(35F60%):& Lupus&Nephritis& o Good&prognosis&&
Diffuse&Proliferative& o Nephritic& • Class&IV&(35F60%):&
Lupus&Nephritis& syndrome& Diffuse&Proliferative&
o Thickened&GBM&in& • Class&V&(10715%):& Lupus&Nephritis&
“wire&loops”& Membranous&Lupus& o 50%&respond&to&
o Subendothelial& Nephritis& immunosuppressiv
deposits&& o Nephrotic& e&therapy&
o Marked& syndrome& o 33%&go&to&ESRD&
hypercellularity& o May&have& o Worst&prognosis&
o Increase&glomerular& microscopic& • Class&V&(10715%):&
size& hematuria&& Membranous&Lupus&
o Anti7IgG&antibodies& • Advanced&Sclerosing& Nephritis&
deposited& Lupus&Nephritis&(4%)& o Normal&plasma&
• Class&V&(10715%):& o Advanced&stage& creatinine&for&5&yrs&
Membranous&Lupus& for&Class&III,&IV& (no&progression)&
Nephritis& and&V& • Advanced&Sclerosing&
o Diffuse&thickening&of& Lupus&Nephritis&(4%)&
capillary&walls& o End&stage&
o Intra7endothelial&
tubule7reticular&
structures&&
o Nephrotic&syndrome&
• Advanced&Sclerosing&
Lupus&Nephritis&(4%)&
o Global&sclerosis&in&
>90%&of&glomeruli&
Disease& Characteristics/&
Information&
Diabetic&Nephropathy& • 30%&of&ESRD&are&DM&
nephropathy&&
• 40%&of&Type&I&and&Type&
II&DM&patients&&
Amyloidosis& • Associated&with&chronic&
inflammatory&
conditions&(rheumatoid&
arthritis)&or&plasma&cell&
disorders&(multiple&
myeloma)&
Wegener& • &
granulomatosis&&
Plasma&Cell&Dyscrasia& • &
TUBULAR&AND&INTERSTITIAL&DISEASES&
• Tubulointerstitial&Nephritis&
o Many&etiologies:&infections&(acute&and&chronic&pyelonephritis),&toxins,&metabolic&diseases,&physical&causes&(obstruction),&neoplasms&(Multiple&Myeloma),&immunologic&causes&(transplant&rejection,&
Sjogren’s&syndrome,&Sarcoidosis)&
o Clinical&course&very&different&from&glomerular&disease:&no&nephritic&or&nephrotic&syndromes&but&there&are&defects&in&tubular&function&!&polyuria,&nocturia,&salt&wasting,&metabolic&acidosis,&defects&in&
reabsorption&and&secretion&&
• Acute&vs.&Chronic&&
o Acute:&rapid&onset,&interstitial&edema,&leukocyte&infiltration,&focal&tubular&necrosis&
o Chronic:&mononuclear&cell&infiltration,&prominent&fibrosis,&widespread&tubular&atrophy&
Acute&Kidney&Injury& • AKA:&Acute&Tubular&
(AKI)& Necrosis&(ATN)&
• Most&common&cause&of&
acute&renal&failure&&
Etiology&&
• &
• &
• &
• Multiple&myeloma&
• Ischemia&due&to&decreased&or&
interrupted&blood&flow&
o Microangiopathies&
o Thrombosis&&
o Hypotension,&shock&
• Toxic&injury&to&tubules&–&drugs,&
Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
• Metabolic&defect&!& • Glomeruli&effects:& • Progression&related&to& • Closely&monitor&
hyperglycemia&!& capillary&BM&thickening,& extent&of&proteinuria& albumin&levels&
advanced& diffuse&mesangial& • Usually&manifests&as& • Reversible&if&albumin&
glycosylation&end& sclerosis& nephrotic&syndrome& stays&in&the&body&&
products&!& • **Nodular&
thickened&GBM&+& glomerulosclerosis**&
increased&matrix&+& (Kimmelstiel&Wilson&
diffuse&mesangial& nodules)&
sclerosis&(narrows& • Arteriolar&sclerosis,&
capillary&lumen)& hyalinosis,&advanced&
• Increase&GFR,& renovascular&
increase&glomerular& atherosclerosis,&
capillary&pressure,& thickened/tortuous&
glomerular& afferent&arteriole&&
hypertrophy& • Papillary&necrosis&&
• Tubular&BM&thickening&&
• Acellular&PAS7positive&
nodules&&
• Marked&thinning&of&the&
cortex&
• Irregular&cortical&
depression&from&
pyelonephritis&&
• & • Amyloid&deposits&in& • Nephrotic&syndrome& • Uremia&!&death&
glomerulus&(AL&=&light& • Amyloid&protein&
chain,&or&AA&type)&at& identified&by&special&
subendothelial&and& stains&(Congo&red)&and&
mesangial&locations& birefringence&under&
polarized&light&&
• Criss7cross&fibrillary&
pattern&under&electron&
microscope&&
• & • Crescentic&RPGN& • Hematuria& • &
• Mild&decline&in&GFR&
• Amyloidosis&IGs& • Lobular&accentuation&& • & • &
(lamba&light&chains)& • Mild&hypercellularity&
cause&nodular&
lesions&
• Tubule&cell&injury& • Ischemic&AKI:&patchy& • Initiation&stage&!& • Worse&prognosis&
(susceptible&due&to& necrosis&in&PCT,&PST&and& maintenance&!& • Prolonged&ischemia&(57
high&energy& ascending&Loop&of&Henle& recovery&& 10&minutes)&!&BM&
requirements)& • Oliguria&in&initial&stage& necrosis,&nowhere&for&
