Mt Notes Kidneys Flashcards
3 pathogenic mechanisms of glomerular pathology
- circulating immune complex deposition
- anti-glomerular basement membrane glomerulonephritis
- heymanns glomerulonephritis
circulating immune complex deposition
⋅ Type III Hypersensitivity reaction
⋅ Formation of antigen-antibody immune complex outside kidney that circulates and deposits in the
subintimal space, between the endothelial cells and the glomerular basement membrane of kidney ⋅ Phagocytes can’t engulf the Ag/Ab complex because they are hidden, so they release enzymes. The
enzymes damage the Ag/Ab complex, the structure of the glomerulus, as well as the vascular walls leading
to vasculitis. Function of the glomerulus is lost due to the damage. ⋅ Eventually develops vasculitis with damage of vascular walls
anti-glomerular basement membrane
⋅ Type II Hypersensitivity reaction (complement-dependent), autoimmune ⋅ Antigen forms against GBM, Ag/Ab complex forms in the kidneys (within glomerulus), attract phagocytic
cells causing chemical injury to GBM – can engulf entire glomerulus
heymanns glomerulonephritis
⋅ No specific type of hypersensitivity reaction, combination of several types ⋅ Ag/Ab complex forms against the visceral epithelial cells (podocytes) within kidneys. ⋅ Fragments of bacterial walls and dead cells get caught in the space between the glomerular basement
membrane and the podocytes. Viruses like to go to where these fragments are.
-sub epithelial deposits
Mesangial Cells
– part of the interstitial tissue within the glomeruli. They are active contractile cells that
produce and release collagen and matrix (mucopolysaccharides).
⋅ Monocytes can also be found in the space between capillaries and mesangial cells
– Located in the space between the capillaries – Contractile – Can produce components of connective tissue and participate in process of proliferation (healing) – Can produce erythropoietin – Can also produce some physiologically active substances
– Interstitial space
Juxtaglomerular Cells “Security”
– produce renin (most important), control blood pressure in the afferent
arteriole.
– Immune system of kidney function, control blood pressure in afferent arteriole – Angiotensin II, which functions to increase blood pressure – Decrease of glomerular filtration rate due to decrease BP (At least 50 mL/Hg)
Visceral Epithelial Cells
– aka podocytes
● Cover the outermost layer ● Podocytes (foot-like cells) aka foot processes
(pedicels, or pedicles)
● Filtration slits exist between podocytes
Glomerulonephritis
-Glomerular cells are extremely permeable to water and absolutely impermeable to proteins like albumins. Protein found in the urine is usually pathological.
⋅ There are two types of Glomerulonephritis – nephritic and nephrotic syndromes.
⋅ Type 3 Hypersensitivity
2 types of nephritic syndromes
- ACUTE PROLIFERATIVE (POST-INFECTOUS-STREPTOCOCCAL) GLOMERULONEPHRITIS
- RAPIDLY PROGRESSIVE (CRESCENTIC) GLOMERULONEPHRITIS
ACUTE PROLIFERATIVE (POST-INFECTOUS-STREPTOCOCCAL) GLOMERULONEPHRITIS
- Type II Hypersensitivity reaction
- B-hemolytic streptococcus A bacteria (pyogenes)
(nephrogenic) - molecular mimicry
- proteinuria and swelling of the eyes in children
3 types of Rapidly progressive a=(aka crescent) glomerulonephritis
-TYPE I: ANTI-GLOMERULAR BASEMENT MEMBRANE GLOMERULONEPHRITIS
-TYPE II: IMMUNE COMPLEX DEPOSITION
TYPE III: PAUCI-IMMUNE
TYPE I: ANTI-GLOMERULAR BASEMENT MEMBRANE GLOMERULONEPHRITIS
Type II hypersensitivity
Goodpasture’s Syndrome
Goodpasture’s Syndrome
type I: antiglomerular basement membrane glomerulonephritis -Type II hypersens -autoimmune -basement membranes of lung and kidneys -hemoptysis treat with plasmapheresis -exudate in arterioles -immune complex mediated inflamation
kinds of TYPE II: IMMUNE COMPLEX DEPOSITION
type III hypersensitivity
- Systemic Lupus Erythematosus
- Post Infectious Glomerulonephritis
- henoch-Scholein Purpura
Systemic Lupus Erythematosus
- autoimmune against cells and nuclei of cells
- Antinuclear antibodies ANA and anti-smith antibodies
- lupus nephritis
- lung involvement leads to cerebral vasculitis
Antinuclear antibodies ANA
-ANA against double strand DNA
-ANA against Anti Smith antigen (antibodies)
-
Henoch-Scholein Purpura
-men under 30
-/\ IgA production
attacks 4 major syndromes
1-cutaneous
2-articular
3-abdominal
4- kidney
1,2,3 have 100% survival rate
4- 70% survival
IgA deposits where?
