Medical kidney diseases

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Micrograph showing a glomerulus with an acute thrombotic microangiopathy. PAS stain.

This article describes medical renal disease or the medical kidney. Much in medical kidney depends on the clinical information. Most of the disease seen by pathologists is... glomerular disease. It is one of the few areas that routinely requires electron microscopy.

Medical kidney is niche area in pathology. In the context of exams for general pathologists, if in doubt... the answer to most questions is diabetes mellitus or systemic lupus erythematosus.

Kidney tumours are dealt with in the kidney tumours article, and pediatric kidney tumours article.

Clinical

Creatinine

  • The standard screening test for renal function.
  • 300 mmol/L is the general cut-point for referral to a nephrologist.[1]

Notes:

  • In some parts of the world they use the units mg/dL; normal with these units is: 0.8 to 1.4 mg/dL.[2]
  • Conversion: 1.0 mg/dL = 88.4 umol/L.[3][4]

Glomerular filtration rate

  • Abbreviated GFR.
  • Ultimate measure of renal function - usually estimated from the serum creatinine using a formula.
  • Declines with age.
  • Normal range (dependent on age): 116-75 mL/min/1.73m2.[5]

Urine protein to creatinine ratio

  • Indicator of proteinuria.
  • Predictor of glomerular filtration rate.[6]

Cut points:[7]

  • Normal (2 years and older): <0.2 g protein / g Creatinine
  • Nephrotic range: >3.5 g protein / g Creatinine.

Complement

C3, C4 levels:[8]

ANCA

Types:[12]

C4d

  • Suggests humoral immunity (antibody-mediated immunity) at play.
  • Important in monitoring of renal transplant recipients.
  • Immunostain also available - see below.

Other blood work

Renal ultrasound

  • Normal adult kidney size ~10.8+/-0.8 cm.[13]
  • Good for assessing the major vessels, drainage system and parenchymal lesions.
    • Renal artery stenosis?
    • Hydronephrosis?
    • Pelviectasis?
    • Renal cyst?
    • Renal mass?

Urine dip

Findings:[14]

  • RBC casts = acute bleed, e.g. nephritic syndrome.
  • WBC casts = interstitial nephritis, e.g. pylonephritis, parenchymal infection.
  • Hemegranular casts = acute tubular necrosis, transplant rejection.

Notes:

  • "Active sediment" = RBCs, RBC casts;[15] implies glomerulonephritis.
    • Some include the above (RBCs, RBC casts) + WBCs & protein.[16]

Urine crystals

Main article: Crystals in body fluids

Clinical presentations

Nephrotic syndrome

Features:

  • Anasarca (whole body - edema).
  • Proteinuria (>3.5 g/24h).
  • Hypercholesterolemia.
  • Hypoalbuminemia.

Nephritic syndrome

Features - mnemonic PHAROH:[17]

  • Proteinuria.
  • Hypertension.
  • Azotemia.
  • RBC casts.
  • Oliguria.
  • Hematuria.

Mixed

Clinical DDx

Renal failure 101

  • Prerenal.
    • Shock - cardiogenic, hypovolemic, neurogenic, septic, anaphylatic.
  • Renal.
    • What this article deals with.
  • Postrenal.

Renal causes

  • Isolated hematuria.
  • Isolated proteinuria.
  • Nephrotic syndrome.
  • Nephritis syndrome.
  • Rapidly progressive glomerulonephritis.
  • Chronic renal failure.
  • A combination of any of the above.

The clinical presentations suggest a pathologic DDx.[18]

Isolated hematuria

Proteinuria

Nephritic syndrome

Classic:

Others:

Rapidly progressive glomerulonephritis

Classification:[20]

  1. Linear immune deposits.
  2. Granular immune deposits.
  3. Pauci-immune.

Nephrotic syndrome

Mixed presentation

Normal kidney

Cells of the glomerulus

  • Podocytes.
  • Mesangial cells.
  • Endothelium.

Epithelium

Features:[21]

  • The glomeruli visceral epithelium is part of the capillary wall (part of the glomerular tuft).
  • The parietal epithelium is part of Bowman's capsule.

Remember: visceral has vessels.

Glomerulus

  • A large autopsy series suggest it is: 223-292 micrometers.[22]
    • Another autopsy series suggests the mean diameter is: 225 micrometers.[23]

Note:

  • If the glomerulus fills half the field at 40x on a microscope with 22 mm diameter eye pieces it is ~ 0.275 mm or 275 micrometers.

Glomerular basement membrane

The glomerular basement membrane (GBM) should be thinner than the tubular basement membrane.

Images

  • Benign kidney - very low mag.

  • Benign kidney - low mag.

  • Benign kidney - low mag.

  • Benign kidney - intermed. mag.

Basic approach to renal biopsy

Basic components

  • Glomeruli.
  • Tubules.
  • Interstitium.
  • Vessels.

Memory device GI TV: glomeruli, interstitium, tubules, vessels.

Glomeruli

  1. Mesangium
    • Matrix should be: "one cell thick" (expanded in diabetes mellitus).
    • Cellularity of the mesangium - normal = upto 3 cells (don't count cell abutting the capillary lumen, don't count at the hilum).
  2. Capillary loops "open"
    • Lumina patent? If not patent is it due to matrix or cells (endocapillary hypercellularity).
    • Capillary wall morphology - wavy thin is normal; hulla-hoop/wire-like abnormal (suggestive of immune complex deposition).
  3. Bowman's space (urinary space) - crescents present?
  • Count the number of glomeruli.
  • Count number of the obsolete glomeruli.

