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					BENIGN              general info                       pathology                                                                                                                    prognosis
TUMORS
renal papillary         originate in renal tubules    GROSS:                                                                                                                           no real
adenoma                 usually papillary                small, discrete, w/in cortex; pale yellow-gray, seemingly encapsulated nodules                                                reliable
                        commonly found @              MICRO:                                                                                                                            marker for
                         autopsy                          complex, branching, papillomatous structures w/ lots of complex fronds projecting into a cystic space                         benignity
                                                          cells may be tubules, glands, cords, &/or totally undifferentiated masses                                                     so usually
                                                          cell types for all are regular w/no atypia                                                                                    treat small
                                                          cells are cuboidal to polygonal w/ small central nuclei & clear cytoplasm                                                     tumors as
                                                          histo. criteria--do not differ from low-grade papillary renal cell adenocarcinoma & share some immunohistochemical &          early CAs
                                                           cytogenic features (trisomies 7 & 17) w/papillary CAs
renal fibroma or        incidental finding @          GROSS:                                                                                                                           no
hamartoma                autopsy                          small foci, gray-white firm, usually <1cm, w/in pyramids                                                                      malignant
(renomedullary                                         MICRO:                                                                                                                            propensities
interstitial cell                                         fibroblast-like cells & collagenous tissue; w/EM--features of renal interstitial cells
tumor)
angiomyolipoma          present in 25-50% of pts          consists of vessels, smooth m., & fat                                                                                       benign
                         w/tuberous sclerosis
                         (lesions of cerebral cortex
                         that produce epilepsy &
                         mental retardation & skin
                         abnormalities)
oncocytoma              epithelial tumor thought      GROSS:                                                                                                                           benign
                         to arise from intercalated       tan or mahogany brown, relatively homogeneous & usually well-encapsulated; up to 12cm
                         cells of collecting ducts     MICRO:
                        not uncommon                      large eosinophilic cells w/small rounded, benign-appearing nuclei; w/EM--eosinophilic cells have lots of mitochondria
MALIGNANT                     general info                                                                  pathology                                                       prognosis
TUMORS
renal cell carcinoma              85% of renal CA in adults                                                GROSS:                                                              has tendency to
(hypernephroma,                   30,000 new cases/yr; 12,000 deaths/yr                                       yellow; may arise in any part but usually in upper poles         metastasize widely
adenocarcinoma of                 usually 6th or 7th decade                                                SERIES OF EVENTS:                                                    before local sx or signs
kidney)                           ♂ > ♀ (2-3:1)                                                               as tumors enlarge, may bulge into calyces & pelvis &            ~25% of new pts have
*there are 3 kinds; the           arise from tubular epithelium                                                eventually extend through walls of the collecting                evidence of metastasis
book listed some general          smokers have 2x incidence                                                    system & even into the ureter                                    (esp. to lungs & bones)
info and then info specific
                                  other risk factors: obesity (esp. ♀); HTN; unopposed estrogen tx;           tendency of tumor to invade renal v.--can extend as             5 yr survival ~45% &
to each type. Info for                                                                                          solid column of cells up the IVC & into rt. side of heart        up to 70% w/o distant
                                   exposure to asbestos, petroleum products, & heavy metals
specific types will follow                                                                                  RANDOM INFO:                                                         metastases
                                  ↑ed incidence in pts w/chronic renal failure, acquired cystic disease,
their corresponding #
                                   & tuberous sclerosis                                                        collecting duct carcinoma is a rare variant w/irregular         w/renal v. invasion, 5 yr
1) clear cell                                                                                                   channels lined by highly atypical epithelium w/hobnail           survival ~15-20%
                                  most is sporadic; some unusual forms are autosomal dominant
(nonpapillary) carcinoma                                                                                        pattern                                                         nephrectomy is tx of
2) papillary carcinoma             familial CA
                                  assoc. w/Von Hippel-Lindau (VHL) syndrome                                   sarcomatoid changes arise infrequently in all types of           choice
3) chromophobe renal                                                                                            renal cell carcinoma
carcinoma                         familial clear cell carcinoma; hereditary papillary carcinoma
1) clear cell                     70-80% of renal cell CAs                                                 GROSS:                                                          no specific px given
(nonpapillary) carcinoma          usually sporadic, can be familial or assoc. w/VHL                             solitary, unilateral; spherical, 3-15cm, bright yellow-
                                  usually (98%) assoc. w/loss of chromosome 3 (harbors VHL gene);                gray-white tissue that distorts renal outline
                                   nondeleted VHL allele shows somatic mutations in ~ 80%                        usually areas of ischemic opaque, gray-white necrosis;
                                                                                                                  foci of hemorrhagic discoloration, & areas of softening
                                                                                                                 margins sharply defined & confined w/in renal capsule