• Necrotic&epithelial& can&cause& the&tubular&epithelial&
cells&sloughed&into& hyperkalemia&(!& cells&to&regenerate&onto&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
dyes,&myoglobin,&Hgb,&radiation&& tubular&lumen&!& • Toxic&AKI:&acute&tubular& cardiac&arrhythmias&a& • Better&prognosis&
• Acute&tubulointerstitial& casts&obstruct&flow&& injury&mostly&in&PCT,&PST& major&risk&at&that& • 95%&recover&
nephritis&(hypersensitivity&rxn& and&patchy&in&ascending& point)&
to&drugs)& Loop&of&Henle& • &
• Urinary&obstruction&–&tumor,& • Ethylene&glycol:&
BPH,&blood&clots& vacuolization&of&all&PCT&
epithelial&cells,&swollen&&
Pyelonephritis& • Acute&Pyelonephritis& • Bacterial&infection:&85%&are& • & • Patchy&interstitial& • Sudden&onset&pain&at& • Can&develop&papillary&
from&gram&(7)&normal&flora&(E.& suppurative&inflammation& CVA& necrosis&(coagulative&
coli&>&Proteus&>&Klebsiella&>& through&renal&cortex&and& • Fever,&malaise& necrosis),&pyonephrosis&
Enterobacter)& medulla&& • Bladder&and&urinary& (pus&buildup&in&renal&
• 95%&are&from&ascending& • Glomeruli&are&SPARED,& irritation&–&dysuria,& system),&or&perinephric&
infection&from&the&bladder& resistant&to&infection& frequency,&urgency&& abscess&
• Associated&with&UTIs& • Yellow7gray&areas&of& • Leukocyte&casts&in& • Antibiotics&cure&in&a&few&
• Usually&some&predisposing& acute&inflammation&on&the& urine&with&high& days&
condition&(urinary&tract& cortex&with&pus&and& neutrophils&(pus&
obstruction,&catheter,& abscess&formation&& casts)&
vesicoureteral&reflux,&
pregnancy,&DM,&
immunosuppression)&
• Chronic&Pyelonephritis& • Bacterial&infection& • & • Irregularly&scarred& • Insidious&onset&or& • Can&progress&to&FSGS&(if&
• More&complex&& kidneys& recurrent&acute& it&does&=&worse&
• Predisposing&conditions:&reflux& • Tubular&damage& pyelonephritis& prognosis)&
nephropathy,&chronic& • Xanthogranulomatous& • Back&pain,&fever,& • Often&leads&to&renal&
obstructive&pyelonephritis& pyelonephritis:&foamy& pyuria,&bacteriuria& HTN&and&ESRD&
(recurrent&infections&on&top&of& macrophages&and&plasma& &
obstructive&lesions)& cells,&usually&due&to&
Proteus&infection&!&
yellow&nodules&form&
Acute&drug7induced& • Adverse&reaction&to& • Sulfonamides,&synthetic& • Onset&not&dose& • Pronounced&edema&in& • 15&days&after&the& • Withdraw&the&offending&
interstitial&nephritis&& drugs& penicillins&(methicillin,& related& kidney&& medication&!&fever,& agent&ASAP&
ampicillin),&rifampin,&diuretics& • Elevated&IgE&+&IgE7 • Lymphocytes&and& eosinophilia,&rash& • Recovery&typically&(may&
(thiazides),&NSAIDs,&allopurinol,& plasma&cells&and& macrophages&infiltrate& (25%&of&pts)& take&several&months)&
cimetidine& basophils&=&Type&I& • Non7necrotizing& • Hematuria,&mild&
• Most&likely&immune&mediated& HS& granulomas&–&methicillin,& proteinuria,&
• Mononuclear&or& thiazides& leukocyturia&
granulomatous& • Normal&glomeruli&except& • 50%&of&patients&get&
infiltration&and& with&NSAIDs:&minimal& oliguria&
positive&skin&tests&=& change&disease,&nephrotic&
Type&IV&HS& syndrome&
Analgesic&nephropathy& • Chronic&renal&disease& • Excessive&intake&of&analgesic& • Papillary&necrosis& • Cortical&atrophy& • More&common&in& • Drug&withdrawal&!&
mixtures,&usually&with& !&cortical& • Variable&necrosis,& women& renal&function&stabilizes&
phenacetin&in&it&or&aspirin& tubulointerstitial& calcification,&sloughing&at& • Recurrent&headaches,& and&eventually&
nephritis&due&to& papillae& muscle&pain&!&these& improves&
impeded&urine&flow& people&take&more&
!&renal&damage& analgesics&
• Aspirin&blocks&PG& • Can’t&concentrate&
vasodilation&!& urine&
papillae&susceptible&
to&ischemia&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
Nephrolithiasis& • Uric&acid&stones& • & • & • & • Symptomatic&when& • &
• 5710%&of&Americans& they&get&to&the&ureter&
have&stones& and&cause&intense&
• 80%&are&unilateral& pain,&ulceration,&
• Men&>&women& bleeding,&or&
• Peak&age&onset:&20730& obstruction&
• Small&stones&are&
worse&
VASCULAR&DISEASES&
• Most&kidney&diseases&have&a&vascular&component&
&
Benign&Nephrosclerosis& • & • & • Hemodynamic& • Changes&associated&with& • Normally&doesn’t& • Increased&risk&of&renal&