Notes:

Components of the glomeruli (anatomical)

Obsolete glomeruli

  • Completely sclerosed glomeruli are not important - unless present in larger numbers than expected for the age of the patient.
Percent of sclerosed glomeruli = (age in years)/2 - 10%.[27]

Example:

  • It is normal for an 80 year-old to have 30% sclerosed glomeruli.

Glomerular disease terms

Number of glomeruli involved:[28]

  • Focal = some of the glomeruli.
    • In practical terms, defined as: <50% of glomeruli.
  • Diffuse = most of glomeruli.

How much of the glomerulus is involved:[28]

  • Global = most of the glomerulus.
    • In practical terms, defined as: >80% of glomerulus.[29]
  • Segmental = part of the glomerulus.

Vessels

  1. Arteriolar hyalinosis - too much glassy pink stuff?
  2. Intimal hyperplasia (atherosclerosis).

Consider:

Arteriolar hyalinosis

Microscopic:

  • Small vessels (afferent +/- efferent arteriole) with:
    • Glassy eosinophilic material in arteriolar wall.

DDx:

Note:

  • Arteriolar hyalinosis - involves afferent and efferent arterioles in diabetes, in others it is only the afferent.

Memory device ADHD:

  • Aging, Diabetes, Hypertension, Drugs.
Image

  • Arterial hyaline - HE - very high mag. (WC/Nephron)

  • Arterial hyaline - PAS - very high mag. (WC/Nephron)

  • Arterial hyaline - HPS - very high mag. (WC/Nephron)

Atherosclerosis

Main article: Atherosclerosis

Microscopic:

  • Intimal thickening of medium-sized vessels.
    • Where is the intima/media interface?
      • Internal elastic lamina - wavy band of eosinophilic material on H&E that is 1-2 micrometres thick.

Grading - based on the thickness of the media and intima:

  • Mild: (tunica) media > (tunica) intima.
  • Moderate: media = intima.
  • Severe: media < intima.

Tubules

Tubules - proximal portion is the most important.

Consider:

  • Casts?
  • Degeneration, i.e. apoptosis.
  • Necrosis?
  • Regeneration, e.g. mitoses.

Interstitium

Interstitium

  • Fibrosis - prognostically important.
    • Grading: mild = <25%, moderate 25-50%, severe >50%.
  • Inflammation?

Important terms/process related

Staining

The standard stain in kidney pathology is PAS. Section are usually 1-2 micrometers, as opposed to 4-5 micrometers seen in rountine section of other organs.

Interpretation of medical renal disease more difficult or even impossible if the sections are thicker, as one does not see the glomerular structures well.

In kidney that is cut thick the glomeruli look more nodular and it is more difficult to find open capillary loops.

Immunofluorescence

Routinue (mnemonic GAM CF):

  • IgG.
  • IgA.
  • IgM.
  • C1q
  • C3.
  • Fibrinogen.
  • Albumin.

Optional:

  • Kappa.
  • Lambda.
  • C4d.
    • Positive staining = peri-tubular capillaries stain.

Negative immunofluorescence

  • Excludes all immune complex associated disease.

Seen in:

Positive immunofluorescence

  • Positive immunofluorescence is usually diagnostic.

Basic patterns:

  1. Linear.
  2. Granular.
  3. Ring-like.

Examples:

Notes:

Immune complex-related disease

Can be:

Pathologic differential diagnosis

Tram-tracking of BM

DDx:[30]

  1. MPGN.
  2. Thrombotic microangiopathy (TMA).
  3. Transplant glomerulopathy (TG).

Mesangial hypercellularity

DDx:

  1. Lupus nephritis.
  2. IgA nephropathy.

Mesangial expansion

  • A patch of matrix can contain more than three mesangial cell nuclei.
    • Highly subjective.

DDx:

Glomerular crescents

General

  • Indicates a rapidly progressive disease.
  • Etiology/definition: break in the glomerular basement membrane (GBM).

Microscopic

Features:

  • Crescentic-shaped lesion in the urinary space of a glomerulus.
    • Crescent = looks like the moon shortly after new moon.
    • Cells & cellular debris in the urinary space.
      • Crescents lack GBM -- thus PAS -ve and MPAS -ve.
  • Break in the glomerular basement membrane - key feature.
  • Fibrin.
  • Inflammatory cells (lymphocytes, plasma cells, eosinophils, macrophages) - extravascular - low power feature.

DDx:

  • Glomerular sclerosis:
    • GBM visible through-out glomerulus.
    • No fibrin.
    • Collagen deposition within the glomerular tuft.
    • +/-Glomerular tuft-capsule adhesions.
    • +/-Glomerular enlargement.
    • Usu. no significant inflammation.

Bland necrotic crescents

DDx:

  • ANCA-related glomerulonephritis.
  • Anti-GBM disease.