                                                                                                            MICRO:
                                                                                                                 cells w/clear or granular cytoplasm; nonpapillary
                                                                                                                 growth pattern can be solid to trabecular (cordlike) or
                                                                                                                  tubular (resembling tubules)
                                                                                                                 most tumors are well-differentiated but some show
                                                                                                                  marked nuclear atypia w/bizarre nuclei & giant cells
2) papillary carcinoma            10-15% of renal CAs                                                      GROSS:                                                          no specific px given
                                  familial & sporadic                                                           can be multifocal & bilateral
                                  papillary growth pattern                                                      typically hemorrhagic & cystic (esp. when large)
                                  not assoc. w/3p deletions                                                     appear as yellow golden flakes
                                  sporadic--usually, abnormalities are trisomies (7, 16, 17) and loss of   MICRO:
                                   Y                                                                             cuboidal or low columnar cells arranged in papillary
                                  familial--usually trisomy 7                                                    formations
                                  chromosome 7 has MET gene                                                     interstitial foam cells in papillary core
                                  PRCC (papillary renal cell carcinoma) on chromosome 1--usually                Psammoma bodies may be present
                                   involved in sporadic tumors in kids                                           stroma--scanty but highly vascularized
3) chromophobe renal              5% of renal CAs                                                          MICRO:                                                          excellent compared w/clear
carcinoma                         multiple chromosome losses & extreme hypodiploidy                             cells w/prominent cell membranes & pale eosinophilic      cell & papillary CAs
                                  thought to grow from intercalated cells of collecting ducts                    cytoplasm usually w/halo around nucleus
                                                                                                                 arranged in solid sheet w/largest cells around blood
                                                                                                                  vessels
urothelial carcinomas of          5-10% of 1º renal tumors                                                 GROSS:                                                              5 yr survival 50-70% for
renal pelvis                      range from benign papillomas to frank papillary                               may be multiple, involving pelvis, ureters, & bladder          low-grade superficial
                                   carcinomasdifficult to differentiate from low-grade papillary CAs       MICRO: exact same as urothelial tumors of bladder                    lesions; 10% w/high-
                                  usually pre-existing or concomitant bladder urothelial tumor             see study ?s for bladder b/c it is a very long list of               grade infiltrating tumors
                                  benign appearance but invasion of wall of pelvis & calyces is            possibilities, depending on the grade of the tumor
                                   common
PEDIATRIC            general info                                  pathology                                                                                     prognosis
KIDNEY
TUMORS
Nephroblastoma           embryonal neoplasm derived form          GROSS:                                                                                            majority of tumors are in "favorable
(Wilm's Tumor)            blastemal cells                             usually solitary, rounded masses sharply defined by pseudocapsule; variable size               group"
                         most common GU CA in kids                   cut surface: bulging & lobulated & usually pale gray or tan & soft consistency; if            "unfavorable group"--small %; areas of
                         most common in kids 1-3 yr                   lots of stromal elements are present, may have firm consistency & whorled                      nuclear anaplasia, or rarely foci of high-
                         no significant sex predilection              appearance                                                                                     grade renal cell carcinoma
                         etiology unknown w/exception of          MICRO:                                                                                            anaplasia = 1) multipolar mitotic
                          several weird conditions at bottom of       triphasic patterns w/blastemal, stromal, & epithelial cell type are most characteristic        figures & 2) marked nuclear
                          p.246 in notes                              biphasic & monophasic lesions w/blastemal or embryonal tubular differentiation                 enlargement of affected nuclei (>3x
                         seems to be a genetic predisposition         have been seen                                                                                 nonanaplastic nuclei in same specimen)
                          (p13 and p15.5 region on                    patterns can be extremely variable                                                            if anaplasia is diffusely distributed, it is
                          chromosome 11; 16q of chromosome            blastema is present in most & may be the only element in the tumor                             associated w/poor px
                          16; also chromosomes 1,8,12                 most have an epithelial component of differentiation--sometimes it is rare rosettes           focal anaplasia (1 or a few sharply
                         incidence in white is about 1 in 8 to        barely recognizable as tubular forms, some are mainly or totally easily recognizable           localized regions of a 1˚ tumor) has ~
                          10,000; higher in blacks; lower in           tubular or papillary elements                                                                  same px as a lesion of favorable
                          orientals                                   glomerular structures usually lack capillaries (w/several exceptions)                          histology
                         about 1% have a + family hx                 heterologous epithelial differentiation may occur (usually mucinous & squamous                anaplasia--marker of ↑ed resistance to
                         probably autosomal dominant                  epithelium, sometimes ciliated epithelium)                                                     current tx rather than a sign of ↑ed
                         bilateral in about 5%                       variety of stromal patterns--myxoid & spindle cells in almost all specimens where              tumor aggressiveness; no effect on px
