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Title:

Acute Nephritic Syndrome

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Lawrence Mason MD PGY II NHRMC IM * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * Prognosis at ... – PowerPoint PPT presentation

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Title: Acute Nephritic Syndrome


1
Acute Nephritic Syndrome
  • Lawrence Mason MD
  • PGY II
  • NHRMC IM

2
Introduction
  • Acute nephritic syndromes classically present
    with the following
  • Hypertension
  • Hematuria
  • Red blood cell casts
  • Pyuria
  • Mild to moderate proteinuria

3
Introduction
  • Extensive inflammatory damage to glomeruli can
    cause a fall in GFR and eventually produce uremic
    symptoms with salt and water retention, leading
    to edema and hypertension.

4
Acute Nephritic Syndromes
  • Poststreptococcal Glomerulonephritis
  • Subacute Bacterial Endocarditis
  • Lupus Nephritis
  • Antiglomerular Basement Membrane Disease
  • IgA Nephropathy
  • ANCA Small Vessel Vasculitis
  • Membranoproliferative Glomerulonephritis
  • Mesangioproliferative Glomerulonephritis

5
Poststreptococcal Glomerulonephritis
  • Poststreptococcal glomerulonephritis is an
    immune-mediated disease involving
  • Streptococcal antigens
  • Circulating immune complexes
  • Activation of complement in association with
    cell-mediated injury.

6
Poststreptococcal Glomerulonephritis
  • Poststreptococcal glomerulonephritis is
    prototypical for acute endocapillary
    proliferative glomerulonephritis.
  • Acute poststreptococcal GN
  • 90 of cases affect children between the ages of
    2 and 14 years
  • 10 of cases are patients older than 40

7
Poststreptococcal Glomerulonephritis
  • The classic presentation is an acute nephritic
    picture with hematuria, pyuria, red blood cell
    casts, edema, hypertension, and oliguric renal
    failure, which may be severe enough to appear as
    RPGN.
  • Systemic symptoms of headache, malaise, anorexia,
    and flank pain (due to swelling of the renal
    capsule) are reported in as many as 50 of cases.

8
Poststreptococcal Glomerulonephritis
  • Poststreptococcal glomerulonephritis caused by
    impetigo and streptococcal pharyngitis
  • Impetigo 26 weeks after skin infection
  • Streptococcal pharyngitis 13 weeks after
    infection

9
Poststreptococcal Glomerulonephritis
  • Treatment is supportive, with control of
    hypertension, edema, and dialysis as needed.
  • Antibiotic treatment for streptococcal infection
    should be given to all patients and their
    cohabitants.
  • There is no role for immunosuppressive therapy,
    even in the setting of crescents.

10
Poststreptococcal Glomerulonephritis
  • Overall, the prognosis is good, with permanent
    renal failure being very uncommon (13), and
    even less so in children.
  • Complete resolution of the hematuria and
    proteinuria in children occurs within 36 weeks
    of the onset of nephritis.

11
Poststreptococcal Glomerulonephritis
  • The renal biopsy in poststreptococcal
    glomerulonephritis demonstrates
  • Hypercellularity of mesangial and endothelial
    cells
  • Glomerular infiltrates of polymorphonuclear
    leukocytes
  • Granular subendothelial immune deposits of IgG,
    IgM, C3, C4, and C5-9
  • Subepithelial deposits (which appear as "humps")

12
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13
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14
Subacute Bacterial Endocarditis
  • Endocarditis-associated glomerulonephritis is
    typically a complication of subacute bacterial
    endocarditis.
  • Particularly in patients who
  • Remain untreated for an extended period of time
  • Have negative blood cultures
  • Have right-sided endocarditis (IVDU)

15
Subacute Bacterial Endocarditis
  • Grossly, the kidneys in subacute bacterial
    endocarditis have subcapsular hemorrhages with a
    "flea-bitten" appearance.
  • Microscopy on renal biopsy reveals a focal
    proliferation around foci of necrosis associated
    with abundant mesangial, subendothelial, and
    subepithelial immune deposits of IgG, IgM, and C3.

16
Subacute Bacterial Endocarditis
  • The pathogenesis hinges on the renal deposition
    of circulating immune complexes in the kidney
    with complement activation.