Diseases with crescents - is a long list.[32]

Primary light microscopy patterns

Pattern Key feature Other findings DDx of the pattern DDx Image
Normal light microscopy Normal histology none or few glomeruli in a biopsy of an under sampled FSGS membranous nephropathy, focal segmental glomerulosclerosis (FSGS) normal kidney, minimal change disease, thin glomerular basement membrane disease, Alport syndrome, early membranous nephropathy
Benign kidney (WC)
Nodular mesangial expansion nodular mesangial matrix expansion, no mesangial hypercellularity GBM thickening, both afferent and efferent arteriole hyalinized diffuse proliferative GN, membranous nephropathy diabetic nephropathy, idiopathic nodular glomerulosclerosis, renal amyloidosis, light chain deposition disease, fibrillary glomerulopathy, immunotactoid glomerulopathy
Nodular GS (WC)
Membranous nephropathy (MN) GBM spikes or pinholes with silver stain, GBM thickening on PAS +/-mesangial hypercellularity +/-tram-tracking/wireloop GBM normal light microscopy, light chain deposition disease hepatitis B, hepatitis C, carcinoma, NSAID toxicity, SLE, idiopathic (MN); light chain deposition disease (plasma cell neoplasm)
MN (WC)
Focal segmental glomerulosclerosis sclerosed glomeruli = mesangial expansion with collagen +/-glomerular enlargement, +/- tuft-capsule adhesions rapidly progressive glomerulonephritis, nodular mesangial expansion primary FSGS, secondary FSGS (HIV, IVDU, obesity, parvovirus B19, Alport syndrome
FSGS (WC)
Diffuse proliferative glomerulonephritis mesangial hypercellularity +/-mesangial expansion, +/-interstitial inflammation nodular glomerulosclerosis post-infectious glomerulonephritis, MPGN, dense deposit disease, diffuse proliferative lupus nephritis (class IV), cryoglobulinemic GN
MPGN (WC)
Rapidly progressive glomerulonephritis glomerular crescents +/-inflammation, +/-vasculitis FSGS, diffuse proliferative glomerulonephritis AGBM, post-infectious glomerulonephritis, pauci-immune GN, immune complex diseases (lupus nephritis, IgA nephropathy, others)
Crescentic GN (WC)
Tubular injury tubular degeneration (loss of cilia in proximal tubule, apoptosis, necrosis), regeneration (mitosis, nucleoli) +/-interstitial fibrosis normal light microscopy acute tubular necrosis, chronic allograft nephropathy
CAN (WC)
Small vessel pathology luminal narrowing; +/-thrombosis, +/-hyalinosis +/-onion-skinning none thrombotic microangiopathy (malignant hypertension, scleroderma renal crisis, DIC, HUS, TTP,
Hyalinosis (WC)
TMA (WC)
Large vessel pathology luminal narrowing or obstruction; +/-intimal thickening; +/-embolization +/-medial thinning none atherosclerosis, cholesterol embolism, other types of embolism, thrombosis
Cholesterol embolus (WC)

Diagnoses - Table

Pattern Key feature Other findings IF & EM Presentation Clinical Pathol. DDx Image
Nodular glomerulosclerosis nodular mesangial matrix expansion GBM thickening, both afferent and efferent arteriole hyalinized EM? proteinuria - typical presentation, nephrotic syndrome[citation needed] diabetes mellitus (DM) amyloidosis, idiopathic nodular glomerulosclerosis (nodular GS without DM)
Nodular GS (WC)
Focal segmental glomerulosclerosis (FSGS) focal sclerosis of gloms +/-interstitial fibrosis IF: negative; EM: foot process loss nephrotic syndrome primary FSGS, secondary FSGS (HIV, IVDU, obesity, parvovirus B19, Alport syndrome); unresponsive to steroids, worse prognosis than MCD minimal change disease
FSGS (WC)
Membranous nephropathy
(AKA membranous GN) 		spikes or pinholes with silver stain mesangial hypercellularity; +/-tram-tracking/wireloop GBM IF: diffuse granular capillary loop IgG, C3, kappa, lambda; EM: diffuse subepithelial deposits - spike forming nephrotic syndrome hepatitis B, hepatitis C, carcinoma, NSAID toxicity, SLE, idiopathic Nodular glomerulosclerosis (?)
MN (WC)
Minimal change disease (MCD) foot process loss on EM usu. none EM: foot process loss nephrotic syndrome primary vs. secondary (lymphoproliferative disorder, NSAIDs); idiopathic responds to steroids FSGS, thin glomerular basement membrane disease (histologic DDx) Image?
IgA nephropathy IgA branching pattern +/-mesangial hypercellularity, +/-crescents IF: IgA +ve (branching pattern); EM: dense mesangial deposits mixed nephrotic/nephritic primary vs. secondary (Henoch-Schoenlein purpura) RPGN
HSN - IgA IHC (WC)
Membranoproliferative glomerulonephritis (MPGN) thick GBM Other findings? subepithelial deposits mixed nephrotic/nephritic SLE, cryoglobulinemia, hepatitis B, hepatitis C Pathol. DDx?
MPGN (WC)
Focal proliferative
glomerosclerosis (FPGS) 		<50% of glomeruli partially sclerosis Other findings? EM? mixed nephrotic/nephritic Clinical? Pathol. DDx? Image?
Rapidly progressive GN (RPGN) crescents Other findings? EM? nephritic syndrome AGBM, ANCA-vasculitis IgA nephropathy with crescents Image?
Dense deposit disease IF: linear C3 with rings +/-thick GBM EM: GBM lamina densa thickening Presentation? mixed nephrotic/nephritic (???) MPGN (nature.com)

Diffuse proliferative glomerulonephritis

Pattern Key feature Clinical
Post-infectious glomerulonephritis IF: capillary loop +/- mesangial IgG/C3; EM: large infreq. hump-like subepithelial deposits post-infection
Membranoproliferative glomerulonephritis (MPGN) mesangial proliferation with thickening of the glomerular capillary loops[33] low C3, normal C4; primary vs. secondary (often hepatitis C)
Dense deposit disease IF: linear C3 with rings
Cryoglobulinemic glomerulonephritis
Diffuse proliferative lupus glomerulonephritis systemic lupus erythematosus; low C3, low C4
Diffuse proliferative IgA nephropathy IF: IgA +ve (branching pattern)

Common diseases

Diabetic nephropathy

General

Microscopic

Features:[34]

  • Thick glomerular basement membrane (GBM).
  • Thickened (eosinophilic) tunica media in both the afferent and efferent arterioles.[35]
  • Mesangial matrix expansion - leads to nodule formation Kimmelstiel-Wilson nodules (nodular glomerulosclerosis).