                         sx-usually abdominal mass,                   they form matrix for most of blastemal & epithelial foci; stromal differentiation can          of tumors confined to kidney (stage I);
                          sometimes pain, hematuria, HTN,              be smooth & skeletal m., adipose tissue, cartilage, bone & osteoid, mature ganglion            seen in ~ 4.5% of nephroblastomas--
                          and sx related to traumatic rupture          cells & neuroglial tissue                                                                      rare in kids < 2 yrs, ↑es to ~10% at 6+
                                                                                                                                                                      yrs
clear cell sarcoma       ~5% of pedi renal tumors                 GROSS:                                                                                            w/chemo tx death rate has dropped
                         lots of clear cells                         tumor-kidney jxn is sharply defined                                                            from 30-40% to 7%
                         predilection for metastasis to bone;        seems to arise from medullary or central region of the kidney                                 outcome is a little uncertain b/c of late
                          poor px                                     cut surface--lt. brown or gray & seems mucoid                                                  recurrences & death
                         rare in <6 months and young adults;         most tumors have a dense, tough texture
                          peaks @ 2 yrs & falls thereafter         MICRO:
                         ♂ > ♀ (1.6:1)                               nests or cords of cells separated by vascular channels of capillary size accompanied
                         no geographic or racial                      by spindle cells
                          predisposition; does not seem to be         most contain numerous vesicles that appear intracytoplasmic w/ light microscope but
                          genetic predisposition or pre-existing       are actually intercellular
                          or co-existing predisposition               nuclei--evenly dispersed chromatin
rhabdoid tumor           median age at dx is 11 months            GROSS:                                                                                            80% of pts died of tumor
                         >90% are >3 yrs                             most <500g; moderately well demarcated & soft consistency; cut surface--pale &                most deaths w/in 1 yr of dx
                         ♂ > ♀ (1.5:1)                                bulging
                         assoc. w/separate 1º tumors of           MICRO:
                          primitive neuroepithelial origin in         sheets of lg., loosely cohesive cells w/acidophilic cytoplasm & distinct cell borders;
                          midline of posterior or middle               cells w/characteristic cytoplasm acidophilic PAS+ hyaline inclusions tend to be
                          cranial fossa                                clustered & not all cells have these inclusions
                         no assoc. w/syndromes predisposing          w/EM--hyaline inclusions correspond to tightly whorled intermediate filaments
                          to nephroblastoma or consistent              adjacent to the nucleus
                          genetic abnormalities                       tumor cells are + for cytokeratin & vimentin
                      NEPHROTIC SYNDROME                                   NEPHRITIC SYNDROME
Proteinuria           > 3 – 3.5g/day (Selective = IgG/albumin < 0.1       < 3.0g/day (Non-selective = IgG/albumin > 0.5)
                      predominant albumin excretion)
Onset                 Insidious                                            Abrupt
Renal Function        Initially normal                                     Increase BUN & Creatinine
Hematuria             Sometimes                                            Often  RBC > 5 per high powered field
Cell proliferation    None                                                 Increase # of cells (hypercellularity)
GFR                   No change                                            Decrease GFR
Other                 - Hypoalbuminemia : Dec. liver funct. (elderly)     - Red cell casts
                           edema                                           - Azotemia – Increase urea in blood 
                      - Hyperlipidemia :                                                  Inc. BUN
                           Decrease break down of lipids                                 Inc. Creatinine
                           shunting from production of albuminlipids     - Oliguria
                           Inc. hepatic synthesis                         - Mild to moderate HTN
                      - Complications
                           Malnutrition
                           Hypercoagubility :
                                 Increase  Factor V, VIII & Fibrinogen
                                 Decrease  antithrombin III, antiplasmin
                           Acute tubular necrosis : NSAIDS Decreased
                               ProstaglandinsVasoconstrictionnecrosis
                           Infection : Decrease Immunoglobulins
                           Fanconi syndrome :
                                 aminoaciduria
                                 glucosuria
                                 phosphaturi
                                 proximal tubular acidosis
                                                                   nd
                           Loss of trace metals  osteomalacia, 2
                               hyperPTH
Diseases (causes)    66% adults & 90% children  Nephrotic syndrome         Acute G-Nephritis
                     2nd to:                                                    Acute Proliferative G-nephritis
                      Membranous G-nephritis                                      Poststreptococcal G-nephritis
                      Lipoid nephrosis (Minimal Change)                           Subacute Bact. Endocarditis
                      Focal segmental G-sclerosis                               Rapid Progressive (Crescentic) G-nephritis
                      Membranoproliferative G-nephritis                           Type I: Anti-GBM
                      IgA nephropathy (minor)                                       - Idiopathic
                                                                                     - Goodpasture syndrome
                     33% adults & 10% children  Nephrotic syndrome                Type II: Immune Complex
                     due to systemic disease:                                        - Idiopathic
                         Diabetes mellitus                                          - Postinfectious
                         SLE                                                        - SLE
                         Amylodiosis                                                - IgA Nephropathy (major)
                                                                                     - Henoch-Schonlein purpura
                                                                                   Type III: Pauci-immune (ANCA)
                                                                                     - Idiopathic
                                                                                                         -       Wegener granuloatosis
     Disease that present with Nephrotic Syndrome                                                        -       Microscopic PAN

              Effected           Pathogenesis             LM                      IF            EM                  Labs                     Prognosis            Therapy
Minimal       2-6 y/o             Dec.
