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PEDIATRICS > Nephritic syndrome and Glomerulonephritis > Flashcards

Nephritic syndrome and Glomerulonephritis Flashcards

1
Q

NEPHRITIC SYNDROME

A

Clinical / Urinary manifestations
- hematuria / urinary casts
- hypertension
- edema
- minimal/mild proteinuria
± kidney failure

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2
Q

Nephritic syndrome /Nephritis
Definition
Common appearance
Etiology

A

is a nonspecific term that covers different kidney diseases

Common appearance: alteration of the kidney filtering membrane

Etiology: infectious or non-infectious

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3
Q

Acute nephritic syndrome
develops

A

suddenly (RPGN) or over a
short time period ( APSGN)

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4
Q

Chronic nephritic syndrome develops

A

and progress slowly ( IgA GN)

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5
Q

Acute Glomerulonephritis ( AGN )- ex

A

post - streptococcal, proliferative, endocapilary- glomerulonephritis

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6
Q

Crescentic glomerulonephritis

A

glomerulonephritis- extracapilary GN ; RPGN
- anti GMB antibody - (Goodpasture syndrome)
- immune complexe (lupus nephritis, PSAGN)
- pauci- immune GN (Wegener′ s granulomatosis-ANCA+)

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7
Q

Mesangial proliferative GN

A

( Henoch-Schonlein purpura)

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8
Q

Membrano proliferative GN(MPGN)

A

idiopatic or secondary

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9
Q

ACUTE ENDOCAPILARY GN(AGN)

A
  • Begins abruptly in children 3-14 years age old
  • Main etiological factor is a prior throat or cutaneos streptococcal infection; staphylococcus, pneumococcus, viral and parasitic infections are also AGN responsible.
  • Gross or microscopic hematuria is always present ( nephritic syndrome! )
  • Fever is not necessarily a clinical sign in AGN
  • Blood presure increases as kidney function becames impaired
    C3↓, C4↓ in the first 6-8 weeks,** ASO titre↑** ,mild increase in plasma creatinine
  • Good prognosis, complete recovery of renal function, in majority of patients, particularly in child
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10
Q

ACUTE ENDOCAPILARY GN
When RPGN develops ( in AGN evolution),

A

weakness, fatigue and fever are the most obvious
early symptoms
 Loss of appetite , nausea, vomiting, abdominal pain and joint pain, edema, and usually oliguria
/ anuria ( AKI or AKF !) are also common

 About 50% of people had a “flu-like” disease in the month before kidney failure started to
develop

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11
Q

AGN treatment

A

Largelly supportive; diet light in protein and sodium, until kidney function recovers

Management of the associated hypertension: diuretic therapy, hydralazine or a calcium channel blocker

Antibiotics are usually ineffective because nephritis begins 1-6 weeks after the streptococcal infection

If RPGN in AGN evolution, promptly treatment shoud be started

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12
Q

Henoch-Schönlein purpura (HSP)
Conforming ISKD - child classification, in HSP there are 6 grades histological nephritis patterns

A

♦ st I : minimal changes
♦ st II: pure mesangial proliferation without crescents, a.focal b. diffuse
♦ st III: mesangial proliferative GN with less than 50% crescents, a.focal b.diffuse
♦ st IV: mesangial proliferative GN with 50-75% crescents, a. focal b. diffuse
♦ st V: mesangial proliferative Gn with more than 75% crescents
♦ st VI: membranoproliferative( mesangioproliferative) GN

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13
Q

Henoch-Schönlein purpura (HSP)
Crescent definition

A

proliferation of the epithelial parietal cells, the monunuclear phagocytes, and fibroblasts in the urinary space
Percentage of crescents is important in appreciate severity and evolution , in any type of GN

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14
Q

Renal involvment in HSP

A

♦ microscopic/macroscopic hematuria
♦ proteinuria ± up to nephrotic range
♦ hypoalbuminemia
♦ severe hypertension
♦ abnormal renal function up AKF

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15
Q

The hypercellularity of post-infectious glomerulonephritis is due to

A

increased numbers of
epithelial,
endothelial,
and mesangial cells as well as neutrophils in, and around the
glomerular capillary loops.
Patients who have had a strep infection typically have an
elevated anti-streptolysin O (ASO) titer.

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16
Q

By electron microscopy, the immune deposits of post-infectious
glomerulonephritis are

A

predominantly subepithelial, with electron dense
subepithelial “humps” above the basement membrane and below the
epithelial cell. The capillary lumen is filled with a leukocyte cytoplasmic
granules.

