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NEPHRITIC SYNDROME By Dr Mai inbiek
NEPHRITIC SYNDROME
By Dr Mai inbiek
Nephritic Syndrome
➢ The nephritic Syndrome is a clinical complex, usually of acute onset.
➢ Is caused by inflammatory lesions of glomeruli.
➢ Characterized by;
1) Hematuria with red cells(dysmorphic) & red blood cell casts in urine (hallmark) .
2) Some degree of oliguria and azotemia.
3) Hypertension.
4) Proteinuria (subnephrotic range 0.3–3 g/d).
5) Edema, if present, in dependent (e.g., periorbital or scrotal) areas.(due to less fluid excreted0
Retention of metabolitesAzotemia HypertensionEdema
+
injures the capillary walls
Hypertension
Renin from the ischemic kidney
Oliguria + azotemia
reduction in the GFR
Hemodynamic changes
escape of red cells into the urine
The lesions that cause the nephritic syndrome have in common proliferation of the cells within the glomeruli,
accompanied by a leukocytic infiltrate
NEPHRITIC SYNDROME
Causes
1- Primary glomerular disease as:
➢ Acute diffuse proliferative GN.
(Poststreptococcal GN).
➢ Membranoproliferative GN
➢ IgA nephropathy
➢ Rapidly progressive GN
➢ Hereditary nephritis
2- Systemic disorders such as SLE
Acute Proliferative Glomerulonephritis
➢ Cluster of diseases characterized histologically by diffuse proliferation of glomerular cells,
associated with influx of leukocytes , especially neutrophils.
➢ This inflammatory reaction injures the capillary walls, permitting escape of red cells into the urine,
and induces hemodynamic changes that lead to a reduction in the GFR.
➢ These lesions are typically caused by immune complexes.
➢ The inciting antigen may be exogenous or endogenous.
➢ The prototypic exogenous antigen-induced disease pattern is post infectious glomerulonephritis.
➢An example of an endogenous antigen-induced disease is the nephritis of SLE.
➢ The most common underlying infections are streptococcal, but the disorder also has been
associated with other infections like pneumococcal ,staphylococcal infections, viral diseases such
as mumps, measles, chicken-pox, and hepatitis B and C.
The classic case, a young child abruptly develops malaise, fever, nausea, oliguria, and hematuria (smoky or cola-colored urine) 1 to 2 weeks after recovery from a sore throat. The patients have red cell casts in the urine, mild proteinuria (usually less than 1 gm/day), periorbital edema, and mild to moderate hypertension.
post-streptococcal glomerulonephritis
Acute Proliferative(Poststreptococcal, Post infectious) GN
Poststreptococcal glomerulonephritis occurs most frequently in children 6 to 10 years of
age, but adults of any age can also be affected.
➢ Only certain strains of group A β-hemolytic streptococci are nephritogenic.
➢ The initial infection is localized to the pharynx or skin (impetigo) appears 1 to 4 weeks after a
streptococcal infection.
➢It is an immune complex disease, when the nature of the pathogenic antigen remains not clear
whether circulating complexes or those formed in situ deposited on the GBM
(subepithelial) complement activation inflammatory reaction in glomeruli
glomerular injury.
Morphology;
Light microscope: The classic diagnostic picture is one of
enlarged, diffuse hypercellular glomeruli .
➢ The hypercellularity is caused by:
(1) Infiltration by leukocytes, both neutrophils and
monocytes.
(2) Proliferation of endothelial and mesangial cells.
(3) In severe cases crescent formation.
➢ Neutrophil exudation, and red cell casts within tubules.
Electron microscopy shows subepithelial immune complex deposits (humps) .
Immunofluorescence shows granular deposits of IgG, IgM, and C3 throughout the glomerulus (staphylococcal infection produce IgA).
Low power H & E This glomerulus is large hypercellular and capillary loops are poorly defined. This is a type of proliferative glomerulonephritis
Sub epithelial electron-dense deposits or “humps”
Clinical course;
➢ Fever, nausea & nephritic syndrome (mild).