                                      neg. charge         No change               No change    Loss foot processes Proteinuria =              25-40%             Children:
Change        60-70 y/o           of BM                                                          – visceral          Selective                 Remission          60mg/m/day 4 wks
(Lipoid       M>F (2:1)           Abnormal T-cell                                               epithelial cells                             50% Relapse        90% response in 4 wks
Nephrosis,                         funct. (assoc.                                                                                             5YSR 50% w/        Adults:
MCD)                               Hodgkin’s                                                   “fusion”of foot                                 Tx.                2mg/kg/day 4 wks
                                   Lymphoma – 20-30                                              processes                                    7-12% w/o Tx       90% response in 8 wks
                                   y/o)
                                                                                                                                              Tx =
                                  1/3 preceding Upper                                                                                         corticosteriods.
                                   Respiratory tract                                                                                                               10% = steroid
                                   infection or Vaccine
                                                                                                                                                                    resistcyclophos-
                                  1/3 small decrease
                                   GFR
                                                                                                                                                                    phamide
                                  RF = norm                                                                                                                       50% Relapse
                                  BP= norm                                                                                                                        50% steroid depend.
Focal         Children     Idiopathic                      Focal                 IgM          Loss foot processes Sterile pyuria = 8-12     Poor:                Corticosteriods = not
Segmental     Young adults 2nd other disease              Segmental             C3             – visceral          WBC hpf w/o               Hematuria          as effective as w/
Glomerulo-                                                 Sclerosis             (deposited     epithelial cells    UTI                       High BP            MCD
sclerosis     Cameron’s pts                                Inc. mesangial         only in                                                     Mesangial         Response to steroids:
               (British study)    - High BP
                                                            matrix                 sclerotic                                                    hypercellularit   MCD=80%
               - 60% pts > 15     - IgA nephropathy
               years                                       Collapse BM            segments)                                                    y                 FSGS=15-20%
                                  - other kidney dx.
                                                           Hylinosis: Inc.                                                                  Transplant:
              Habib’s pts                                   hyaline masses                                                                    High
               (French study)    Theory:
                                                                                                                                               recurrence
               - 60% pts. < 5    Mesangial cell
                                                          Hyalinosis also seen                                                                Mesangial          Long term Tx:
               years              dysfunction 
                                                          in:                                                                                  hypercellularity   Cyclophosphamide
                                  microfibrils
                                                                                                                                              Rapid               Chlorambucil
                                  dysfun. Dec.               - Diabetic
                                                                                                                                               progression
                                  contractility                 nephtopathy
                                                              - Reflux                                                                       Well matched
                                  dilation capillary
                                                                 nephropathy                                                                  donor (=bad)
                                  loops  Inc.
                                  filtration                 - Atherosclerosis
                                                                                                                                             Spont. remission:
                                  structural damage
                                                                                                                                              MCD=25-40%
                                   sclerosis
                                                                                                                                              FSGS=20%

Membran-      Adults             Immune complex:          Stage 1: normal         Stage 1:     Stage 1: granular      Labs = not helpful     25% spont.           Young 
ous Nephro-   40-50y/o           Ab (in blood)           Stage 2: (silver        IgG & C3     deposit                  for diagnosis          Remission           corticosteriods
pathy         M>F (2:1)           binds  Ag (in          stain)                  on outer     Stage 3: complex                              25% nephritic        70-80 + y/o  no Tx
(Most                             GBM)                    epimembranous           surface      in BM                  Kidney biopsy            range              Diabetic  no Tx
Common)       - Elderly          No circulating immune    spikes                  Stage 2:     Stage 4: complex        confirms dx.           proteinuria w/
                                  complexes
                                                          Stage 3: no spikes      dots         seem empty                                      norm RF            Primary  steroids
               assoc. w/                                  Stage 4: no spikes      between                             Diff Tx. for primary   ESKD:                2nd 
                                 No RBC casts                                     spikes                                & 2nd MN               50% adult          Syphilis – can’t tx
                                 RF & BP = normal                                 Stage 3:                                                     15% children         w/ steroids
               malignancy                                               dots inside                                                                         Hep B – interferon
                            Complications:                              membrane                                                                            Lupus nephritis -
                             Anti GBM dx.                                                                                                                   steriods
              - Kids assoc.