in the mesangium
Henoch-Scholein Purpura Cutaneous syndrome
multiple subcutaneous hemorrhages up to 2 cm typically found on the thighs, buttocks and abdomen
Henoch-Scholein Purpura articular syndrome
arthralgia,
on and off benign pain to joints with NO inflammation
Henoch-Scholein Purpura abdominal syndrome
gastroduodenitis,
painful inflammation of the stomach and duodenum
sometimes with bleeding
Henoch-Scholein Purpura kidney syndrome
rapidly progressive glomerulonephritis (crescentic nephritis)
o The more crescents present, the shorter the life span
o Once kidneys reach 80% crescentic, prognosis is very bad
TYPE III: PAUCI-IMMUNE
- Wegener’s Granulematosus
- Polyarteritis Nodosa (aka Periarteritis)
- Berger’s Disease (aka IgA Nephropathy)
- Alport Syndrome (aka Hereditary Glomerulonephritis)
diseases that are nephritic syndrome
- goodpasture’s syndrome
- systemic lupus erythematosus
- post infectious glomerulonephritis
- henoch-scholein purpura
- Wegener’s granulematosus
- polyarteritis nodosa (Periarteritis)
- berger’s disease (IgA nephropathy)
- alport syndrome (hereditary glomerulonephritis)
diseases that are associated with nephrotic syndrome
- Minimal Change Disease aka Lipoid Nephrosis
- Membranous Glomerulonephritis
- Focal Segmental Glomerulonephritis
- Membranoproliferative Glomerulonephritis
- diabetes mellitus
- acute pyelonephritis
- chronic glomerulonephritis
- amyloidosis
nephritic syndrome
- azotemia
- oliguria
- decrease GFR
- uremia
- hematuria
- hypertension
nephrotic syndrome
- massive proteinuria (>3.5g/day)
- hypoalbuminemia
- generalized edema
- muehrcke’s lines/nails/leukonychia striata (white stripes)
- hyperlipidemia
- cardiac tamponade
- kidney amyloidosis
Wegener’s granulematosus
Classified by Necrotic Vasculitis of three major systems:
1. Upper Respiratory Tract – soft tissue and bones decay (facial skull), death occurs within
months, supporative inflammation accompanied with bad smelling person
2. Lower Respiratory Tract – cavities form in the lungs due to vasculitis
o Causes decrease in pressure in the pulmonary artery
3. Rapidly Progressive Glomerulonephritis – crescentic nephritis
Polyarteritis Nodosa (aka Periarteritis)
– Vasculitis and pouching all over body except the lungs and aortic arch (medium and small)
● Narrowing of the artery lumen caused by swelling of the vascular wall (due to inflammation),
leading to ischemia of the tissue
– Arterial walls become thin, brittle, and distend in pouches (nodes) further weakening wall – Pouching out of the walls of the arteries compress adjacent tissues, causing atrophy of these tissues,
therefore infarctions
– High mortality rate, swelling of soft tissues/loss of soft tissue with no damage to bones
– Autoimmune disease, unknown cause. It may be a genetic predisposition involving antigens.
– Associated with Wet Gangrene
Berger’s Disease (aka IgA Nephropathy)
– Most common glomerular disease in the world and most common cause of gross hematuria
– Not part of rapidly progressive
– Usually develops in children and young people, particularly boys.
– Gross hematuria (most common) develops within 1-2 days of non-specific respiratory infection (common
cold), lasts for several days and subsides on its own. Occurs again in several months
● (or after urinary or gastrointestinal tract infection, which may be associated with
development of gross hematuria)
– Patient will complain of loin pain (low back, buttocks)
– Increased findings of IgA, which are deposited into mesangium (hallmark of disease)
● Compare to purpura, which is a systemic disease ● If missed, it is not a real big deal. (chronic nephritis)
BUERGER DISEASE aka Thromboantitis Obliterans
- autoimmune vasculitis of middle to small sized arteries
- TIBIAL, RADIAL, ULNAR a.
- “disease of smoking people”
- young people 30-35
- instep claudication
- reynauds syndrome
- thrombophlebitis
- dry gangrene
- india, japan, israel genetic predisposition
Alport syndrome
aka hereditary glomerulonephritis
- boys = 10
- nerve deafness, lens dislocation, posterior cataracts, corneal dystrophy
- chronic glomerulonephritis
Things that lead to chronic Glomerulonephritis (GN)
- Poststreptococcal GN
- rapidly progressive (crescent) GN
- Membranous GN
- Focal Glomerulosclerosis
- Membranoprliferative GN
- bergers IgA nephropathy
Amyloidosis
– pathologic proteins (Amyloid AA or Amyloid BB) deposit into tissue interstitium and create pressure
atrophy in the kidneys, liver, heart and skin . or brain
-nephrotic syndrome= kidney amyloidosis
most likely nephritic syndrome to become chronic GMN
rapidly progressive crescent GMN
most likely nephrotic syndrome to become GMN
Focal segmental GMN
gouty GMN
- itching due to urine excretion through the skin
- CNS infection
Acute Pyelonephritis
– infection of the urinary tract, commonly traveling up the ascending pathway through the
ureter and into the kidney paths and parenchyma
-renal pyramids broke down
-necrotizing papilitis (suppurative)
-assoc w/ diabetes mellitus
-usually e coli or strep, g- rods
-chronic pyelonephritis is #1 cause of female Chronic GMN
diabetic nephropathy
- glomerular lesions
- thickening of basement membrane
- diffuse glomerulosclerosis
- nodular glomerulosclerosis - renal vascular lesions
- pyelonephritis
Diffuse glomerulosclerosis
– diffuse increase of mesangial matrix, mesangial cell proliferation
Nodular glomerulosclerosis
– characterized by Kimmelstiel-Wilson lesions (ball-like deposits of a
laminated matrix within the mesangium)
Chronic GMN presentation
- kidney becomes granulated
- breath smells like urine (uremia)
- manifestations of either nephrotic or nephritic syndromes or both