changes&!&medial& hyaline&sclerosis&of&renal& cause&renal& failure&if&African&
and&intimal& arterioles&and&small& insufficiency&or& descent,&higher&BP,&or&
thickening&!& arteries& uremia& second&underlying&
plasma&proteins& • Multi7focal&ischemia&of& disease&(DM)&
extravasate&through& kidney&parenchyma&
epithelium&!&BM& supplied&by&a&sclerotic&
matrix&deposition&!& vessel&&
hyaline&deposition& • Densely&adherent&capsule&
in&arteriolar&walls& on&autopsy&with&pebbled&
surface&
• Cortex&surface&has&fine&
homogenous&granularity&
• Fibroelastic&hyperplasia&
(narrows&the&lumen&of&
arterioles)&
• Ischemic&atrophy&
Malignant&HTN&+& • Renal&disease& • & • Kidney&vasculature& • Ischemic&kidneys& • Elevated&renin& • Medical&emergency&&
Accelerated& associated&with& damage&(long7 • Small&pin7point& • Systolic&BP&>&200& • Anti7HTN&therapy&&
Nephrosclerosis& malignant&HTN& standing&HTN)&!& hemorrhages&on&cortex& mmHg& • 75%&survive&5&years&
• Usually&superimposed& increase& surface& • Diastolic&>&120&& • 50%&get&restored&renal&
on&pre7existing&benign& permeability&of& • Hyperplastic&arteriolitis& • Papilledema,& function&&
essential&HTN& small&vessels&!& (not&the&same&as&hyaline& encephalopathy,&CV&
• Uncommon&(175%&of& endothelial&injury&!& changes)& abnormalities,&renal&
people&with&HTN)& focal&death&of&cells& • Fibrinoid&necrosis&of& failure&
in&vessel&wall&(focal& arterioles& • Increased&cranial&
hemorrhage)&!& • “Onion&skinning”& pressure&!&HA,&
platelet&deposition& appearance&of&the&intimal& nausea,&vomiting,&
!&fibrinoid&necrosis& layers& visual&impairment,&
in&arterioles&and& scotomas,&spots&in&
small&arteries&!& sight&
hyperplastic&
arteriolosclerosis&&
Renal&artery&stenosis& • Unilateral&is&a&common& • Most&common&cause&is& • Constrict&renal& • & • Presents&like&normal& • Renal&scans,&IVP&to&
cause&of&HTN&(275%&of& atheromatous&plaque&occlusion& artery&!&stimulate& HTN& diagnose&
cases)& at&origin&of&the&artery&(more& renin&secretion&by& • & • Localize&the&stenosis&
• Curable&HTN& common&in&men)& JG&!&ang&II& with&ateriography&&
• Fibromuscular&dysplasia&of&the& increases&!&
renal&artery&(media&layer& vasoconstriction&!&
thickening,&more&common&in& increase&BP&
women,&“string&of&beads”&on&
contrast&or&dye7image)&
Disease& Characteristics/&
Information&
Thrombotic& • HUS&(hemolytic&uremic&
microangiopathies& syndrome)&
• TTP&
Atherosclerotic& • B/L&renal&artery&
ischemic&renal&disease& disease&
Atheroembolic&renal& • &
disease&
Sickle&cell&disease& • &
nephropathy&
Renal&infarct& • Kidneys&are&
predisposed&to&infarcts&
because&of&high&flow&
and&end7organ&nature&
of&arterial&blood&supply&
CONGENITAL&ANOMALIES&
• 10%&of&babies&born&have&GU&malformations&
Kidney&agenesis& • Unilateral&agenesis:&
very&uncommon&
Ectopic&Kidney& • &
Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
• Typical&HUS&=&epidemic,&classic,& • Endothelial&injury& • Patchy&or&diffuse&cortical& • Prodrome:&influenza7 • Manage&renal&failure&
diarrheal,&due&to&E.&coli& !&HUS& necrosis&& like&symptoms,& with&dialysis&
(0157:H7),&usually&in&children& • E.&coli&infection&!& • Subcapsular&petechiae& diarrhea& • Most&recover&within&
endotoxin&(shiga7 • Sudden&onset&of& weeks&
like&toxin)&injures& bleeding& • 25&years&to&kidney&
endothelial&cells&!& manifestations:& disease&&
microangiopathy& hematemesis,&melena&
• Oliguria&&
• Hematuria&
• Microangiopathic&
hemolytic&anemia&
(fragmented&RBCs&in&
blood)&
• Thrombocytopenia&&
• 50%&have&HTN&&
• Atypical&HUS&=&non7epidemic,& • Excessive&and& • Increased&cellularity&in& • Typically&in&adults& • Worse&prognosis&
no&diarrhea,&50%&have& inappropriate& arterial&walls&in&chronic& • Relapses&and& • Some&have&neurologic&
mutation&in&complement& activation&of& forms&& exacerbations& symptoms&
regulatory&proteins&or&acquired& complement&& • Progress&to&ESRD&
forms&(adults)&
• Inherited&or&acquired& • Large&polymers&of& • & • Neurologic& • Transfusion&and&
deficiency&of&ADAMST13& vWF&!&platelet& manifestations& immunosuppression&if&
(regulates&vWF)& aggregation&!& prominent& autoantibodies&found&
micro7thrombi&and& • Fever& (decreases&mortality&
vascular&obstruction& • Microangiopathic& <50%)&