Others:

  • Armanni-Ebstein change = cytoplasmic vacuolization of tubular cells (usu. loop of Henle) -- innermost cortex, outer medulla;[36] not specific to diabetes mellitus.[37]
  • Capsular drop = hyaline material deposited in the Bowman's capsule.[38]
  • Fibrin cap = subendothelial deposition of hyaline material.[38]

Other - with weak evidence:

  • Extra efferent vessels.[39]

Memory device:

  • GBM = thick GBM, both afferent & efferent arterioles thickened, mesangial matrix expansion.

Images

  • Diabetic glomerulosclerosis. (WC/KGH)

  • Diabetic glomerulosclerosis. (WC/KGH)

  • Diabetic glomerulosclerosis. (WC/KGH)

  • Nodular glomerulosclerosis. (WC)

www:

Notes:

  • Hypertensive kidneys have changes only in the afferent arteriole, i.e. the efferent arteriole is spared (see hypertension).

Grading

The severity of changes can be indicated by the grade:[40]

Grade Criteria
Grade I GBM thickening on EM only
Grade II Mild-to-moderate mesangial expansion only on LM
Grade III Glomerulus with mesangial nodules (Kimmelstiel–Wilson nodules)
Grade IV Stage III + global sclerosis in >50% of glomeruli

IF

  • Negative.
  • +/-Nonspecific linear IgG.

EM

  • Severe thickening of GBM.
  • Mesangial sclerosis.

Lupus nephritis

  • Abbreviated LN.

General

Main article: Systemic lupus erythematosus
  • Bread & butter of nephropathology.
  • The biopsy is done to determine treatment, i.e. how much immunosuppression is needed.

Immunofluorescence

  • "Full house" = all of 'em light up.

Classification

International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification:[41][42]

  • Class I - minimal mesangial LN.
  • Class II - mesangial proliferative LN.
    • Mesangial hypercellularity. (???)
  • Class III - focal lupus nephritis; <50% of glomeruli.
    • Mesangial hypercellularity. (???)
  • Class VI-S - diffuse segmental LN; >50% of glomeruli.
    • Mesangial hypercellularity. (???)
  • Class VI-G - global LN; >50% of glomeruli.
  • Class V - Membranous lupus nephritis.
  • Class IV - Advanced sclerosing LN; essentially end-stage kidney.

Notes:

  • Most of the action is in Class III and Class IV.
    • Class I is near normal - doesn't get biopsied.
    • Class IV is essentially a dead kidney - doesn't get biopsied.

Images

  • SLE nephritis - schematic. (WC)

Nephrotic syndrome

Main article: Nephrotic syndrome

This classically includes the following:

It also includes:

Mixed nephrotic and nephritic

IgA nephropathy

General

Microscopic

Features:

  • Variable:
    • Mesangial hypercellularity - may be only light microscopy finding.

Note:

  • Diagnosis based on immunofluorescence (IgA+).

Images

  • HSP - IgA IHC (WC)

Scoring

IgA nephropathy can be scored using an assessment of mesangial proliferation, endocapillary proliferation, glomerulosclerosis and tubular atrophy and interstitial fibrosis (abbreviated MEST).[47]

IF

  • IgA +ve in a branching pattern - diagnostic.

EM

  • Mesangial deposits.
    • These are electron dense, ergo dark on EM images.

Membranoproliferative glomerulonephritis

  • Abbreviated MPGN.
  • Old name MPGN type 1.

General

Clinical:

  • Nephrotic syndrome or nephrotic/nephritic syndrome.

Pathology:

  • May be primary, i.e. idiopathic, or secondary, i.e. a consequence of another pathologic process.[33]

Secondary causes

Other causes:

Microscopic

Features:

  • Endothelial cell proliferation.
  • Basement membrane double layering (tram-tracking).
  • Mesangial hypercellularity.

DDx:

Images

  • MPGN - intermed. mag. (WC/Nephron)

  • MPGN - very high mag. (WC/Nephron)

EM

Features:

  • Subendothelial immune deposits - classic finding.[49]
  • Subepithelial immune deposits - historically considered uncommon.[50]

Dense deposit disease

  • Abbreviated DDD.
  • AKA MPGN type 2 (old name).

General

  • Usually children and young adults.
  • No longer considered a type of MPGN.[51]

Microscopic

Features:

  • Variable - may be like MPGN.
    • Four patterns:[51]
      1. Hypercellularity and lobular (membranoproliferative-like).
      2. Mesangial proliferative.
      3. Crescentic.
      4. Acute proliferative and exudative.

Images:

IF

  • Linear C3 with mesangial rings (donut-like).
  • IgG negative.
  • IgA negative

Images:

EM

  • Electron dense transformation of GBM lamina densa - key feature.
    • Dense = darker.

Images:

Nephritic syndrome

Rapidly progressive glomerulonephritis

  • Abbreviated RPGN.
  • AKA crescentic glomerulonephritis.