                             Renal vein
               w/ infection
                                thrombosis
               or collagen
               dx.
Membrano- 5-30 y/o          Immune complex          - Inc. mesangial    IgG           Subendo-thelial      Fluctuation of          Poor Prognosis:       Cocktail -
proliferative M=F (1:1)                                matrixLobular                  depos.               complement levels         Nephrotic range    steroids
                                                                                                                                      Gross hematuria
G-nephritis                   dx.                      appearance of    Granular      “Railroad track”                                                    Cytotxic meds
                                                                                                                                      HTN
Type 1                                                 glomeruli         deposits       split of BM                                   Crescents          Antiplatelet agents
                                                    - Thick GBM                                                                       Sclerosing         Anticoagulants
                            Classic & alternative                                                                                      glomeruli
                               complement pth.
                               Activation

              Effected      Pathogenesis            LM                  IF             EM                   Labs                   Prognosis             Therapy
Membrano- 5-30 y/o          NOT immune              Inc. mesangial      C3            Intramem-            C3Nef Ab against       Poor Prognosis:       Cocktail -
proliferative M=F (1:1)       complex dx.             matrixLobular                    branous             C3 convertase            Nephrotic range    steroids
                                                                                                                                      Gross hematuria
G-nephritis                 Alternative               appearance of                     deposits            continued activation                          Cytotxic meds
                                                                                                                                      HTN
Type 2                        complement pth.         glomeruli                       “Railroad track”      of complement            Crescents          Antiplatelet agents
                              activation                                                split of BM         Hypo-complement-          Sclerosing         Anticoagulants
                                                                                                            emia                       glomeruli
                                                                                                                                                         Corticosteriod = not
 IMPORTANT 20% of MPGN with both:           Nephritic synd. -- RBC casts, High albumin & creatinine                                <5% w/ spont.          helpful
                                                                                                                                    Remission
                                           Nephrotic synd.-- 24 hour protein > 3.5g
Drug Related                Heroine associated
Nephro-                     IV drug user 
pathy                       - GN 2nd to bacterial                        Spontaneous Remission Rates:
                               endocarditis
                            - Hep B. associated                          MPGN (5%) < FSGN (20%) < MN (25%) < MCD (40%)
                               w/ MN, MPGN,
                               classic PAN

      Disease that present (primarily) with Nephritic Syndrome
                                                                        IgG           Subepithelial        Streptozyme Test:       Spontaneous           None  spontaneous
Acute Proliferative GN      Immune complex          Diffuse             IgM            “humps”              ASO titer             recovery:               recovery
                            Ags:                    Hypercellularity    C3            Mesangial deposits    Streptokinase           Children: 90%      Antibiotics  Group A
Poststrep-                    M-Protein               crescents                                           Hyaluronidase           Adults: 67%          Strep infection
                                                                                                            DnaseB
tococcal       2-12 y/o        (bacterial wall)                         Granular                                                                         Sodium Restriction
Glomerulo-                    Endostrptosin                             deposits                          Urine Findings:                               Biopsy if:
nephritis                      (intracellular)                                                             - Fe(na) < 1%                                  Prolonged oliguria
                              Cationic Protein
                                                                                                           - RFI < 1%                                     Anuria
                               (secreted)                                                                  - Urine > 40                                   Persistent
                                                                                                            Protein(cret.)                                 hypocomplementemi
                                Alternate pth.                                                                                                                 a
                                  activation  C3 =                                                                                                           Nephritic syndrome
                                  decreased
Subacute                        Staph Aureus           Focal proliferative    IgG          Subendo-thelial    Hypocomple-
Bact. Endo-                     Strep Viridans           GN                   IgM          Mesangial deposits   mentemia
carditis G-                        - Right side        Necrosis               C3                                Cryoglobu-linemia
nephritis                             cardiac          Intracapillary         Granular                          Rheumatoid factor
                                      problems           thrombi               deposits
                                   - Negative
                                      bacterial
                                      culture




Rapid Progressive (Crescentric) GN
               Effected          Pathogenesis          LM                      IF           EM                Labs                     Prognosis         Therapy
Type 1:       20-30 y/o         Immune Complex       Glomeruli:               IgG           Disruption of GBM Radio-immuno-           50%  ESKD         High dose:
Anti GBM                        Ag in GBM: Ab         Fibrinoid              C3            Endothelial        assay =                Anti GBM GN   Corticosteriod
CGN                               binds  Ag in        necrosis               Linear          swelling         diagnostic               worse              Immunosuppressive
(11%)                             situ               Tubule: epithelial         depositio Loss foot processes                           prognosis          Plasmaphoresis
              w/ Lung hemorrhage = Goodpasture’s Synd.