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17
Q

AGN treatment

A

Largelly supportive; diet light in protein and sodium,
until kidney function recovers

Management of the associated hypertension: diuretic therapy, hydralazine or a calcium channel blocker

Antibiotics are usually ineffective because nephritis begins 1-6 weeks after the streptococcal infection

If RPGN in AGN evolution, promptly treatment shoud be started

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18
Q

Management in HSP

A

♦ st I, IIa,IIb – no treatment ± ACE inhibitor if persistent
proteinuria

♦ st.IIIa, IIIb – pulsed Methilprednisolone 600mg/m2 for three
days , oral prednisolone 2mg/kg/day( max 60 mg) for 1 month
than taper; consider Cyclophosphamide 2,5 mg/kg/day, for 8
weeks, ± ACE inhibitor if persistent proteinuria

♦ st. IVa,IVb, Va,Vb &VI necesitate steroids as above ,
Cyclophosphamide 8 weeks, consider plasmapheresis, ± ACE
inhibitor

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19
Q

SLE glomerulonephritis

A

Renal manifestations are present in nearly two thirds of children with SLE, and may be present with a combination of symptomatic signs/syndromes

  • hypertension
  • nephrotic syndrome
  • gross hematuria
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20
Q

SLE glomerulonephritis
(SLEGN)

A

A wide range of extrarenal manifestations is
commonly present

The choice therapy is influenced by potential renal or
extrarenal complications

There is a wide variation in the histological
appearances in lupus nephritis

21
Q

SLE-GN histology ( WHO clasif.)

A

♦ st 1. normal glomeruli ( a. negative IF& EM b. deposits on IF
& EM)

♦ st 2. mesangial alterations ( a. mild mesangial hypercellularity
b. moderate mesangial hypercellularity)=

diffuse mesangial deposits on IF&EM

♦ st 3. focal proliferative GN+ diffuse mesangial &
subendothelial/subepithelial deposits on IF & EM

♦ st 4. diffuse proliferative GN, presents mesangial,
subendotelial &subepitelial deposits on IF & EM

♦ st 5. membranous GN with diffuse subepithelial & few
mesangial and focal subendothelial deposits on IF & EM

22
Q

Glomerular disease with systemic lupus erythematosus (SLE) is common, and lupus
nephritis can have many morphologic manifestations as seen on renal biopsy;

A

the more immune complex deposition and the more cellular proliferation, the worse the disease. In this case, there is extensive immune complex deposition in the thickened glomerular capillary loops, giving a so-called ” wire loop appearance”

23
Q

SLE – GN treatment

A
  1. Renal involvment in children is often well controlled with
    corticosteroids alone
  2. FSGN &mild SN : corticosteroids, diuretic,antihypertensive
    agents if necessary
  3. If not DPGN , prednisolone 1mg/kg, preferably by alternate
    days
  4. Stage IV – Methilprednisolone (600mg/m2, in severe
    SLE-GN →good anti-inflammatory effect, but not for long
    term control
  5. In severe SLE-GN, corticosteroid unresponsive, intermitent
    IV cyclophosphamide →dramatically improves evolution
24
Q

SLE – severe GN treatment

A

1.Prednisolone + Azathioprine /Mychophenolat –Mofetil
could be a satisfacatory alternative

2.IV- Cyclophosphamide is given by monthly IV infusion,
500-1000mg/m2; in comparison with daily oral therapy, the immunosuppresive effects appears to be greater, and the
toxicity( especially bladder toxicity) appears to be less

  1. The optimal duration of IV treat - Cyclophosphamide
    therapy has not conclusively established; once 7 doses of
    therapy has been completed, continued administration at three month intervals, or alternatively oral Azathioprine may be substituted
25
Q

Familial Hematuria
The major forms

A
  • Thin Basement Membrane Nephropathy ( TBMN)
  • Alport syndrome

are caused by mutations in type IV collagen

Since 1990 four genetic loci, have been identified as sites of mutations in famillies transmitting hematuria

A critical type IV collagen nettwork is located in specialized BM of the kidney, cochlea and eye

Alport nephropathy evolution can be broken down into 4 phases

26
Q

Familial Hematuria/ Alport syndrome
Histology

A

Phase I: from birth until late childhood / early adolescence: mild mesangial proliferation & GMB attenuation, with focal areas of lamellation and normal
podocyte foot processes

Phase II: overt proteinuria in addition to hematuria ; GFR is normal. Mesangial proliferation by light microscopy, FSG, tubulointerstitium is spared.
EM: diffuse thickening and lamellation of the GBM, and extensive foot processes fusion

Phase III: declining renal function, interstitial fibrosis and tubular atrophy

Phase IV: clinical & histological features of ESRD

27
Q

hereditary nephritis:

A

Alport syndrome in which patients may also manifest
nerve deafness, and eye problems. The renal tubular cells appear foamy because of the accumulation of neutral fats and mucopolysaccharides. The glomeruli show irregular thickening and splitting of basement membranes.