General

  • Acute renal dysfunction.
  • Nephritic syndrome.

DDx:

  1. Linear immunofluorescence.
  2. Granular immunofluorescence
  3. Pauci-immune.

Microscopic

Features:

Images

  • RPGN (WC/KGH)

  • RPGN (WC/KGH)

  • RPGN - intermed. mag. (WC/Nephron)

  • RPGN - high mag. (WC/Nephron)

Post-infectious glomerulonephritis

  • Abbreviated PIGN.
Main article: Post-infectious glomerulonephritis

Rare diseases

Antiglomerular basement membrane disease

  • Abbreviated AGBM.

General

  • Known as Goodpasture disease (AKA Goodpasture syndrome), if renal failure is accompanied by pulmonary hemorrhage.[52]
  • Rare - estimated incidence 1/1-2 million.[53]
  • Antibody mediated hypersensitivity.
  • Thought to occur in genetically susceptible individuals.[53]
    • Associated with a specific HLA type (HLA-DRB1*1501) and two gene families.
    • Antibodies directed against COL4A3,[54] which is not mutated.
  • Oligouria - poor prognosticator.[citation needed]

Tx:

  • Immune suppression & plasma exchange.[55]

Clinical DDx:

Microscopic

Features:

  • RPGN.
    • Crescentic glomerulonephritis.

Images

  • RPGN - intermed. mag. (WC/Nephron)

  • RPGN - high mag. (WC/Nephron)

www:

IF

  • Linear IgG deposits - diagnostic.
  • Fibrinogen in crescents.

DDx:

  • Goodpasture syndrome with the pulmonary hemorrhage.

Image:

Thin glomerular basement membrane disease

General

Clinical:

  • Hematuria.
  • FHx.
  • Nonprogressive.

Microscopic

  • Normal.

IF

  • Normal.

EM

  • GBM thin <200-250 - key feature.

Note:

  • Normal GBM: 300-350 nm.

Alport syndrome

General

Clinical:

  • Hearing loss (sensorineural).
  • Hematuria - usually preceeds hearing loss.[57]

Etiology:

  • Genetic defect - collagen type IV.

Inheritance:[57]

  • X-linked - 80%.
  • Autosomal recessive - 15%.
  • Autosomal dominant - 5%.

Microscopic

Features:[59]

  • Usu. normal.
  • +/-Foamy appearing renal tubular cells.[60]

IF

  • Negative.

EM

Features:[59]

  • Abnormal glomerular basement membrane (GBM); thinning or thickening.
    • Classically thinning with thick lamellation (splitting/multi-layering).

Idiopathic nodular glomerulosclerosis

General

Associations:[61]

Microscopic

Features:[61]

  • Looks like diabetic nodular glomerulosclerosis.

IF

Nonspecific.

EM

Nonspecific.

Fabry disease

Main article: Fabry disease

Myeloma

See: Haematopathology.
  • AKA myeloma kidney.

Myeloma cast nephropathy

General

  • Renal failure.

Microscopic

Features:[63]

  • Crap in tubules, eosinophilic.
    • Classically angulated.
    • Refractile.
  • Cast with cellular reaction - virtually diagnostic.
    • Macrophages (CD68 +ve).
Images

www:

  • Myeloma cast nephropathy - high mag. (WC/Nephron)

  • Myeloma cast nephropathy - cropped - very high mag. (WC/Nephron)

Stains

  • Myeloma casts = PAS -ve.
    • Hyaline casts = PAS +ve.

Amyloidosis

Main article: Amyloidosis
  • Usually associated with lambda clone.

Light chain deposition

  • Usually associated with kappa clone.

Immunotactoid glomerulopathy

General

Presentation:

Microscopic

Features:

  • Mesangial expansion.

DDx:

  • Other causes of mesangial expansion.

IHC

Fibrillary glomerulonephritis

  • AKA fibrillary glomerulopathy.

General

  • Rare ~ 1% native kidney biopsies.[64]
  • Presents as nephrotic syndrome.
  • Prognosis poor; large number progress to ESRD.

Microscopic

Features:

  • Mesangial matrix expansion.
  • Thickened capillary walls.

Images:

IF

  • IgG predominant.[64]

Stains

EM

  • Fibrils:[64]
    • Non-branching.
    • Randomly arranged.
    • Usu. 18-20 nm in diameter -- larger than amyloid (see below).
      • Size range 12-24 nm.

Note:

  • Amyloid between 8 and 15 nm (diameter), most often 8-12 nm.[64]

Aristolochic acid nephropathy

General

  • Nephropathy due to chinese herbs for slimming.[68]
  • Associated with urothelial atypia/urothelial carcinoma.

Clinical:

  • Chinese herb use.
  • Low-molecular weight proteinuria.

Microscopic

Features:[68]

  • Extensive interstitial fibrosis.
  • Tubular atrophy - typically greater in outer cortex than inner cortex.
  • Glomeruli spared.
  • +/-Nuclear atypia of the urothelium.

DDx:

  • Balkan endemic nephropathy.

Image:

Cystic kidney diseases

Main article: Cystic kidney diseases

These are discussed in a separate article and include:

Pyelonephritis

Pyelonephritis is a misnomer; it is not an inflammatory process affecting the renal pelvis, as the name suggests. Pyelonephritis actually refers to a nephritis or tubulointerstitial nephritis.[69]

It can be subdivided into:

Acute pyelonephritis

  • AKA acute infectious tubulointerstitial nephritis.
  • AKA diffuse suppurative nephritis.

