Renal Pathology Flashcards
Nephrotic Syndrome Major Characteristics (4)
Proteinuria (>3.5 g/day)
Hypoalbuminemia (Plasma < 3 g/dL)
Generalized Edema
Compensatory Hyperlipidemia
Nephritic Syndrome Major Characteristics (3)
Hematuria
HTN
Azotemia
In Situ Immune Complex Formation
Mechanisms with Microscopy (2) and Examples (4)
Abs to intrinsic tissue antigens
Linear immunofluorescence
Goodpasture syndrome
Abs to extrinsic antigens planted in glomerulus Granular immunofluorescence Postinfectious Glomerulonephritis Membranous nephropathy Membranoproliferative Glomerulonephritis
Circulating Complex Deposition
Examples (4)
Systemic Lupus Erythematosus
IgA Mediated Nephropathy
HBV/HCV Infections
Allergens
Acute Proliferative Glomerulonephritis
Pathogenesis, Morphology (4), Causes (3)
Immune complex mediated damage most often from deposition of postinfectious antigens
Hypercellularity
Leukocyte Infiltration
IgG/IgM/C3 granular deposits
Subepithelial humps
M protein Streptococcus (kids)
Post-staphylococcus
Some viruses and parasites
Acute Proliferative Glomerulonephritis Presentations
Kids (5) and Adults (4)
Age 6-10 Post strep pharyngitis/impetigo* Nephritic syndrome Dysmorphic RBCs Periorbital edema
Aggressive and atypical* Sudden HTN Edema Increased BUN Only 60% fully recover
RPGN Type I
Etiology (2), HLA Association, Treatment
Anti-Basement Membrane Abs
Abs to Type IV collagen (Goodpasture)
HLA-DRB1
Plasmapharesis and immunosuppression
Rapidly Progressive Glomerulonephritis
Morphology (4) and Presentation (3)
Basement membrane tears (wrinkling)
Crescentic lesions of proliferating epithelial cells
Collapsed glomerular tufts
Macrophage/Leukocyte infiltrates
Rapid progressive renal function loss
Severe oliguria
Nephritic syndrome
RPGN Type II
Cause, Microscopy and Treatment
Immune complex deposition (SLE, IgA, APG)
Subendothelial Granular immunofluorescence
Treat the underlying condition
RPGN Type III
Diagnosis and Examples (2)
Cirulating ANCA proteins
Granulomatosis with polyangiitis (PR3 ANCA) Microscopic Polyangiitis (MPO ANCA)
Nephrotic Syndrome Causes
Primary (3) and Secondary (5)
Membranous Nephropathy
Minimal Change Disease
Focal Segmental Glomerulosclerosis
Diabetes Amyloidosis SLE Drugs (NSAIDS, Heroin) Malignancy
Membranous Nephropathy
Pathogenesis (3), Morphology (2) and Presentation (3)
Diffuse thickening of glomerular capillary wall
From HLA-DQA1 mutation and PLA2 receptor Abs
Subepithelial IgG4 deposits
Spike formations
Nephrotic syndrome
Non-selective proteinuria
Hematuria
Minimal Change Disease
Microscopy, Clinical Features (3), Treatment, Association
Foot Process Effacement
Only visible on Electron Microscopy
Most common Primary NS in children
Selective proteinuria
Edema
Very responsive to corticosteroids
Secondary to Non-Hodgkin Lymphoma (adults)
Focal Segmental Glomerulosclerosis Causes
Primary (1) and Secondary (3)
Primary
Idiopathic: Most common cause of NS in US**
Secondary (glomerular injury)
HIV Associated: collapsing variant possible
Ablation Nephropathy: loss of renal tissue
Genetic Mutation: NPHS, TRPC6, alpha-Actinin
Focal Segmental Glomerulosclerosis
Morphology (3), Clinical Features (6), Microscopy (2)
Foot Process Effacement (electron microscopy)
Sclerosis
Collapsing Glomerulopathy
Mixed Nephrotic/Nephritic: Hematuria Nonselective proteinuria Decreased GFR HTN Progresses to CKD