➢There is usually gross hematuria due to oxidation of hemoglobin to methemoglobin.
➢In children; Complete recovery occurs in most cases(spontaneously resolves).
➢In some cases the proteinuria is severe--- nephrotic syndrome.
➢ Few develop crescentic GN or CRF.
➢In adults; 15-50% develop CRF in 10-20 years.
Blood tests:
-Antibodies against group A sterpto=AntiDNase B.
-Decreased complement( hypocomplementemia)
(Note: Post infectious GN due to staphylococcal infection , Note the red cell casts in the tubules.
- NEPHRITIC SYNDROME
Causes 1-Primary glomerular disease as:
➢Acute diffuse proliferative GN.
➢ IgA nephropathy
➢Rapidly progressive GN
➢Membranoproliferative GN
➢ Hereditary nephritis
2- Systemic disorders such as SLE.
*Approximate prevalence of primary disease is 95% of the cases in children and 60% in adults.Approximate prevalence of systemic disease is 5% of the cases in children and 40% in adults.
IgA Nephropathy (Berger disease)
(Disease That Present Mostly With Asymptomatic Hematuria) .
➢ It usually affects children & young adult, men affected more common than women.
➢ Begins by gross hematuria that occurs within 1-2 days of upper respiratory tract infection.
➢Hematuria subsides after days & recurs every few months.
➢IgA nephropathy is the commonest cause of recurrent gross hematuria or microscopic hematuria
& is the most common glomerular disease worldwide.
• The pathogenic hallmark is the deposition of IgA in the mesangium. Some have considered
IgA nephropathy to be a localized variant of Henoch-Schönlein purpura, also characterized by
IgA deposition in the mesangium. In contrast to IgA nephropathy, which is purely a renal
disorder, Henoch-Schönlein purpura is a systemic syndrome involving the skin (purpuric rash),
gastrointestinal tract (abdominal pain), joints (arthritis), and kidneys.
• Pathogenesis:
• IgA is present in plasma at low concentration normally. Mostly present in mucosal secretion.
• There are two subclasses of IgA molecules in humans (IgA1 and IgA2), only IgA1 forms the
nephritogenic deposits of IgA nephropathy. An abnormally glycosylated IgA1 (i.e., galactose-
deficient IgA1 [Gd-IgA1]) immunoglobulin is thought to play a central role in the pathogenesis.
Pathogenesis,cont’d,
➢genetically susceptible individuals, respiratory or gastrointestinal exposure to microbial or other
antigens (e.g., viruses, bacteria, food proteins) lead to increased IgA synthesis, some of
which is abnormally glycosylated bind to IgG and form antiglycan antibodies
forming IgA-containing immune complexes which deposited with IgA in the mesangium. The
presence of C3 points to activation of the alternative complement pathway
proinflammatory cytokines and initiate glomerular injury.
➢Patients with IgA nephropathy may have increased production of IgA ,this occurs in cases of
celiac disease due to presence of mucosal defect and in cases of liver diseases where there is
defective hepatobiliary clearance of IgA complexes (secondary IgA nephropathy).
➢Genetic influence is suggested by the occurrence of this condition in families.
Morphology:
Light microscope: The glomeruli may be normal or
show:
➢ Segmental proliferation confined to some
glomeruli(FGN) , or
➢Diffuse mesangial proliferation
(mesangioproliferative GN) ,or
➢Crescentic GN.
Electron microscopy confirms the presence of
electron-dense deposits in the mesangium.
By immunofluorescence microscopy
➢ Mesangial deposition of IgA often
with C3 and properdin and lesser
amounts of IgG or IgM.
mesangial widening andinflammation
Clinical course
The disease can be suspected by light microscopic examination, but the diagnosis is made only by immunocytochemical techniques .