General

  • Typically preceeded by a (lower) urinary tract infection (UTI).
  • Usually diagnosed clinically:
    • Urine C&S, urine R&M, +/-CT abdomen.
    • Fever, costovertebral tenderness.

Gross

Features:

Microscopic

Features:

Images

  • Acute pyelonephritis - intermed. mag. (WC/Nephron)

  • Acute pyelonephritis - high mag. (WC/Nephron)

  • Acute pyelonephritis - very high mag. (WC/Nephron)

Chronic pyelonephritis

  • Reflux nephropathy is considered synonym in some sources.[72]

General

  • Inflammation of the kidney (nephritis) and renal pelvis (pyelo-[73]).
  • May be associated with vesicoureteral reflux.
  • Chronic pyelonephritis may be a reason for nephrectomy.[74]

Gross

Microscopic

Features:[75]

  • Mononuclear tubulointerstitial infiltrate.
    • Usu. more dense at the pelvis.
  • Interstitial fibrosis.
  • +/-Renal casts (PAS positive); may result in a thyroid-like apparance.

DDx:

Stains

  • PAS +ve -- renal tubular casts.

Disease that does not commonly get biopsied

End-stage kidney

Main article: End-stage kidney

Malignant hypertension

See: hyperplastic arteriolosclerosis.
See: thrombotic microangiopathy.

Acute tubular necrosis

  • Often abbreviated ATN.

General

Diagnosed clinically:

  • Using urine R&M - hemegranular casts[76] are diagnostic.
  • Anuria or low urine output.

Gross

  • Poorly defined corticomedullary junction - soft finding.
  • Slightly heavier ~ 180 grams.[77]

Microscopic

Features:[77]

  • Tubular epithelial whorls - present in approx. one third of cases - most important.
    • Detached epithelium within the luminal space surrounded by epithelium.
      • Similar to epithelial telescoping seen in endometrial biopsies.
  • Tubulorrhexis - present in approx. one third of cases.
    • Disruption of the tubular basement membrane.
  • Interstitial edema - sensitive... but not specific.

Notes - not particularily useful findings:

  • Mitoses.
  • Casts in tubules.

IHC

  • Ki-67 - focal nuclear staining of the tubular epithelium.[77]

Hepatorenal syndrome

General

  • Acute renal failure secondary to liver failure (e.g. fulminant liver failure, cirrhosis with marginal liver function).

Clinical:

  • Urine sodium is low,[78] unlike in ATN (the main DDx).

Pathophysiology:

  • Renal vasoconstriction.[79]

Treatment: Medical and surgical:[80]

  • Vasoconstrictors (e.g. midodrine, terlipressin (counteracts splanchnic vasodilation), norepinephrine).
  • Albumin.
  • TIPS (transjugular intrahepatic portosystemic shunt).
  • Liver transplantation.

Note:

  • I suspect a portal vein pump would work... it reduces portal pressure and would likely increase hepatic function.

Microscopic

Features (kidney):

  • Normal.

Nephrocalcinosis

General

Microscopic

Features:

  • Calcification of the renal parenchyma - key feature.

Images:

Renal transplant pathology

Main article: Renal transplant pathology

See also

References

  1. ↑ Mendelssohn DC, Barrett BJ, Brownscombe LM, et al. (August 1999). "Elevated levels of serum creatinine: recommendations for management and referral". CMAJ 161 (4): 413–7. PMC 1230545. PMID 10478168. http://www.cmaj.ca/cgi/content/full/161/4/413.
  2. ↑ URL: http://www.nlm.nih.gov/medlineplus/ency/article/003475.htm. Accessed on: 8 November 2010.
  3. ↑ URL: http://www.sydpath.stvincents.com.au/other/Conversions/ConversionMasterF3.htm. Accessed on: 8 November 2010.
  4. ↑ URL: http://www.unc.edu/~rowlett/units/scales/clinical_data.html. Accessed on: 8 November 2010.
  5. ↑ URL: http://www.kidney.org/professionals/KLS/gfr.cfm. Accessed on: 8 November 2010.
  6. ↑ Ruggenenti P, Gaspari F, Perna A, Remuzzi G (February 1998). "Cross sectional longitudinal study of spot morning urine protein:creatinine ratio, 24 hour urine protein excretion rate, glomerular filtration rate, and end stage renal failure in chronic renal disease in patients without diabetes". BMJ 316 (7130): 504–9. PMC 2665663. PMID 9501711. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2665663/pdf/9501711.pdf.
  7. ↑ URL: http://www.fpnotebook.com/urology/lab/urnprtntcrtnrt.htm. Accessed on: 8 November 2010.
  8. ↑ Levo Y, Pick AI (1974). "The significance of C3 and C4 complement levels in lupus nephritis". Int Urol Nephrol 6 (3-4): 233–8. PMID 4549215. http://www.springerlink.com/content/l1657797661468g1/fulltext.pdf.
  9. ↑ 9.0 9.1 Nusinow SR, Zuraw BL, Curd JG (May 1985). "The hereditary and acquired deficiencies of complement". Med. Clin. North Am. 69 (3): 487–504. PMID 3892188.
  10. ↑ URL: beckmancoulter.com. Accessed on: 9 November 2010.
  11. ↑ URL: beckmancoulter.com. Accessed on: 9 November 2010.
  12. ↑ Kallenberg, CG. (Mar 2011). "Pathogenesis of ANCA-associated vasculitides.". Ann Rheum Dis 70 Suppl 1: i59-63. doi:10.1136/ard.2010.138024. PMID 21339221.
  13. ↑ Guzman, RP.; Zierler, RE.; Isaacson, JA.; Bergelin, RO.; Strandness, DE. (Mar 1994). "Renal atrophy and arterial stenosis. A prospective study with duplex ultrasound.". Hypertension 23 (3): 346-50. PMID 8125561.
  14. ↑ URL: http://www.nlm.nih.gov/medlineplus/ency/article/003586.htm. Accessed on: 20 September 2010.
  15. ↑ URL: http://emedicine.medscape.com/article/238158-overview. Accessed on: 9 November 2010.
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  19. ↑ 19.0 19.1 Kim, BS.; Kim, YK.; Shin, YS.; Kim, YO.; Song, HC.; Kim, YS.; Choi, EJ. (Dec 2009). "Natural history and renal pathology in patients with isolated microscopic hematuria.". Korean J Intern Med 24 (4): 356-61. doi:10.3904/kjim.2009.24.4.356. PMID 19949735.
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  22. ↑ Hoy, WE.; Douglas-Denton, RN.; Hughson, MD.; Cass, A.; Johnson, K.; Bertram, JF. (Feb 2003). "A stereological study of glomerular number and volume: preliminary findings in a multiracial study of kidneys at autopsy.". Kidney Int Suppl (83): S31-7. PMID 12864872. http://www.nature.com/ki/journal/v63/n83s/fig_tab/4493733t2.html#figure-title.
  23. ↑ Nyengaard, JR.; Bendtsen, TF. (Feb 1992). "Glomerular number and size in relation to age, kidney weight, and body surface in normal man.". Anat Rec 232 (2): 194-201. doi:10.1002/ar.1092320205. PMID 1546799.
  24. ↑ Grcevska, L.; Ristovska, V.; Nikolov, V.; Petrusevska, G.; Milovanceva-Popovska, M.; Polenakovic, M. (Dec 2010). "The Oxford classification of IgA nephropathy: single centre experience.". Prilozi 31 (2): 7-16. PMID 21258273.
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  26. ↑ AH. 13 August 2009.
  27. ↑ Fogo, Agnes B.; Kashgarian, Michael (2005). Diagnostic Atlas of Renal Pathology: A Companion to Brenner and Rector's The Kidney 7E (1st ed.). Saunders. pp. 16. ISBN 978-1416028710.
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  29. ↑ Berden, AE.; Ferrario, F.; Hagen, EC.; Jayne, DR.; Jennette, JC.; Joh, K.; Neumann, I.; Noël, LH. et al. (Oct 2010). "Histopathologic classification of ANCA-associated glomerulonephritis.". J Am Soc Nephrol 21 (10): 1628-36. doi:10.1681/ASN.2010050477. PMID 20616173. http://jasn.asnjournals.org/content/21/10/1628/T1.expansion.html.
  30. ↑ AH. 17 July 2009.
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  32. ↑ URL: http://path.upmc.edu/cases/case51/dx.html. Accessed on: 9 November 2010.
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  34. ↑ Zelmanovitz T, Gerchman F, Balthazar AP, Thomazelli FC, Matos JD, Canani LH (2009). "Diabetic nephropathy". Diabetol Metab Syndr 1 (1): 10. doi:10.1186/1758-5996-1-10. PMC 2761852. PMID 19825147. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2761852/.
  35. ↑ Østerby R, Hartmann A, Bangstad HJ (April 2002). "Structural changes in renal arterioles in Type I diabetic patients". Diabetologia 45 (4): 542–9. doi:10.1007/s00125-002-0780-2. PMID 12032631.
  36. ↑ Ritchie S, Waugh D (1957). "The pathology of Armanni-Ebstein diabetic nephropathy". Am. J. Pathol. 33 (6): 1035–57. PMC 1934668. PMID 13478656. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1934668/?page=1.
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  38. ↑ 38.0 38.1 URL: http://www.ndt-educational.org/ferrariodiabete.asp. Accessed on: 29 April 2012.
  39. ↑ Stout LC, Whorton EB (August 2007). "Pathogenesis of extra efferent vessel development in diabetic glomeruli". Hum. Pathol. 38 (8): 1167–77. doi:10.1016/j.humpath.2007.01.019. PMID 17490718.