                                                       flattening,              n           Rupture Bowman’s                          Anti GBM GN w/
                                                       necrosis               Not diagnostic Capsule                                    ANCA + 
              Ag = alpha 3 chain in Type IV collagen Interstitium: inflam                                                               better prognosis
                                                       infiltrate, fibrosis
              History  flu-like illness
Type II:      20-30 y/o      Macroscopic               Focal                  Diffuse      Mesangial deposits                          70%  expected No specific Tx.
Immune        M>F (3-6 X)    hematuria (2-6            Segmental              Global                                                     life span         Control of HTN
                       Hydrocarbon exposure (glue)
Complex                      days)                     Rarely >30% of         IgA                                                      30% chronic
CGN         Low in           Microscopic                 glomeruli            IgG (30%)                                                  RF
(29%)       Association w/ HLA DR2
             African         hematuria (persists                              C3 (60%)                                                 50% 
             Americans       btw attacks)                                                                                                Recurrence
                                                                                                                           Henoch Schonlein Purpura: Systemic Manifestations
            US < Europe 30-35% :
IgA Nephro- 20-30 y/o                                           Factors predicting unfavourable outcome:                   - Palpable purpura – lower extremities & buttocks
pathy        & Australia       asymp
                                                                                                                           - Arthralgia of large joints
(Berger’s    < Japan            proteinuria
                                                                Clinical                    Histological                   - GI  colic & bleeding
Disease)                       micro hematuria
                                                                - Sustained HTN             - HTN vascular changes         - Renal involvement  adults  Acute Nephritic Synd.
            Higher Freq:       HTN
                                                                - Persistant Proteinuria    - Interstial Fibrosis                                   50%  nephritic syndrome
              HLA Bw35 30% :
                                                                - Macroscopic Hematuria     - Glomerulosclerosis                                     25%  progressive renal failure
              HLA DR4        acute nephritis
                              nephritic
                                                                - Nephrotic Syndrome        - Subendo/subepi deposits
                                syndrome
                              acute/chronic
                                    RF
                                   malignant HTN
Type III:                      No IgG or              Focal                 Weak            Disruption of:        Serum Creatinine ANCA+                   High dose:
Pauci-                           Complement           Segmental/global         (“pauci”)      GBM                   = best predictor of    pts.better      Corticosteriod
immune                           deposition           Fibrinoid necrosis    staining for      Bowman’s              outcome                response to tx   Cyclophosphamide
necrotizing                    ANCA +                 No Inc. # cells          Igs: (IgA,      Capsule
CGN                            2 forms:               Tubular lesions:         IgM, C3)                                                                    Plamaphoresis = no
(61%)                            Renal limited       Acute-necrosis,                                                                                        benefit (opposite to
                                 Component of          flattening                                    pANCA                       cANCA                      anti GBM GN)
  (Greater severity)
                                   Systemic           Chronic-atrophy,              found in:         neutrophils                 lysosomes in monocytes
                                   necrotizing          BM thickening               specific for:     Myeloperoxidase             Proteinase 3             .
                                   vasculitis         Interstitial lesions:         produces:         Perinuclear staining        Cytoplasmic staining
                                                      Acute-edema,
                                                        monocyte infiltra.                        ANCA + diseases:
                                                      Chronic-fibrosis
                                                      Vessels:
                                                      Acute-fibrinoid                             Wegener’s Granulomatosis – Lung, Kidney, Vasculitis
                                                        necrosis,                                 Chung-Strauss Synd. – Pauci-immune necrotizing CGN + Systemic sympts.