!&endothelial&injury&& hemolytic&anemia&
• Thrombocytopenia&
• 50%&go&into&renal&
failure&
• & • & • & • & • Surgical&
revascularization&&
• & • & • Embolization&of& • & • &
fragments&of&
atheromatous&plaques&
from&aorta&or&renal&artery&
into&intra7parenchymal&
renal&vessels&
• & • & • & • & • &
• Embolism&(mural&thrombosis& • & • & • & • &
due&to&MI,&vegetative&
endocarditis)&
• & • & • & • Rule&this&out&if&a& • &
patient&is&going&to&
undergo&a&procedure&
• & • & • & • Abnormally&lower& • &
• Typically&in&the&pelvis&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
Horseshoe&kidneys& • 90%&fused&lower&poles& • & • & • & • & • &
• 10%&fused&at&upper&
Multicystic&renal& • Sporadic&disorder& • Failure&of&ureteric&bud&to&reach& • Extensive&cysts&with& • Multiple&large&variably7 • Unilateral,&isolated& • Normal&life&expectancy&
dysplasia& • “Renal&cystic&disease”&& renal&blastema&during& poorly7 sized&cysts&and&cartilage& anomaly& but&kidney&has&to&be&
• Most&common& embryonic&development& differentiated& & • Found&as&an& removed&
congenital&renal&cystic& • Never&stimulates&formation&of& mesenchyme&& abdominal&mass&& • Early&diagnosis&is&most&
disease& the&renal&cortex,&no&formation& • No&normal& • 90%&have&an&absent& important&&
• Most&common&palpable& of&the&nephron& glomeruli& ureter&or&
mass&in&the&newborn&& • & • & ureteropelvic&
obstruction&&
CYSTIC&DISEASES&
• Renal&cysts&are&pretty&common:&usually&they&are&small&and&asymptomatic&and&found&incidentally&
• Confusion&is&to&distinguish&them&from&malignancies&&
Adult&Polycystic&Kidney& • Common& • Autosomal&dominant& • Both&proteins&are& • Large,&multicystic&kidneys& • Hematuria& • Chronic&renal&failure&age&
Disease&(ADPKD)& • 1/400&births,&5710%&of& • High&penetrance&& localized&to&the& • Multiple&expanding&cysts& (hemorrhage&from& 40760&
chronic&renal&failure& • PKD&1&mutation&=&85%&of&cases,& primary&cilium&on& that&ultimately&destroy& progressive&dilation&of& • Increased&incidence&of&
cases&that&require& encodes&for&polycystin71& the&tubular& renal&parenchyma&and& the&cysts)& UTIs,&nephrolithiasis&
dialysis&or&transplant& • PKD&2&mutation,&encodes&for& epithelial&cells& cause&renal&failure& • Flank&pain,&palpable& • PKD1:&worse&prognosis,&
• Always&bilateral& polycystin72&& • Kidney&enlarged&b/l&& mass& earlier&symptoms&
• Occurs&in&Northern& • & • HTN& • PKD2:&older&onset,&later&
European&ancestry&& • Liver&cysts&(40%)& development&of&renal&
• Berry&aneurysms,& failure,&5%&get&ESRD&by&
subarachnoid& age&50)&
hemorrhages&(4710%& • Faster&progression&in&
die&from&this)& black&men&with&HTN&
• Commonly&
symptomatic&around&
age&40&
• 82%&have&diverticular&
disease&
Childhood&Polycystic& • All&are&autosomal& • PKHD1&gene&mutation,&encodes& • Unknown& • Kidneys&enlarged&with& • & • &
Kidney&Disease& recessive& fibrocystin&& • Protein&is&localized& smooth&external&
(ARPKD)& to&the&primary& appearance&
cilium&of&tubular& • Dilated&elongated&
epithelial&cells&& channels&that&replace&the&
medulla&and&cortex&
• 4&subtypes:& • Most&common&subtype& • >90%&of&collecting&ducts& • Mild&hepatic&fibrosis& • Survive&for&only&a&few&
o Perinatal& are&cystic& hours&
• Death&due&to&
hypoplastic&lungs&
o Neonatal&& • & • 60%&of&renal&collecting& • Mild&hepatic&fibrosis& • Survive&for&a&few&
ducts&are&cystic& months&
• Die&from&renal&failure&
o Infantile& • & • 20%&of&collecting&ducts& • Hepatic&fibrosis&& • 90%&survive&neonatal&
are&cystic& • Hepatic&failure& period&
• Portal&HTN& • Die&in&early&childhood&
• Systemic&HTN&
common&
o Juvenile& • & • <10%&of&collecting&ducts& • Progressive&hepatic& • Most&don’t&survive&to&
are&cystic& fibrosis&& adolescence&&
• Portal&HTN&with&
esophageal&varices&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
Medullary&sponge& • Relatively&uncommon& • & • Unknown&& • Medullary&cysts&on& • Hematuria& • Benign&
kidney& excretory&urography& • UTI& • Typically&incidental&
• Papillary&ducts&are&dilated& • Recurrent&renal& finding&
stones&
Nephronophthisis&& • Most&common&genetic& • 7&gene&loci&are&implicated&in&the& • Distal&tubule&injury& • Small&kidneys& • & • &
causes&of&ESRD&in& set& !&disrupts&BM&!& • Contracted&granular& • &&