28
Q

Alport syndrome – treatment

A
  • No specific treatment to alter the natural history of
    human Alport syndrome
  • Alport syndrome is primarly a glomerulopathy; loss of
    renal function is tightly correlated with expansion and
    fibrosis in the interstitial compartment
  • Early ACE inhibition has no effect on the onset of
    proteinuria but could delay ESRD. Angiotensin
    blockade, initiated early in the course of the disease may
    be beneficial in human Alport syndrome
  • Cyclosporine appeared to supress proteinuria &
    stabilize renal function
29
Q

MEMBRANOPROLIFERATIVE
GLOMERULONEPHRITIS (MPGN)
Epidemiology and types

A

Affects children & young adults, mean age of onset
between 8-30 years

It has been classified as idiopatic & secondary

Idiopatic MPGN is subdivided into types 1-3, based
on biopsy morphological patterns

30
Q

MPGN Type I electron miograph

A

splitting and reduplication of basement membrane that is piled up above the mesangial cytoplasm in this micrograph.
- These changes occur when the mesangial cell (which has a macrophage-like function) goes after subendothelial immune deposits, but makes a mess of the basement membrane in the process.

31
Q

MPGN Type II

A

The bright deposits scattered along capillary walls and in the mesangium by
immunofluorescence microscopy with antibody to complement component C3 are typical for membranoproliferative glomerulonephritis, type II. MPGN type II, known as dense deposit disease, produces a nephritic syndrome. Most patients have detectable circulating C3 nephritic factor, an IgG autoantibody.

32
Q

MPGN - treatment

A

**If plasma albumin >2,5g/L **± hypertension, no specific therapy

If hypertension is present, an ACE inhibitor should be used

If plasma albumin <2,5g/L ± normal/elevated plasma creatinine:
prednisolone 40mg/m2 , on alternate days,
6-12 months+aspirin1mg/kg, 3 times/week+
dipyridamole 1,5 mg/kg, 3 times/day

If there is improvement or clinical/biochimical stability, the prednisolon regimen could be continued, on alternate days, with progressive
tapering, for the next 30 months ( e.g. 20mg/m2, 15mg/m2, 10 mg/m2)

33
Q

PRIMARY IgA NEPHROPATHY
IGAN ( Berger disease)

A

The disease occors at all ages; affects males more often than
females

Five different clinical syndromes can be identified at onset

  • Recurrent macroscopic hematuria
  • Asymptomatic microscopic recurrent hematuria &
    proteinuria
  • Acute nephritic syndrome
  • Nephrotic syndrome
  • Nephritic/nephrotic syndrome

The commonest presentation of IGAN in children is
recurrent macroscopic hematuria + reccurent respiratoy
infections

34
Q

IGAN
high power microscopy

A

IgA nephropathy (Berger disease). The IgA is deposited mainly within the mesangium, which
then increases mesangial cellularity as shown at the arrow. Patients with IgA nephropathy usually
present with hematuria.

35
Q

IGAN
fluoroscopy

A

This immunofluorescence pattern demonstrates positivity with antibody to IgA. The pattern is that of mesangial deposition in the glomerulus.

36
Q

IGA NEPHROPATHY

A

The interval between precipitating URI and the appearance of hematuria ranges from 1-2 days

Serum IgA levels are increased in 16 – 18 % of children

Serum complement concentrations are usually normal

Difuse mesangial IgA deposits may be also observed in HSP, SLE ,and chronic liver disease

The prophylactic antibiotics and tonsillectomy may reduce the frequency of episodes of macroscopic hematuria; the long term benefit remains questionable

37
Q

IGA NEPHROPATHY
treatment

A

Glucocorticoids : in nephrotic syndrome and minimal mesangial
lesions but long term glucocorticoid and immunosupresant
treatmens does not confer any benefit

  • Patients with mild/moderate proteinuria ( PCR : 20-200
    mg/mmol) and diminished GFR : fish oil & ACE inhibitor
  • Glucocorticoid therapy should be considered in patients who
    have nephrotic range proteinuria & a normal GFR
38
Q

FOCAL GLOMERULOSCLEROSIS
FSGS

A

Diagnosis based on the presence of focal & glomerular
segmental scarrring on biopsy&steroid resistant nephrotic
syndrome

May also be present with asymptomatic proteinuria and/or
hematuria

In nephrotic syndrome steroid resistance, FSGS is definied
by the persistence of proteinuria following at least 4 weeks of
60 mg/m2 of oral prednisone administration

39
Q

FSGS
microscopy

A

An area of collagenous sclerosis runs
across the middle of this glomerulus. In contrast to minimal change disease, patients with FSGS are more likely to have non-selective proteinuria, hematuria, progression to chronic renal failure, and poor response to corticosteroid therapy.