➢Many patients present with gross recurrent hematuria(red or cola-coloured urine) 1-2 days after an
infection of the respiratory or, less commonly, gastrointestinal or urinary tract.
➢The hematuria typically lasts for several days and then subsides, only to return every few months.
➢ 30% to 40% have only microscopic hematuria, with or without proteinuria.
➢ 5% to 10% develop a typical acute nephritic syndrome.
➢Many patients maintain normal renal function for decades.
➢ Slow progression to chronic renal failure occurs in 15% to 40% of cases over a period of 20 years.
➢ Onset in old age, heavy proteinuria, hypertension, and the extent of glomerulosclerosis on biopsy
are clues to an increased risk of progression.
Lab findings: Serum IgA level (50%), normal serum complement level .
Indications of renal biopsy in IgA nephropathy :
• It is better to be earlier if there is indication .what indication?
• Recurrent hematuria with proteinuria or any degrees of renal dysfunction or hypertension .
• But if patient presented only with isolated hematuria with no or mild proteinuria( or less than
1gm) ,and normal RFT no biopsy, and should follow up the pt.
• Relative renal biopsy indication , if pt with isolated hematuria come with relative as a donor
for renal transplantation , so biopsy may done.
.
1- NEPHRITIC SYNDROME
Causes Primary glomerular disease as:
➢ Acute diffuse proliferative GN.
➢ Membranoproliferative GN
➢ IgA nephropathy
➢ Rapidly progressive GN
➢ Hereditary nephritis
2- Systemic disorders such as SLE
RAPIDLY PROGRESSIVE (CRESCENTIC)
GLOMERULONEPHRITIS
Rapidly progressive GN (RPGN, Crescentic GN)
➢ It is a clinical syndrome assoc. with sever,intense glomerular injury, characterized by rapid
progressive loss of renal function(uremia) & severe oliguria and signs of nephritic syndrome. It
does not denotes a specific etiologic form of GN.
➢ If untreated , renal failure occurs in weeks or months. Usually in adults 50s -60s.
➢ The most common histologic picture is the presence of crescents in most glomeruli .
Classification and Pathogenesis
➢ RPGN may be caused by a number of different diseases, some restricted to the kidney and
others systemic.
➢ Although no single mechanism can explain all cases, there is little doubt that in most cases the
glomerular injury is immunologically mediated.
➢ A practical classification divides RPGN into three groups on the basis of immunological
findings .
➢ In each group the disease may be associated with a known disorder, or it may be idiopathic.
Type I RPGN; (Anti-GBM), 12%
➢May be idiopathic or in cases of Goodpasture syndrome.
➢Patients benefit from plamapharesis.
➢Anti-GBM antibodies in serum.
➢There is linear deposits of IgG & C3.
Type II RPGN; (Immune complex), 44%
➢May be idiopathic or may complicate any of the immune complex nephritis as in cases of SLE,
post-infectious GN, IgA nephropathy, Henoch-Schonlein purpura.
➢This type shows cellular proliferation and influx of leukocytes within the glomerular tuft, in
addition to crescent formation.
➢There is granular deposits of immunoglobulins in GBM and/or mesangium and/or
complement on immunofluorescence studies. This disorder usually does not respond to
plasmapheresis.
• Type III RPGN; (Pauci-immune), 44%
➢May be idiopathic or in some cases of systemic vasculitis such as Wagener granulomatosis, microscopic polyarteritis.
➢No immune deposits ( lack of anti-GBM antibodies or significant immune complex deposition).
➢ANCA antibodies in serum.
• The common denominator in all types of RPGN is severe glomerular injury.
GOODPASTURE SYNDROME(autoimmune disease)
-(anti-GBM disease) is due to the production of antibodies(mainly
IgG) directed against basement membrane (anti-GBM antibodies)
,which results in damage of the lungs and the kidneys ,because anti-
GBM antibodies also bind to (Cross reaction) pulmonary alveolar
capillary basement membranes to produce the clinical picture of
pulmonary hemorrhages associated with renal failure.