children&and&young& chronic&and& surfaces&
adults&& progressive&tubular& • Corticomedullary&cysts&
• Sporadic,&non7familial& • & atrophy&involving& • Widespread&atrophy&and& • & • &
(25%&of&cases)& medulla&and&cortex& BM&thickening&
• Adult7onset&medullary& • Autosomal&dominant&& !&interstitial& • Manifests&in&adults& • Progresses&to&chronic&
cystic&disease&(15%)& • MCKD1&and&MCKD2&genes&are& fibrosis&and& • Salt&wasting& renal&failure&
affected& eventual&renal& • Polyuria&&
insufficiency&&
• Familial,&juvenile& • Most&common& • Manifests&in&children&& • Difficult&diagnosis:&cysts&
nephronophthisis&(407 • Autosomal&recessive& • Salt&wasting& are&very&small&
50%)& • NPH1,&NPH2,&NPH3&genes&are& • Tubular&acidosis&& • Chronic&renal&failure&
affected& • Polyuria,&polydipsia& begins&in&childhood,&
• Can’t&concentrate& terminal&in&5710&years&
urine&
• Growth&retardation&
• Anemia&&
• Extra7renal&
associations:&
oculomotor&
abnormalities,&liver&
fibrosis,&cerebellar&
abnormalities&&
• Renal7retinal&dysplasia& • Autosomal&recessive& • Kidney&disease&+& • &
(15%)& ocular&lesions&
• Manifests&in&children&&
Simple&cysts& • & • No&genetic&factors& • & • Single&or&multiple&cysts&in& • Microscopic& • Benign&
normal7sized&kidneys& hematuria&is&rare&& • Common&post7mortem&
• Usually&occur&on&renal& finding&
cortex&surface,&175&cm,&
filled&with&clear&fluid&&
Acquired&renal&cystic& • Occurs&in&patients&on& • & • Obstruction&of& • Cystic&degeneration&in& • Hemorrhage,& • Dependence&on&dialysis&
disease& dialysis&for&long& tubules&from& ESKD& hematuria& for&life&
periods&of&time&(>10& interstitial&fibrosis& • Numerous&cortical&and& • Erythrocytosis& • Kidney&transplant&is&
years)& or&oxalate&crystals&& medullary&cysts& • Neoplasia&(RCC&in&the& preferred&
• 7%&of&patients&develop& • Cysts&contain&clear&fluid& wall&of&the&cyst)&
RCC&in&the&walls&of&the&
cysts&
UROPATHY&
• Obstructive&uropathy&=&urinary&tract&obstruction&
o Increased&susceptibility&to&infection&and&stone&formation&
o Hydronephrosis:&dilation&of&the&renal&pelvis&and&calyces&associated&with&progressive&atrophy&of&the&kidney&due&to&obstruction&of&urine&flow&
o Occurs&at&any&level&between&urethra&and&renal&pelvis&&
o Morphology&
" Sudden,&complete&obstruction:&GFR&decreases&!&mild&dilation&of&pelvis&and&calyx,&sometimes&get&parenchymal&atrophy&if&not&relieved&
" Sub7total&or&intermittent&obstruction:&Normal&GFR,&progressive&dilation&of&either&the&bladder&or&ureter,&ultimately&the&kidney&&
" Chronic&cases:&cortical&tubular&atrophy&with&diffuse&interstitial&fibrosis,&pyramid&apexes&get&blunted/cupped,&cortical&thinning&at&end&stages&
Disease& Characteristics/&
Information&
Urolithiasis&& • Affects&5710%&of&the&
population&&
• More&common&in&men&
• Peak&onset&age&20730&
• Hereditary&
predispositions&to&
stone&formation:&gout,&
cystinuria,&
hyperoxaluria&&
• Unilateral&in&80%&of&
patients&
TUMORS&OF&THE&KIDNEY&
• Benign&and&malignant&tumors&exist&in&the&kidney&&
• Benign&tumors&rarely&cause&clinical&problems&(except&oncocytoma)&
• Malignant&tumors&are&clinically&important&–&pay&attention&to&symptoms&and&prognosis&&
Renal&Papillary& • Most&common&
Adenoma& • Found&in&7722%&of&
people&at&autopsy&&
Angiomyolipoma& • Present&in&25750%&of&
people&with&Tuberous&
Sclerosis&
• Technically&a&
hamartoma&
Etiology&&
• 4&main&types&of&stones&
o Calcium&stones&=&70%&
(50%&of&these&are&
idiopathic&hypercalciuria&
without&hypercalcemia)&
o Struvite&stones&=&15%&
(magnesium&ammonium&
phosphate,&usually&after&a&
bacterial&infection&with&
proteus&or&staph)&
o Uric&acid&stones&=&5710%&
(associated&with&gout,&
leukemias,&or&idiopathic,&
stones&are&insoluble&in&
acidic&urine)&
o Cystine&stones&=&172%&(due&
to&genetic&defects&in&renal&
reabsorption&of&cysteine,&
stones&form&at&low&urinary&
pH)&
• &
• &
Pathophysiology&& Morphology&&
• & • Typically&stones&form&in&
renal&pelvis&or&calyx&or&in&
the&bladder&
• & • Small&(<0.5&cm)&
• Present&in&the&cortex&
• Derived&from&renal&
tubules&&
• Yellow7gray,&pale,&discrete&
nodules&
• Have&an&acidophilic&
cytoplasm,&papillae&and&
thin&fibrovascular&cores&
• & • Lesions&in&the&cerebral&