17
Subacute Bacterial Endocarditis
  • Patients present with
  • Gross hematuria
  • Microscopic hematuria
  • Pyuria
  • Mild proteinuria
  • RPGN with rapid loss of renal function (less
    common)

18
Subacute Bacterial Endocarditis
  • Primary treatment is eradication of the infection
    with 46 weeks of antibiotics, and if
    accomplished expeditiously, the prognosis for
    renal recovery is good.

19
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21
Lupus Nephritis
  • Lupus nephritis is a common and serious
    complication of systemic lupus erythematosus
    (SLE) and most severe in African-American female
    adolescents.

22
Lupus Nephritis
  • Thirty to fifty percent of patients will have
    clinical manifestations of renal disease at the
    time of diagnosis.
  • Sixty percent of adults and eighty percent of
    children develop renal abnormalities at some
    point in the course of their disease.

23
Lupus Nephritis
  • Lupus nephritis results from the deposition of
    circulating immune complexes
  • Which activate the complement cascade
  • Leads to complement-mediated damage
  • Leukocyte infiltration
  • Activation of procoagulant factors
  • Release of various cytokines

24
Lupus Nephritis
  • The most common clinical sign of renal disease is
    proteinuria, but hematuria, hypertension, varying
    degrees of renal failure, and an active urine
    sediment with red blood cell casts can all be
    present.

25
Lupus Nephritis
  • Hypocomplementemia is common in patients with
    acute lupus nephritis (7090) and declining
    complement levels may herald a flare.
  • Renal biopsy, however, is the only reliable
    method of identifying the morphologic variants of
    lupus nephritis.

26
Lupus Nephritis
  • Patients with crescents on biopsy may have a
    rapidly progressive decline in renal function.
  • Without treatment, this aggressive lesion has the
    worst renal prognosis.

27
Lupus Nephritis
  • Treatment must combine high-dose steroids with
    either cyclophosphamide or mycophenolate mofetil.
  • Current evidence suggests that inducing a
    remission with administration of steroids and
    either cyclophosphamide or mycophenolate mofetil
    for 26 months, followed by maintenance therapy
    with lower doses of the same

28
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29
Antiglomerular Basement Membrane Disease
  • Patients who develop autoantibodies directed
    against glomerular basement antigens frequently
    develop a glomerulonephritis termed
    antiglomerular basement membrane (anti-GBM)
    disease.

30
Antiglomerular Basement Membrane Disease
  • When they present with lung hemorrhage and
    glomerulonephritis, they have a pulmonary-renal
    syndrome called Goodpasture's syndrome.

31
Antiglomerular Basement Membrane Disease
  • Goodpasture's syndrome appears in two age groups
  • Young men in their late 20s
  • Men and women in their 6070s
  • Disease in the younger age group is usually
    explosive
  • Hemoptysis
  • Sudden fall in hemoglobin
  • Fever
  • Dyspnea
  • Hematuria

32
Antiglomerular Basement Membrane Disease
  • The performance of an urgent kidney biopsy is
    important in suspected cases of Goodpasture's
    syndrome to confirm the diagnosis and assess
    prognosis.
  • Renal biopsies typically show focal or segmental
    necrosis that later, with aggressive destruction
    of the capillaries by cellular proliferation,
    leads to crescent formation in Bowman's space

33
Antiglomerular Basement Membrane Disease
  • The presence of anti-GBM antibodies and
    complement is recognized on biopsy by linear
    immunofluorescent staining for IgG (rarely IgA).

34
Antiglomerular Basement Membrane Disease
  • Prognosis at presentation is worse if the
    following
  • gt50 crescents on renal biopsy with advanced
    fibrosis
  • Serum creatinine is gt56 mg/dL
  • Oliguria is present
  • Need for acute dialysis

35
Antiglomerular Basement Membrane Disease
  • Patients with advanced renal failure who present
    with hemoptysis should still be treated for their
    lung hemorrhage, as it responds to plasmapheresis
    and can be lifesaving.
  • Treated patients with less severe disease
    typically respond to 810 treatments of
    plasmapheresis accompanied by oral prednisone and
    cyclophosphamide in the first 2 weeks.

36
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38
IgA Nephropathy
  • IgA nephropathy is an immune complex-mediated
    glomerulonephritis defined by the presence of
    diffuse mesangial IgA deposits often associated
    with mesangial hypercellularity.