-The Goodpasture antigen is the noncollagenous component of type
IV collagen.
globular noncollagenousdomain (NC1) at the C terminus.
Morphology of RPGN;
Gross;
Kidneys are enlarged, pale with petechial hemorrhages on cortical surfaces
Microscopically
➢ Depending on the underlying cause, the glomeruli may show cellular proliferation outside the capillary loops, sometimes in association with segmental capillary necrosis, breaks inGBM, and deposition of fibrin in Bowman’s space .
➢ More than half of the glomeruli show crescents.
➢ Crescents are formed by proliferation of parietal cells of Bowman's capsule & by migration of monocytes and macrophages into the urinary space.
➢ Fibrin strands are frequently prominent between the cellular layers in the crescents.
➢ Crescents may obliterate Bowman's space & compress the glomeruli tuft.
❑The escape of fibrinogen into Bowman space and its conversion to fibrin are an important contributor to crescent formation.
Crescentic glomerulonephritis (PAS stain). Note the collapsed glomerular tufts.Crescent between Bowman capsule and
glomerular tuft.
• Electron microscopy
▪ Regardless of type, electron microscopy may show distinct ruptures in the GBM, indicating severe injury that allows leukocytes, proteins, and inflammatory mediators to reach the urinary space, where they trigger the crescent formation.
▪ In time, most crescents undergo sclerosis, but restoration of normal glomerular architecture may be achieved with early aggressive therapy. -In type II, electron dense immune complex deposits are seen within the glomeruli.
Electron micrograph
showing characteristic
wrinkling of GBM with
focal disruptions
(arrows)
• By immunofluorescence microscopy
➢Goodpasture syndrome cases show
linear GBM fluorescence for IgG and C3
complement,
➢ Immune complex–mediated cases show
granular immune deposits;
➢pauci-immune cases have little or no
deposition of immune reactants. (-ve)
Linear IF
Clinical Course
➢RPGN is a clinical entity with features of the nephritic syndrome (hematuria , dysmorphic RBC,
RBC casts, hypertension , ..) with rapid loss of renal function and progress rapidly to renal
failure(uremia ).
➢ Oliguria and azotemia are more pronounced. Proteinuria sometimes approaching nephrotic
range may occur.
➢ In Goodpasture syndrome the course may be dominated by recurrent hemoptysis or even life-
threatening pulmonary hemorrhage.
➢ Serum analyses for anti-GBM antibodies, antinuclear antibodies, and ANCAs are helpful in the
diagnosis of specific subtypes.
• The prognosis can be roughly related to the number of crescents ,those with crescent are
present in less than 80% of the glomeruli have a more favorable prognosis than those in
whom the percentage of crescents is higher, and it has poor prognosis if not treated early.
Treatment:
• Anticoagulant to reduce fibrin .
• Plasma exchange(in association with immunosuppressants) may be of benefit in those with
anti-GBM antibody GN and in some cases of ANCA-related pauciimmune crescentic GN.
• Immunosuppressants .
• If irreversible , hemodialysis.
• May require transplantation.
Hereditary nephritisAlport syndrome ,Fabrys disease nail-patella syndrome
NEPHRITIC SYNDROME
Causes1-Primary glomerular disease as:
➢Acute diffuse proliferative GN.
➢ IgA nephropathy
➢ Rapidly progressive GN
➢Membranoproliferative GN
➢ Hereditary nephritis
2- Systemic disorders such as SLE
HEREDITARY NEPHRITISA collection of genetic diseases which principally involve glomeruli .
Alport syndrome (triad of nephritis, nerve deafness, and eye disorders)
X-linked dominant disorder having mutation in alpha -5 chain of type IV collagen located on X-
chromosome. It affects males more severely than females . It has systemic manifestations.
• Slowly progressive, terminating in chronic kidney disease in the 2nd to 3rd decades of life.