                                                        neutrophil/mono-                                   Asthma, Eosinophilia, Granulomatous lung disease
                                                        cyte infiltrate                           Microscopic PAN – Pauci-immune necrotizing CGN + small vessel vasculitis
                                                      Chronic-fibrosis                                                  Fibrinoid necrosis
                                                                                                           Different from Wegener’s  not granulomatous inflammation
    Lupus Nephritis                                                                                        Different from Chung-Strauss  no asthma

                    Effected   Pathogenesis           LM                    IF             EM                 Labs                     Prognosis          Therapy
Class II                       Type IIA = no Inc. #   Inc. mesangial       Igs             Mesangial deposits Proteinuria             90% 5YSR            Corticosteriods
(minimal /                       cells                  matrix             C3                                 Hematuria               1/5  severe
mesangial                      Type IIB = Inc. #                                                                                        disease
LN)                              cells
Class III                                             Focal                Igs             Subendothelial       Proteinuria       90% 5YSR         Corticosteriods
(Focal                                                < 50% glomeruli      (looks like       deposits- not      Hypocomple-       20%  Nephrotic
proliferative LN)                                                             mesangial)     mesangial            mentemia        transform  type
                                                                                                                                     IV
Class IV                       Most Severe /          Diffuse            Igs               Subendo-thelial    Proteinuria          75% 5YSR        Corticosteriods
(Diffuse                        Common                > 50% glomeruli    C3                Subepithelial      50% nephrotic range 5% 5YSR w/o tx. cyclophosphamide
proliferative LN)
                                                      Crescents                            Mesangial deposits 90% nephrotic
                               Active dx. =           Fibrinoid necrosis                                        syndrome
                                 Dec.                Inc. # cells                                            75% Renal insuff 
                                  Complement          BM = thick                                                  Inc. BUN
                                                                                                                  Inc. Cr.
                                 Inc. anti-DNA
                                                                                                                  Dec. GFR
                                 Circulating
                                  immune
                                  complexes
Class V                        Behaves like type II   Epimembranous        IgG             Subepitheial deposits Nephritic synd.      90% 5YSR            Corticosteriods
(Membranous                    Usually benign          spikes              C3              Effacement foot       Dec. anti-DNA Ab     transfor  type
LN)
                               15% of Lupus           No mesangial                           proccesses                               IV
nephritis    involvment
            No crescents
            BM = thick
Anomaly /         Pathology                                         Other Associated Lesions                     Clinical                      Prevalence /               Prognosis
Disease                                                                                                          Manifestations                Incidence
Agenesis of       There's no kidney! Can be unilateral or           Bilateral: Often results in Potter's         -                             -                          Bilateral: stillbirth
Kidney            bilateral. Results from failure of ureteric bud   sequence (agenesis oligohydramnios                                                                   Unilateral: remaining
                  to contact metanephric blastema. Adrenals of       limb & facial deformities and                                                                       kidney at risk for
                  affected sides show a nice disc shape.            pulmonary hypoplasia)                                                                                 progressive glomerular
                                                                    Unilateral: Opposite kidney undergoes                                                                 sclerosis.
                                                                    compensatory hypertrophy
Hypoplasia of     Reduced # (<6) of lobes. May be bilateral but     -                                            -                             -                          Bilateral disease usually
Kidney            unilateral more common. True hypoplasia                                                                                                                 results in renal failure in
                  due to developmental failure is rare - usually                                                                                                          infancy or early
                  its 2 to scarring. Oligomeganephronia –                                                                                                                childhood.
                  kidney is small but nephrons are
                  hypertrophied.
Ectopic Kidney    Kidney may lie above pelvic brim or even          Kinking of ureters                           -                             -                          Predisposed to infection
                  w/in pelvis. Usually normal in size.                                                                                                                    and stones.
Horseshoe         Fusion of upper / lower poles of kidneys -        -                                            Kidneys lie anterior to       Usually found at           Slight increase in
Kidney            often causing failure to ascend above the                                                      great vessels and may         autopsy (prevalence is     incidence of stones
                  IMA. Usually involves lower poles (90%).                                                       compress arteries - leading   1:500 to 1:1k)
                                                                                                                 to HTN
Cystic Renal      Enlarged kidney, cysts of varying sizes lined     Thought to be due to obstruction or          Bilateral: incompatible       Occurs sporadically –      Bilateral: Ultimately leads
Dysplasia         by flat epithelium. Characterized                 stenosis of urinary tract during                                           no familial clustering     to renal failure
(TypeII)          histologically by persistence of cartilage,       intrauterine life.                           w/ life, most common                                     Unilateral: Normal life
Fig. 21-5         undifferentiated mesenchyme, & immature           Bilateral disease also associated w/                                                                  span.
                  collecting ductules. May be bilateral /           Prune Belly syndrome (absence of
                  unilateral / segmental.                           abdominal musculature)
                                                                                                                 cause of renal failure in

                                                                                                                 newborn.