cortex&can&cause&epilepsy,&
mental&retardation&and&
skin&abnormalities&
• Made&up&of&vessels,&
smooth&muscle&and&fat&
Clinical&Presentation& Treatment&and&Prognosis&
• Increase&urine& • &
concentration&of&
whatever&the&stone&is&
made&of&(they&are&
supersaturated)&&
• Urine&flow&obstruction&
!&ulcerations&and&
bleeding&
• Could&be&
asymptomatic&&
• Asymptomatic&& • Benign&tumor&
• Size&is&prognostic&
• <3&cm&=&metastasis&rare&
• Typically&presents&in& • Benign&tumor&
adults,&middle7aged,&
F>M&
• Susceptible&to&
spontaneous&
hemorrhage&!&shock&
can&be&initial&
presentation&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
Oncocytoma& • 5715%&of&all&surgically& • Arise&from&the&intercalated&cells& • & • Eosinophilic&cells&with& • Present&in&adulthood,& • Rarely&invades&or&
resected&renal& of&the&collecting&duct& numerous&mitochondria& rarely&familial&& metastasizes&&
neoplasms& • Large,&tan&or&brown&to& • Familial&forms&have&
mahogany&color,& multicentricity,&
homogeneous,&well7 bilaterality&or&
encapsulated& oncocytosis&
• Tend&to&be&small&with&a&
central&stellate&scar&
• Abundant&acidophilic&
cytoplasm,&granular&
• May&show&alveolar,&
nesting,&tubular&or&solid&
patterns&
Renal&fibroma/& • & • & • & • Reno7medullary& • & • Incidental&findings&on&
hamartoma& interstitial&cell&tumor&–& autopsy&
adults&&
• Mesoblastic&nephromas&
are&found&in&children&&
JG&Cell&Tumor& • Very&rare&renal&tumor& • & • & • Renin7producing&tumor& • Present&in&young& • Surgical&removal&of&the&
adults&with&severe& tumor&to&treat&the&HTN&
HTN&due&to&elevated&
renin&secretion&&
Renal&Cell&Carcinoma& • 3%&of&all&newly7 • Arise&from&tubular&epithelium&& • & • Gross&yellow&color,&can& • Diagnostic&features:& • Tend&to&metastasize&
(adenocarcinoma&of&the& diagnosed&visceral& • Most&cases&are&sporadic& happen&anywhere&in&the& CVA&pain,&palpable& widely&before&there&are&
kidney)& cancers&in&the&US& • 4%&are&familial&–&usually&a& kidney& mass,&hematuria& any&signs&or&symptoms&&
• 85%&of&renal&cancers&in& younger&person& • Originates&in&renal& • Generalized& • 25%&of&patients&present&
adults&& • Several&sub7types&of&RCC:&(see& tubules&and&usually&arise& symptoms:&malaise,& with&a&metastasis&&
• Usually&older&(age&607 below)& in&one&of&the&poles& weakness,&fever,& • 45%&5&year&survival&
70)&and&males& (frequently&upper&pole)& weight&loss& rate&
• AKA&Hypernephroma&& • Secondary& • Worse&prognosis&with&
• Risk&factors:&smoking,& polycythemia&(from& renal&vein&invasion&&
obesity,&HTN,&estrogen& Epo&production)&
therapy,&other&renal& • Clear&cell&carcinoma:&most& • Arise&from&proximal& • Oil&Red&O+&(lipid& • &
diseases& common,&70780%&of&RCC& tubule&epithelium&& accumulation&in&the&
• Cells&with&clear&or& cells&turn&them&yellow,&
granular&cytoplasm&& test&positive&for&fat)&
• Non7papillary&&
• Sporadic&(95%)&
• 80%&have&a&non7deleted&
VHL&allele&
• Solitary,&unilateral&lesion&&
• Papillary&carcinoma:&10715%& • Papillary&growth&pattern& • Typically&hemorrhage,& • Better&prognosis&
• From&distal&convoluted& cystic&as&they&grow&
tubules&
• Multifocal&and&b/l&
• May&have&psammoma&
bodies&
• Highly&vascularized,&little&
stroma&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
• Chromophobe&renal&carcinoma:& • Cells&have&prominent&cell& • & • Excellent&prognosis&&
5%& membranes,&pale&
eosinophilic&cytoplasm&
with&a&halo&around&the&
nucleus&&
• Multiple&chromosome&
losses&and&hypodiploidy&
in&the&cells&&
• Well7defined&cell&
membrane&
• Collecting&duct&(Bellini&duct)& • From&the&collecting&duct& • & • Poor&prognosis&&
carcinoma:&1%& cells&in&the&medulla&
Urothelial&carcinomas& • 5710%&of&primary&renal& • Come&from&the&urothelium&in& • Block&urinary&flow& • Exactly&like&urinary& • Symptomatic&early&on& • Poor&prognosis&
of&the&renal&pelvis& tumors& the&pelvis&& !&palpable& bladder&carcinomas&& –&sit&in&the&pelvis&and& • Typically&infiltrate&the&
• Increased&incidence&in& hydronephrosis&and& produce&hematuria& wall&of&the&pelvis&and&
patients&with&analgesic& flank&pain& calyx&
nephropathy&or&Balkan& • 10%&5&year&survival&if&
nephropathy& high7grade&infiltration&&
LOWER&URINARY&TRACT&
Double/Bifid&Ureter& • Associated&with&double& • & • & • & • Usually&unilateral& • &