The presenting features are persistent or recurrent hematuria accompanied by erythrocyte casts,
proteinuria and hypertension.
• Light microscopy, segmental proliferation of mesangial cells, lipid-laden foam cells in the
interstitium glomerular sclerosis, tubular atrophy and interstitial fibrosis.
• Electron microscope:
The GBM shows irregular foci of thickening alternating with attenuation (thinning),with longitudinal splitting and lamination of lamina densa, often producing a distinctive basket-weave
Appearance(due to defective GBM production). Similar alterations can be found in the tubular basement membranes.
• Treatment: ACE inhibitors and transplantation.
NEPHRITIC SYNDROME
Causes 1- Primary glomerular disease as:
➢Acute diffuse proliferative GN.
➢ Rapidly progressive GN
➢ IgA nephropathy
➢Membranoproliferative GN
➢ Hereditary nephritis
2-Systemic disorders such as SLE
Lupus nephritis:
➢ Renal manifestations of systemic lupus erythematosus (SLE) are termed lupus nephritis.
➢ Lupus nephritis affects up to 50% of SLE patients.
➢It occurs between 20 & 30 years.
➢ Common in females, F:M ratio=10:1.
➢SLE gives rise to a heterogenous group of glomerular lesions & clinical presentations.
➢ The principal mechanism of injury is immune complex deposition in the glomeruli, tubular or
peritubular capillary basement membranes, or larger blood vessels. It activate complements.
➢Other injuries may include thrombi in glomerular capillaries, arterioles, or arteries,
often associated with antiphospholipid antibodies.
➢All of the glomerular lesions are the result of deposition of immune complexes that
are regularly present in the mesangium causing cellular proliferation with infiltration of
PMNs ,or along the entire basement membrane and sometimes throughout the
glomerulus .
➢ The immune complexes consist of DNA and anti-DNA antibodies, but other antigens
such as histones have also been implicated.(TYPE III HS).
Classes of Lupus Nephritis (WHO classification)
Class I: Lupus with no renal lesion (20%)
Class II: Mesangial ("mesangial proliferative") lupus nephritis (10%)
Class III: Focal proliferative lupus nephritis (10%)
Class IV: Diffuse proliferative lupus nephritis (50%)
Class V: Diffuse membranous lupus nephritis (10%)
Class VI: Sclerosing lupus nephritis .
Class III, IV, V may show
➢ Focal necrosis of capillary wall & PNL infiltrate
➢ Fibrinoid deposits
➢ Capillary thrombi
➢ Wire-loop thickening of capillary wall
➢ Crescent formation
Class I: Minimal lesions
• On light microscopy, these cases do not show any abnormality. But examination by electron microscopy and immunofluorescence microscopy shows deposits within the mesangium which consist of IgG and C3.
• No clinical finding.
Class II: Mesangial lupus nephritis
These cases have mild clinical manifestations, and characterized by mesangial cell proliferation and immune complex deposition without involvement of glomerular capillaries.
By light microscopy, there is increase in the number of mesangial cells and amount of mesangial matrix.
Electron microscope and immunofluorescence studies reveal granular mesangial deposits of IgG and C3; sometimes IgA and IgM are also present in the deposits.
Class III: Focal segmental lupus nephritis(focal proliferative LN)
• Is defined by less than half glomeruli are involved . The lesions may be segmental or global.
• By light microscope: Focal and segmental proliferation of endothelial and mesangial cells, together with infiltration by macrophages and sometimes neutrophils.
• Clinically, class II and III, come with mild to moderate proteinuria and hematuria.
Lupus nephritis, focal proliferative type. There are two focal necrotizing lesions in the glomerulus (arrows).
Class IV: Diffuse proliferative lupus nephritis
• all the morphologic manifestations of lupus are present in most advanced form .This is the most severe and the most common form of lupus nephritis (occurring in 35% to 60% of patient).