                                                                                                                 Unilateral: Incidental
                                                                                                                 finding
Autosomal         Bilateral occurrence of cysts in cortical &       Liver cysts – derived from biliary           1st clinical symptoms         Most common                10% of patients on
Dominant          medullary areas – gives lacy appearance.          epithelium & usually asymptomatic            usually appear in 30s or      defective allele on 16p.   maintenance dialysis
Polycystic        Cysts may arise from any level of nephron /       Berry aneurysms – death due to rupture       40s – often leads to ESKD     Most common genetic        Death may occur b/c of
Kidney Disease    collecting tubule. Often filled w/ fluid          in 4-10% of PKD cases                        ~10 yrs later.                disorder in humans.        intrapartum dystocia or
Fig. 21-8 a & b   resembling urine normally found @ that            Cysts in pancreas, spleen, lung              Abdominal pain                Mild cases may be          postnatal renal failure
                  segment. Lined w/ thin epithelium that often      Aortic aneurysms / dissection /              Enlargement of kidneys        confused w/ other
                  undergoes hyperplasia.                            coarctation, valvular abnormalities          Hematuria                     cystic diseases.
                                                                    Marfan’s synd., colon diverticulosis         HTN – 50% of cases

Autosomal         Bilateral involvement. Subcapsular surface        Portal fibrosis – often the cause of death   Renal failure occurs w/       1:6k –14k live births      Spectrum of expression of
Recessive         studded w/ 1-2mm cysts that are formed from       when small % nephrons involved.              anemia, osteodystrophy,       Each offspring has 25%     disease – depends on %
Polycystic        fusiform radiation of collecting ducts from       Bile ducts increased in # and are often      growth retardation &          chance of symptomatic      nephrons involved.
Kidney Disease    medulla  cortex. Cysts lined w/ cuboidal         tortuous, but not dilated.                   failure 2 thrive.             disease                    Generally, the milder the
Fig 21-8 c        cells. Glomerular obsolescence, tubular           Free communication w/ extra-hepatic          Presentation later in life                               kidney disease, the more
                  atrophy, and interstitial fibrosis due to         bile ducts                                   involves renomegaly and                                  severe the liver fibrosis.
                  compression.                                                                                   hepato(spleno)megaly.

Simple Cysts      Single / Multiple. Cortex / Medulla.              Complicated simple cysts – contain           Microscopic hematuria         Present in 50% of          Hemorrhagic contents,
                   Spherical, tense, thin-walled and contain      clotted or hemolyzed blood. Capsule                                  pts>50 at autopsy       papillary projections,
                   fluid w/ features of plasma ultrafiltrate.     may be thickened by fibrous tissue.                                                          multicellular layers may
                                                                                                                                                               indicate cystic carcinoma
                   Lined w/ low cuboidal  attenuated
                   flattened epithelium




Medullary          Intrapyramidal cysts with calcerous deposits   Associated w/ :                       Hematuria, stones,             1:5k-20k general pop.   Uncomplicated cases
Sponge Kidney      (50%). Lined by cuboidal or transitional       Ehlers Danlos syndrome, horseshoe     recurrent UTI, Proteinuria     More common in adults   follow a benign course
Fig. 21-9          epithelium. Accompanied by infiltration of     kidney, ectopic kidney, mongolism     in 50%, Hyperuricemia,         Congenital, but not
                   interstitium w/ inflammatory cells – most                                            hypercalciuria, mild salt-     heritable
                   pronounced near pelvis.                                                              wasting, distal tubular
                                                                                                        acidosis
Nephronophthisis   Kidney reduced in size, involves both cortex   -                                     Nocturia,                      Rare – 4 varieties      No recurrence in
(Uremic            & medulla – CM junction indistinct.                                                  polyuria,polydipsia,           1.Familial juvenile     transplant
Medullary          Granular surface. Thin-walled, spherical                                             enuresis, pallor, disordered   nephronophthisis -50%
Disease            cysts from 1-10 mm containing watery,                                                bone growth.                   Autosomal recessive
Complex)           straw-colored fluid. Arrayed along CM                                                HTN in 30%                     2.Renal-retinal
                   junction. Small cysts, resembling retention                                          Red/Blond hair                 dysplasia –15%
                   cysts, seen in cortex. Tubular atrophy, nild                                         Renal salt-wasting             Autosomal recessive
                   mononuclear infiltration, & interstitial                                             syndrome                       Assoc. w/ retinitis
                   fibrosis.                                                                            Progressive renal failure      pigmentosa
                                                                                                        Usually no                     3.Adult onset
                                                                                                        nephrocalcinosis or            medullary cystic
                                                                                                        urolithiasis                   disease –15%
                                                                                                                                       Autosomal dominant
                                                                                                                                       4. Sporadic non-
                                                                                                                                       familial –20%
                                                                                                                                       Dominant transmission

				
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