Focal IgM and C3 immunofluorescence
Focal Segmental Sclerosis (light microscopy)
Type I Membranoproliferative Glomerulonephritis
Pathogenesis (3), Presentation (2) and Microscopy (3)
Immune complexes in glomerulus
Activation of classic and alternative complement paths
Mixed GD: hematuria and proteinuria
Mesangial proliferation (Light) Subendothelial deposits (Electron) IgG, C3, C1q, C4 granular immunofluorescence
Type I MPGN Primary vs Secondary
Age, Prognosis, Associations (2)
Primary Presents younger Nephrotic/Nephritic HTN 50% progress to chronic
Secondary Presents in adults Associated with Chronic Antigenemia Seen in Hep C/SLE/Cancer patients 50% progress to chronic
Type II Membranoproliferative Glomerulonephritis
Pathogenesis (2), Presentation (4) and Microscopy (4)
C3 Nephritic Factor binds C3 convertase
C3C activates alternative complement pathway
Hematuria
Nephritic Syndrome
Progression to CKD (poorer prognosis)
Primary disease in kids/young adults
Mesangial proliferation (LM) GBM splitting (LM) C3 and IgG deposits (IF) Dense deposits (EM)
IgA Nephropathy Clinical Features (5), Microscopy (1)
Recurrent Hematuria after mucosal infections**
Most common cause of glomerulonephritis
More common in young adults, whites/asians, males
Familial component
Associated with gluten enteropathy
IgA in mesangium (IF)**
Chronic Glomerulonephritis
Description (3), Causes (5), Pathologic Features (3)
End stage glomerular disease
From either Acute or Asymptomatic Glomerulonephritis
Resulting in Uremia
Crescentic GN** FSGS MPGN Membranous nephropathy IgA nephropathy
Thinned cortex
Collagenous replacement of Glomeruli
Arterial sclerosis
Alport Syndrome Pathologic Features (4), Inheritance (2), Presentation (5)
Irregular thickening/thinning of GBM
Lamina densa lamination
Moth eaten or frayed lamina densa appearance
Mutations in Type IV collagen alpha-molecules
X-Linked: males most affected
Autosomal: males/females equally affected
Hematuria RBC casts Proteinuria Neural deafness Vision disturbances
Thin Basement Membrane Disease
Clinical Feature, Inheritance (2), Pathologic Feature
Familial asymptomatic hematuria**
Most people heterozygous carriers
Homozygous see defective Type IV collagen
Diffuse thinning of GBM
Secondary Nephrotic Syndrome Causes (4)
Diabetic Nephropathy
SLE (mixed)
Hepatitis C (MPGN Type I)
HIV Nephropathy (FSGS)
Secondary Nephritic Syndrome Causes (4)
SLE (mixed)
Bacterial Endocarditis (Acute Proliferative)
Goodpasture Syndrome (RPGN)
Henoch-Shonlein Purpura (IgA Nephropathy)
Diabetic Mellitus
Epidemiology (2) and Common Complications (4)
Leading cause of end stage renal failure in US (30%)**
Happens in 40% of diabetics
Nephropathy
Retinopathy
Cataracts
Neuropathy
Diabetic Nephropathy Morphologic Changes
Glomerulus (4), Papilla (2), Vasculature
Thickened GBM
Diffuse mesangial sclerosis
Nodular glomerulosclerosis
Via hyperglycemia and hypertrophy
Necrotizing papillitis (via pyelonephritis)
Arteriolosclerosis
Lupus Nephritis
Pathogenesis and Spectrum (I-VI)
Subendothelial immune complex deposits disrupt glomerulus
Minimal mesangial lupus nephritis (class I) Focal lupus nephritis (class II) Mesangial proliferative lupus nephritis (class III) Diffuse lupus nephritis (class IV) Membranous lupus nephritis (class V) Advanced sclerosing lupus nephritis (class VI)
Henoch-Schönlein Purpura