• Clinically, combination of nephrotic and nephritic syndrome.
• light microscopy. diffuse proliferation of endothelial,mesangial, and sometimes epithelial cells, involving most or all glomeruli with crescent formation .
-Subendothelial immune complex deposits may create a circumferential gross thickening of the capillary wall, forming “wire-loop” structures on light microscopy.
due to immune complexes deposition.
• Electron microscopy shows large electron-dense deposits in the mesangium and in the subendothelial region. Endothelial proliferation .
• Immunofluorescence are positive for IgG; sometimes also for IgA or IgM, and C3.
Lupus nephritis, diffuse proliferative type. Note the marked increase in cellularity throughout the glomerulus.
wire-loop” structures
Class V: Membranous lupus nephritis
• These lesions resemble those of idiopathic membranous GN.
• light microscopy :These consist of diffuse thickening of glomerular capillary wall. Mesangial hypercellularity is present in some cases.
• Electron microscope ,IF: show subendothelial deposits of immune complexes containing IgG, IgM and C3.
• Is usually accompanied by severe proteinuria or nephrotic syndrome, or diffuse lupus nephritis.
• Clinically Nephrotic syndrome.
Class VI: Sclerosing lupus nephritis:
• This is chronic kidney disease of SLE. Similar to chronic GN.
• Most glomeruli are sclerosed and hyalinised and there may be remnants of preceding lesions.
Clinical features:
-Lupus nephritis more commonly manifested as nephrotic syndrome , but many cases presented
as niphritic syndrome (according to location of injury and extent of lesion).
- Although in a given case, the lesions in lupus nephritis fit into one of the classes described
above, it is not unusual to find overlapping and progressive transformation of lupus lesions during
the course of disease, or progressed to RPGN renal failure.
• Diagnosis is typically require kidney biopsy which establish the pattern of injury and guide immunosuppressive therapy.
-Hypocomplementamia (specially C3, C4 due to overuse).
• Treatment:
• Class I and II: No treatment required.
• Class III,IV,V: Immunosuppression (corticosteroids, cyclophosphamide and |or azathioprine)
• Note: Renal lesion severity often determines overall prognosis of SLE patients.
• Henoch-Schönlein Purpura
• Type of vasculitis, causing purpuric skin lesions characteristically involving the extensor surfaces of arms and legs as well as buttocks and abdominal manifestations, nonmigratoryarthralgia; and renal abnormalities. The renal manifestations occur in one third of patients and include gross or microscopic hematuria, nephritic syndrome, and nephrotic syndrome, or some
combination of these.
• On histologic examination, the renal lesions vary from mild focal mesangial proliferation to diffuse mesangial proliferation and/or endocapillary to crescentic rapidly progressive GN
• IF: Deposition of IgA, sometimes with IgG and C3, in the mesangial region.
• recurrences of hematuria may persist for many years after onset. Most children have an excellent prognosis(self limited). Patients with the more diffuse lesions, crescents, or the nephrotic syndrome have a somewhat poorer prognosis.
➢Why do patients with nephritic syndrome develop oliguria and azotemia?-GFR?-Tubular function?
• 1- Inflamed glomeruli decrease GFR
• 2- Tubular function is intact, so reabsorbing fluid to make up for decreased GFR
➢What is seen in the urine sediment of patients with nephritic syndrome?
• RBC casts
• Dysmorphic RBC
➢Post-Strep Glomerulonephritis : Immune complexes deposit in the subepithelial layer. What are the immune complexes composed of?
• IgG, IgM, C3
➢Post-Strep Glomerulonephritis: When does this typically present?
• Presents 2-3 weeks after strep infection
➢In nephritic syndrome, neutrophils invade and damage the glomeruli. What would be seen on a biopsy?
• Hypercellular, inflamed glomeruli-
• Hypercellularity due to invading neutrophils-Inflamed due to damage from neutrophils
➢ Post-Strep Glomerulonephritis: Only certain strains of GAS are able to cause glomerulonephritis. What pathogenic factor must be present for this to develop?