  40. ↑ Tervaert, TW.; Mooyaart, AL.; Amann, K.; Cohen, AH.; Cook, HT.; Drachenberg, CB.; Ferrario, F.; Fogo, AB. et al. (Apr 2010). "Pathologic classification of diabetic nephropathy.". J Am Soc Nephrol 21 (4): 556-63. doi:10.1681/ASN.2010010010. PMID 20167701.
  41. ↑ Weening JJ, D'Agati VD, Schwartz MM, et al. (February 2004). "The classification of glomerulonephritis in systemic lupus erythematosus revisited". J. Am. Soc. Nephrol. 15 (2): 241–50. PMID 14747370. http://www.nature.com/ki/journal/v55/n2/full/4490631a.html.
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  43. ↑ Wei, Q.; Zhang, L.; Liu, X. (Feb 2011). "Outcome of severe preeclampsia manifested as nephrotic syndrome.". Arch Gynecol Obstet 283 (2): 201-4. doi:10.1007/s00404-009-1338-z. PMID 20033418.
  44. ↑ Smerud, HK.; Fellström, B.; Hällgren, R.; Osagie, S.; Venge, P.; Kristjánsson, G. (Aug 2009). "Gluten sensitivity in patients with IgA nephropathy.". Nephrol Dial Transplant 24 (8): 2476-81. doi:10.1093/ndt/gfp133. PMID 19332868.
  45. ↑ Komatsuda, A.; Wakui, H.; Yasuda, T.; Imai, H.; Miura, AB.; Tsuda, A.; Nakamoto, Y. (Nov 1994). "Successful delivery in a pregnant women with crescentic IgA nephropathy.". Intern Med 33 (11): 723-6. PMID 7849391.
  46. ↑ Kim, JK.; Kim, JH.; Lee, SC.; Kang, EW.; Chang, TI.; Moon, SJ.; Yoon, SY.; Yoo, TH. et al. (Mar 2012). "Clinical features and outcomes of IgA nephropathy with nephrotic syndrome.". Clin J Am Soc Nephrol 7 (3): 427-36. doi:10.2215/CJN.04820511. PMID 22322610.
  47. ↑ Coppo, R.; Cattran, D.; Roberts Ian, SD.; Troyanov, S.; Camilla, R.; Cook, T.; Feehally, J. (Jul 2010). "The new Oxford Clinico-Pathological Classification of IgA nephropathy.". Prilozi 31 (1): 241-8. PMID 20693944.
  48. ↑ 48.0 48.1 Pakasa, NM.; Binda, PM. (Jul 2011). "HIV-associated immune complex glomerulonephritis with lupus-like features.". Saudi J Kidney Dis Transpl 22 (4): 769-73. PMID 21743226.
  49. ↑ Sethi, S.; Fervenza, FC. (Jul 2011). "Membranoproliferative glomerulonephritis: pathogenetic heterogeneity and proposal for a new classification.". Semin Nephrol 31 (4): 341-8. doi:10.1016/j.semnephrol.2011.06.005. PMID 21839367.
  50. ↑ Sato, H. (Sep 1990). "[Ultrastructural study on membranoproliferative glomerulonephritis with special reference to subepithelial deposits].". Nihon Jinzo Gakkai Shi 32 (9): 973-83. PMID 2263028.
  51. ↑ 51.0 51.1 51.2 Walker, PD.; Ferrario, F.; Joh, K.; Bonsib, SM. (Jun 2007). "Dense deposit disease is not a membranoproliferative glomerulonephritis.". Mod Pathol 20 (6): 605-16. doi:10.1038/modpathol.3800773. PMID 17396142.
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  58. ↑ AM. 13 August 2009.
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  64. ↑ 64.0 64.1 64.2 64.3 64.4 64.5 Alpers, CE.; Kowalewska, J. (Jan 2008). "Fibrillary glomerulonephritis and immunotactoid glomerulopathy.". J Am Soc Nephrol 19 (1): 34-7. doi:10.1681/ASN.2007070757. PMID 18045849.
  65. ↑ Korbet, SM.; Schwartz, MM.; Lewis, EJ. (Mar 1991). "Immunotactoid glomerulopathy.". Am J Kidney Dis 17 (3): 247-57. PMID 1996564.
  66. ↑ Yang, L.; Su, T.; Li, XM.; Wang, X.; Cai, SQ.; Meng, LQ.; Zou, WZ.; Wang, HY. (Jun 2011). "Aristolochic acid nephropathy: variation in presentation and prognosis.". Nephrol Dial Transplant. doi:10.1093/ndt/gfr291. PMID 21719716.
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  68. ↑ 68.0 68.1 Reginster, F.; Jadoul, M.; van Ypersele de Strihou, C. (Jan 1997). "Chinese herbs nephropathy presentation, natural history and fate after transplantation.". Nephrol Dial Transplant 12 (1): 81-6. PMID 9027778. http://ndt.oxfordjournals.org/content/12/1/81.
  69. ↑ Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Reuter, Victor E; Stoler, Mark H (2009). Sternberg's Diagnostic Surgical Pathology (5th ed.). Lippincott Williams & Wilkins. pp. 1725. ISBN 978-0781779425.
  70. ↑ 70.0 70.1 Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 251. ISBN 978-1416002741.
  71. ↑ Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Reuter, Victor E; Stoler, Mark H (2009). Sternberg's Diagnostic Surgical Pathology (5th ed.). Lippincott Williams & Wilkins. pp. 1726. ISBN 978-0781779425.
  72. ↑ Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Reuter, Victor E; Stoler, Mark H (2009). Sternberg's Diagnostic Surgical Pathology (5th ed.). Lippincott Williams & Wilkins. pp. 1728. ISBN 978-0781779425.
  73. ↑ URL: http://medical-dictionary.thefreedictionary.com/pyelo-. Accessed on: 5 June 2015.
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  75. ↑ 75.0 75.1 Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Reuter, Victor E; Stoler, Mark H (2009). Sternberg's Diagnostic Surgical Pathology (5th ed.). Lippincott Williams & Wilkins. pp. 1729. ISBN 978-0781779425.
  76. ↑ Kanbay, M.; Kasapoglu, B.; Perazella, MA. (Jun 2010). "Acute tubular necrosis and pre-renal acute kidney injury: utility of urine microscopy in their evaluation- a systematic review.". Int Urol Nephrol 42 (2): 425-33. doi:10.1007/s11255-009-9673-3. PMID 19921458.
  77. ↑ 77.0 77.1 77.2 Kocovski, L.; Duflou, J. (Mar 2009). "Can renal acute tubular necrosis be differentiated from autolysis at autopsy?". J Forensic Sci 54 (2): 439-42. doi:10.1111/j.1556-4029.2008.00956.x. PMID 19207286.
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