Description, Presentation (5), Morphology (2)
IgA nephropathy associated with systemic vaculitis
Purpuric skin lesions** Abdominal pain Intestinal bleeding Arthralgias Recurrent Hematuria**
IgA, IgG and C3 deposition in mesangium (IF) Mesangial proliferation (LM)
Bacterial Endocarditis Glomerulonephritis
Presentation (2) and Microscopy
Hematuria
Proteinuria
Glomerular immune complex deposits
Fibrillary Glomerulonephritis
Presentation (3) and Morphology (2)
Nephrotic syndrome
Hematuria
Progressive renal insufficiency
Fibrillar deposits in mesangium and glomerular capillaries
Common Histology of Goodpasture, Granulomatosis with Polyangiitis and Microscopic Polyangiitis (2)
Crescent Lesions
Foci of Glomerular Necrosis
Acute Tubular Injury
Etiology (2), Pathology (4), Histology (2)
Ischemia
Endogenous/Exogenous Toxic Agents
Tubule cell injury leading to:
Tubuloglomerular feedback
Tubule obstruction (by casts)
Tubular back leak
Focal Tubular epithelial necrosis
Swollen epithelial cells with vacuolization
Acute Tubular Injury Clinical Phase Descriptions
Initiation (1), Maintenance (2), Recovery (3)
Initiation: Oliguria
Maintenance: Uremia, Hyperkalemia*
Recovery: Polyuria, Hypokalemia*, Infection susceptibility
Tubulointerstitial Nephritis
Presentation (2), Etiologies (5)
and Features of Acute (3) vs Chronic (2)
Azotemia**
Inability to concentrate urine
Infections, Toxins, Metabolic Diseases, Obstruction, Neoplasms
Acute shows edema, eosinophils and neutrophils
Chronic shows fibrosis and tubular atrophy
Acute Pyelonephritis Predisposing Factors (4), Etiologies (5), Morphology (4)
Vesicoureteral reflux**
Diabetes
Pregnancy
Males with BPH
Ascending Cystitis from gram negative bacteria
(E. coli, Klebsiella, Proteus, Enterobacter)
Patchy interstitial inflammation
Intratubular neutrophils
Tubulitis
Tubular necrosis
Pyelonephritis Complications (3)
Papillary necrosis
Pyonephrosis
Perinephric abscess
Chronic Pyelonephritis
Definition (3), Etiologies (3) and Morphology (3)
Chronic tubulointerstitial inflammation leading to scarring of calyces and pelvis
**Analgesic nephropathy: Vesicoureteral reflux with superimposed UTI
Chronic Obstructive Pyelonephritis: BPH
Xanthogranulomatous: Proteus infections
Polar scarring
Dilated, blunted or deformed calyces
Flattened papillae
Papillary Necrosis Etiologies (3)
Ratios, Time course, Infection and Calcification
Diabetes Mellitus
Mostly female, 10 years, Infectious, no calcification
Analgesic Nephropathy
Mostly female, 7 years, Non-Infectious, calcification
Obstruction
Mostly male, variable time, Infectious, Calcification
Myeloma Kidney
Etiology (2), Morphology (3) and Clinical Features (2)
Bence-Jones proteinuria and cast nephropathy
Pink/Blue masses distending tubular lumens
Interstitial inflammation and fibrosis
Insidious chronic kidney disease
Proteinuria
Nephrolithiasis
Epidemiology (2), Predisposing Factors (4), Minerals (4)
Usually unilateral
Most often in men aged 20-30**
Increased mineral concentration**
pH disturbances
Decreased urine volume
Bacteria
Mostly Calcium**
Also Magnesium, Uric acid, and cystine
Benign Nephrosclerosis
Definition, Etiologies (3) and Morphology (3)
General process of hyaline sclerosis of renal arterioles and small arteries (not a diagnosis)
Increasing age
HTN
Diabetes mellitus
Cortical scarring and shrinking
Hyaline arteriolosclerosis
Patchy ischemic atrophy