• Only strains with M protein
➢Post-Strep Glomerulonephritis: How is this diagnosed?
• Anti-DNAse antibodies
• Anti-ASO antibodies-Typical diagnosis for GASPost-Strep Glomerulonephritis
• What is seen on EM?
• Subepithelial humps-Immune complexes deposit in the subepithelial layer
➢What is Rapidly Progressive Glomerulonephritis :?-What does this rapidly progress to?
• Nephritic syndrome that rapidly progresses to renal failure-Weeks to months
➢ Rapidly Progressive Glomerulonephritis :What is the characteristic findings on LM?
• Crescents in Bowman's space
➢ Rapidly Progressive Glomerulonephritis : All types of these will show crescents in Bowman's capsule. How can you
differentiate between the different types?
• Differentiate based on clinical picture and immunofluorescence
➢ Rapidly Progressive Glomerulonephritis :Wegener’s granulomatosis can cause RPGN with a negative IF. What antibodies is
this associated with?
• Wegener's granulomatosis = c-ANCA-cytoplasmic anti-nuclear-cytoplasmic-antibody
➢ Rapidly Progressive Glomerulonephritis : What will cause linear IF?
• Goodpasture’s syndrome
➢ Rapidly Progressive Glomerulonephritis : There are characteristically crescents seen in Bowman's space. What are the crescents
composed of?
• Crescents are composed of fibrin and macrophages-Also C3b and glomerular parietal cells
➢ Rapidly Progressive Glomerulonephritis : What are the possible causes of granular IF?
• Post-strep glomerulonephritis that progressed Diffuse proliferative glomerulonephritis
➢IgA Nephropathy (Berger's Disease):Because the excess IgA can't be cleared fast enough, it is able to form immune complexes. Where do these immune complexes deposit?
• Deposit in the mesangium of the glomeruli
➢IgA Nephropathy (Berger's Disease):What is the prognosis?
• Slowly progresses to chronic renal failure
➢IgA Nephropathy (Berger's Disease):Does this affect adults or kids?
• Affects both adults and kids
➢IgA Nephropathy (Berger's Disease):What is seen on IF?
• IgA immune complexes seen in mesangium
➢IgA Nephropathy (Berger's Disease):IgA immune complexes deposit in the mesangium of the glomeruli. How does this present?
• Episodic hematuria and RBC casts-Usually following a respiratory/GI infection
Specific Glomerular Diseases (sammary)
• Glomerular diseases are divided into primary glomerulonephritis (GN) and secondary forms.
• Acute GN is known to follow acute infection (poststreptococcal) and characteristically presents as acute nephritic syndrome in children.
• RPGN is an acute reduction in renal function resulting in acute renal failure rapidly and is characterised by formation of ‘crescents’.
• Minimal change disease is nephrotic syndrome in children accompanied by no apparent change in glomeruli by light microscopy.
• Membranous GN is characterised by widespread thickening of the glomerular capillary wall and is the most common cause of nephrotic syndrome in adults.
• Membranoproliferative GN is characterised by increase in mesangial cellularity associated with increased lobulation of the tuft, and irregular thickening of the capillary wall.
Focal segmental glomerulosclerosis is sclerosis and hyalinosis of some glomeruli and
portions of their tuft.
IgA nephropathy or Berger’s disease is another common form of GN in which there are
mesangial deposits of IgA.
Chronic GN is the final stage of a variety of glomerular diseases which result in
irreversible impairment of renal function.
• Renal manifestations of systemic lupus erythematosus are termed lupus nephritis and consists of 6 progressive classes (class I to VI).
• Diabetic nephropathy encompasses diabetic glomerulosclerosis, vascular lesions, diabetic pyelonephritis and tubular lesions.
• Hereditary nephritis includes Alport’s syndrome, Fabry’s disease and nail-patella syndrome.