renal&pelves&or&with& • Asymptomatic&&
large&kidney&
development&
Diverticula&of&the& • & • Congenital&form:&failure&to& • & • Pouch7like&evagination&of& • Site&of&urine&stasis&!& • &
urinary&bladder& develop&normal&musculature&or& the&bladder&wall&& infection,&bladder&
obstruction&during& • Frequently&have&multiple& calculi&common&&
development& narrow&necks&& • Most&are&small&and&
• Acquired:&due&to&prostatic& asymptomatic&&
enlargement&typically,&
obstructed&urine&flow&!&
thickened&bladder&wall&!&
increase&pressure&!&diverticula&
Exstrophy&of&the& • & • Developmental&failure&of&the& • Exposed&bladder& • & • Increased&risk&of& • Can&be&surgically&
baldder& anterior&abdominal&wall&!& mucosa&!&colonic& adenocarcinoma&in& corrected&
bladder&communicates&through& glandular& bladder&remnants& • Long&term&survival&is&
the&large&defect&with&the& metaplasia&!& possible&
surface&of&the&body&or&lies&open&& subject&to&infection&
!&spreads&to&upper&
urinary&tract…&&
Vesicoureteral&reflux& • Most&common&bladder& • & • Urinary&stasis&!& • & • Most&commonly& • Management&depends&
anomaly&& infection&!&ureter& diagnosed&in&infancy& on&the&grade&of&reflux&
• 1/3&of&kids&with& and&kidney& or&childhood&& • Grades&173:&reflux&
recurrent&UTIs&have&it& involvement& spontaneously&
regressed&as&child&
grows&and&bladder&
matures&
• Low&dose&antibiotics&to&
suppress&infections&and&
bacterial&growth&&
Urachal&cysts& • & • Central&region&of&the&urachus& • & • & • Can&develop&glandular& • &
persists& carcinomas&from&cysts&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
Acute&cystitis&& • More&common&in& • Bacterial:&E.&coli&>&Proteus&>& • & • Non7specific&acute& • Triad&of&symptoms:& • &
women&due&to&shorter& Klebsiella&>&Enterobacter&>& inflammation&of&the& increased&urinary&
urethra&& Staph&saprophyticus& bladder& frequency,&lower&
• Predisposing&factors:& • Candida&albicans&in& • Hyperemia&of&the&bladder& abdominal&
bladder&stones,&urinary& immunosuppressed&patients&or& mucosa,&exudate&possible& pain/suprapubic&pain,&
obstruction,&BM,& long7term&antibiotic&therapy& • Hemorrhagic&cystitis:& dysuria&
catheter&insertion,& • Schistosomiasis&rare&in&the&US,& occurs&in&patients&on& • Low&grade&fever&
immuno7deficient,& common&in&Egypt&and&Sudan& cytotoxic&chemo&drugs& • Turbid&urine&
trauma&during&sex& • Virus& • May&have&hematuria&
• Chlamydia&&
• Mycoplasma&&
Chronic&cystitis&& • & • & • & • & • Must&have&thickened& • &
bladder&wall&
(hypertrophy&of&the&
thick&muscularis&
layer)&
• Trabeculated&
appearance&of&the&
inner&bladder&surface&
Interstitial&cystitis& • Most&common&in& • & • & • Persistent,&painful&form&of& • Intermittent&severe& • Biopsy&to&rule&out&CIS&
(Hunner&Ulcer)& women& chronic&cystitis& suprapubic&pain&
• Chronic&mucosal&ulcers& • Urinary&frequency&
(Hunner&ulcers)&in& • Urgency&&
bladder&mucosa&& • Hematuria&
• Dysuria&without&
evidence&of&bacterial&
infection&&
Malacoplakia& • Related&to&bacterial& • & • & • Soft,&yellow&slightly&raised& • & • &
infections&commonly&–& mucosal&plaques&374&cm&
E.&coli&and&Proteus& wide&
• More&common&in& • Large&foamy&macrophages&&
immunosuppressed& • Michaelis7Gutmann&
patients,&transplant& bodies:&laminated&
recipients&& mineralized&concretions&
from&calcium&deposits&in&
enlarged&macrophages&
stuck&in&a&lysosome&
• Unusual&macrophages&
and&giant&phagosomes&&
Polypoid&cystitis&& • Inflammatory&condition& • Most&commonly&due&to&in7 • Urothelium&!& • & • Resemble&papillary& • &
from&irritation&to& dwelling&catheters&& broad&bulbous& urothelial&carcinomas&
bladder&mucosa& polypoid&
projections&due&to&
submucosal&edema&
Cystitis&Glandularis&and& • Usually&coexist& • & • & • Nests&of&urothelium& • & • &
Cystitis&Cystica& growing&downward&into&
the&lamina&propria&
(“Brunn&nests”)&
Follicular&cystitis& • & • & • & • Aggregations&of& • Chronic&infection& • &
lymphocytes&into&
lymphoid&follicles&in&the&
mucosa&and&underlying&
wall&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
Eosinophilic&cystitis&& • Can&be&a&manifestation& • No&known&specific&etiology&& • & • Infiltration&by& • & • &
of&a&systemic&allergic& submucosal&eosinophils&&