40
Q

FSGS
treatment (1)

A

Mendosa protocol has been advocated in FSGS

Cyclophosphamide has been used more often than
Chlorambucil in FSGS ( 2,5 mg/kg/day for 90 days)

**Cyclosporin **has given in FSGS, in doses of 5mg/kg/day
with low dose alternate day steroids; found to be effective in
reducing urinary protein excretion & delaying the progression
to ESRD

41
Q

FSGS
treatment (2)

A

Malignant clinical course FSGS defines rapid deterioration in renal function completely unresponsive to all therapies; in these cases regular salt poor albumin infusion with diuretics are helpful

Patients with malignant course have a significant risk of recurrence post renal transplantation

42
Q

RAPIDLY PROGRESSIVE
GLOMERULONEPHRITIS( RPGN)

A

RPGN is a clinical definition, usually associated with a
crescentic GN on renal biopsy

  • Severe proliferation that results in circumferential layers of
    fibroblasts, macrophages &epithelial cells within Bowman′s
    space described as crescents
  • Primary- crescentic GN: Idiopathic, Anti- GMB disease,
    IgA- nephropathy, Membranous -GN
  • Secondary-crescentic GN: PSAGN, Shunt nephritis,
    Infective endocarditis, SLE, HSP, PAN, Wegener′s
    granulomatosis, Cryoglobulinemia
43
Q

RPGN / Crescentic GN - histology
three IF staining patterns on renal biopsy

A

a . Immune complexe : (e.g. PSAGN, HSPGN, SLE, MPGN, MGN ,Mixed Cryoglobulinemia). This condition have granular immune deposition on IF ( the commonest observed pattern)

b . Pauci – immune : Vasculitis ( e.g.PAGN, Wegener)& Idiopathic GN. These conditions are typified by the absence of immune deposits on IF & ANCA positivity

c . Anti- GMB antibody disease: (e.g. Goodpasture syndrome). This condition is typified by linear GMB staining &the presence of circulating anti-GMB antibody. May be associated with anti-alveolar MB
antibody staining ,and pulmonary hemorrhage

44
Q

RPGN with crescents
light microscopy

A

Crescents are composed of proliferating epithelial cells. Crescentic glomerulonephritis is known as rapidly progressive glomerulonephritis (RPGN) There are several causes, and in this case is
due to SLE. Note in the lower left glomerulus that the capillary loops are markedly thickened (the so-called “wire loop” lesion of lupus nephritis).

45
Q

RPGN with crescents
fluoroscopy

A

This immunofluorescence micrograph of a glomerulus demonstrates positivity with antibody to fibrinogen. With a rapidly progressive GN, the glomerular damage is so severe that fibrinogen leaks into Bowman’s space, leading to proliferation of the epithelial cells and formation of the bright
crescent shown here.

46
Q

RPGN with crescents
light microscopy in Goodpasture syndrome

A

Another glomerulus with epithelial crescents squashing the glomerular tufts from all sides. RPGN may be idiopathic or may result from SLE, post-infectious GN (as in some cases of post-infectious GN), from various types of vasculitis, and from Goodpasture syndrome.

47
Q

RPGN / CRESCENTIC GN
Clinical presentation /Treatment

A

Children with crescentic GN present oliguria, a significant & rapid elevation in plasma creatinine, hypoproteinemia, nephrotic range proteinuria in
association with other signs of acute GN

Patients with immune complex crescentic GN should be managed according to their specific underlying condition.

Pulse Methylprednisolone will be considered

48
Q

RPGN / CRESCENTIC GN
Treatment

A

The following regime should be considered in patients
who are ANCA ±

Methylprednisolone 600 mg/m2/day for 3 days ; oral
Cyclophosphamide 2,5 mg/kg/day, for 6 – 8 weeks
begining day 4, alternate day oral Prednisolone 2mg/kg
begining day 5, with a slow tailoring over 6 months

Other regime recommend : monthly boluses
Cyclophosphamide for 6 months or Mychophenolate-
Mophetil introduced progressively untill the full dose of
1g/1,73m2, twice daily, for 1 year, with fewer adverse
events

49
Q

NEPHRITIC SYNDROME (GN)
CONCLUSIONS

A

Nephritic syndrome defines a clinical presentation reflecting different pathological patterns ( GN) that some are progressive to ESRD

In the case of a severe, or unfavourable nephritic syndrome course, especially if nephrotic syndrome is associated , a renal biopsy should be considered

A severe clinical acute GN ( RPGN) presentation often correlates with a severe underlying histological pattern, e.g.crescentic GN

PEDIATRICS (12 decks)

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