reaction,&immune& • Non7specific&subacute&
disorder,&parasitic& inflammation&&
infection&or&a&sequel&to&
chemotherapy&or&
radiation&&
Urinary&Bladder& • 7%&of&all&cancers& • & • & • & • Bladder&tumor&!& • Prognosis&depends&on&
Neoplasms& painless&hematuria& grade&of&papillary&tumor&
• Frequency,&urgency,& when&diagnosed&&
dysuria,&hematuria&are& • Early&detection&is&most&
occasionally&seen&on& important&
presentation&& • Treatment:&cystoscopy,&
• Pyelonephritis&or& biopsy,&cytologic&exams,&
hydronephrosis& urine&test&for&specific&
possible&if&the&ureteral& markers&
orifice&is&involved&& • Radical&cystectomy&only&
• 60%&are&discovered&as& for&a&tumor&invading&the&
a&single&tumor& muscularis&propria&or&a&
localized&to&the& high7grade&tumor&that&
bladder& doesn’t&respond&to&
other&treatments&&
• Urothelial& • & • & • & • & • 50%&have&invaded&
(transitional)&tumors& bladder&wall&when&
=&epithelial&origin& discovered&
o 90%&of&all&bladder& • Lamina&propria&invasion&
tumors& =&worse&prognosis&&
o Men&>&women,& • Muscularis&propria&
urban&areas,& invasion&(detrusor&
developed&nation& muscle)&=&decreased&
• Contributing&factors& survival&(30%&5&year&
o Smoking&increases& survival&rate)&
risk&377x&
o Schistosoma&
haematobium&
infections&&
o 17p&deletions&!&
associated&with&
p53&mutations&
• Several&specific&types:&
• Papillomas& • Exophytic&Papilloma& • & • Single&papilloma,&small& • & • Recurrence&and&
(<2&cm),&superficially& progression&is&rare&
attached&to&mucosa&by&a& • <10%&progress&to&
stalk& higher&grade&lesions&
• Central&core&of&loose&CT&
• Inverted&Papilloma& • & • Raised&bump&in&the& • Slightly&younger&males& • Benign&lesion&
trigone&region&of&the& • Cured&by&excision&&
bladder,&covered&by& • Low&recurrence&rate&
normal&mucosa& • <10%&progress&to&
higher&grade&lesions&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
• Papillary&urothelial& • & • & • Thicker&urothelium&than&a& • & • May&recur&
neoplasms&of&low& papilloma&& • Don’t&invade&
malignant&potential& • Tend&to&be&larger& • <10%&progress&to&
(PUNLMPs)& higher&grade&lesions&
• Low&grade&papillary& • The&most&common&(45%&of& • & • Orderly&appearance,&cells& • & • Low&grade&cancer&
urothelial&cancers& papillary&tumors)& are&evenly&spaced& • <10%&recur&and&invade&
• Minimal&but&definite&
evidence&of&nuclear&atypia&
–&hyperchromatic&nuclei,&
mitotic&figures,&mild&
variation&in&size&and&
shape&
• High&grade&papillary& • 30%&of&papillary&tumors& • & • Cells&that&are&not& • & • High&recurrent&rate&
urothelial&cancer& cohesive,& • 25%&mortality&
chaotic/disorganized& • 80%&will&invade&into&
• Large&hyperchromatic& muscular&layer&&
nuclei& • Mets&to&prostate,&
• Frequent&mitotic&figures& seminal&vesicles,&
ureters,&
retroperitoneum&and&
regional&nodes&in&40%&
• CIS&(carcinoma&in&situ)& • & • & • Flat,&non7invasive& • Malignant&cells&shed& • 50775%&will&invade&
urothelial&carcinomas& into&the&urine& muscle&if&left&untreated&&
• Full7thickness&atypia&or& • Commonly&multifocal&&
malignant&cells&scattered&
around&the&normal&
urothelium&&
• Mucosal&reddening&
• Invasive&urothelial& • & • & • & • & • Extent&of&invasion&is&
cancer&(invasive& prognostic&&
papillary&carcinoma)& • Low&stage&=&99%&5&year&
survival&
• Muscle&invasion&=&50%&
5&year&survival&rate&
• 30%&mortality&&
• Squamous&cell& • 377%&of&bladder&cancer&in&the& • & • & • Associated&with& • &
carcinomas& US& chronic&bladder&
• Higher&in&areas&of& irritation&or&infection&&
schistosomiasis&
Disease& Characteristics/& Etiology&& Pathophysiology&& Morphology&& Clinical&Presentation& Treatment&and&Prognosis&
Information&
& • Mesenchymal&Tumors& • Benign&tumors& • & • & • & • &
(non7epithelial)& o Leiomyoma&–&smooth&
o Very&rare& muscle,&most&common&&
o Lipoma&–&mature&adipose&
tissue&
o Fibroma&
o Neurofibroma&
o Inflammatory&pseudo7
tumors&(mistaken&for&
papillary&carcinomas&
frequently)&
• Malignant&tumors&
o Rhabdomyosarcoma&=&
sarcoma&botryoides&
(children&age&4,&M=F)&
o Leiomyosarcoma&(adults,&
age&60,&2:1&M>F,&very&rare)&
o Lymphoma&(usually&B&cells,&
adults,&age&65,&6:1&F>M,&
most&have&chronic&cystitis,&
good&prognosis&because&
radio7sensitive)&
&