39
IgA Nephropathy
  • IgA nephropathy is one of the most common forms
    of glomerulonephritis worldwide.
  • There is a male preponderance, a peak incidence
    in the second and third decades of life, and rare
    familial clustering.

40
IgA Nephropathy
  • Deposits of IgA are also found in the glomerular
    mesangium in a variety of systemic diseases,
    including
  • Chronic liver disease
  • Crohn's disease
  • Gastrointestinal adenocarcinoma
  • Chronic obstructive bronchiectasis
  • Idiopathic interstitial pneumonia
  • Dermatitis herpetiformis
  • Mycosis fungoides
  • Leprosy
  • Ankylosing spondylitis

41
IgA Nephropathy
  • The two most common presentations of IgA
    nephropathy are recurrent episodes of macroscopic
    hematuria during or immediately following an
    upper respiratory infection in children
    (Henoch-Schönlein purpura) or asymptomatic
    microscopic hematuria most often seen in adults.

42
IgA Nephropathy
  • Rarely, patients can present with acute renal
    failure and a rapidly progressive clinical
    picture.
  • Risk factors for the loss of renal function
    include the presence of hypertension or
    proteinuria, the absence of episodes of
    macroscopic hematuria, male, older age of onset,
    and more severe changes on renal biopsy.

43
IgA Nephropathy
  • Studies of patients with IgA nephropathy support
    the use of angiotensin-converting enzyme (ACE)
    inhibitors in patients with proteinuria or
    declining renal function.

44
IgA Nephropathy
  • When presenting as RPGN, patients typically
    receive
  • Steroids
  • Cytotoxic agents
  • Plasmapheresis

45
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46
ANCA Small Vessel Vasculitis
  • A group of patients with small-vessel vasculitis
    (arterioles, capillaries, and venules rarely
    small arteries) and glomerulonephritis who have
    serum ANCA positivity.
  • The antibodies are of two types
  • Anti-proteinase 3 (PR3)
  • Anti-myeloperoxidase (MPO)

47
ANCA Small Vessel Vasculitis
  • Wegener's granulomatosis (PR3)
  • Microscopic polyangiitis (MPO)
  • Churg-Strauss syndrome (MPO)
  • Belong to this group because they are
    ANCA-positive and have a pauci-immune
    glomerulonephritis with few immune complexes in
    small vessels and glomerular capillaries.

48
ANCA Small Vessel Vasculitis
  • Induction therapy usually includes some
    combination of plasmapheresis, methylprednisolone,
    and cyclophosphamide.
  • The steroids are tapered soon after acute
    inflammation subsides, and patients are
    maintained on cyclophosphamide or azathioprine
    for up to a year to minimize the risk of relapse.

49
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50
Membranoproliferative Glomerulonephritis
  • MPGN is sometimes called mesangiocapillary
    glomerulonephritis or lobar glomerulonephritis.
  • It is an immune-mediated glomerulonephritis
    characterized by thickening of the GBM with
    mesangioproliferative changes 70 of patients
    have hypocomplementemia.

51
Membranoproliferative Glomerulonephritis
  • MPGN is subdivided pathologically
  • Type I
  • Type II (idiopathic)
  • Type III (idiopathic)

52
Membranoproliferative Glomerulonephritis
  • Type I MPGN is commonly associated with
  • Persistent hepatitis C infections
  • Autoimmune diseases (lupus)
  • Cryoglobulinemia
  • Neoplastic diseases

53
Membranoproliferative Glomerulonephritis
  • Type I MPGN, the most proliferative of the three
    types.
  • Tram-tracking - mesangial proliferation with
    lobular segmentation on renal biopsy and
    mesangial interposition between the capillary
    basement membrane and endothelial cells.

54
Membranoproliferative Glomerulonephritis
  • Type I MPGN is secondary to glomerular deposition
    of circulating immune complexes
  • Patients with MPGN present with
  • Proteinuria
  • Hematuria
  • Pyuria (30)
  • Systemic symptoms of fatigue and malaise that are
    most common in children with Type I disease.

55
Membranoproliferative Glomerulonephritis
  • In the presence of proteinuria, treatment with
    inhibitors of the renin-angiotensin system is
    prudent.
  • There is some evidence supporting the efficacy of
    treatment of primary MPGN with steroids,
    particularly in children.
  • In secondary MPGN, treating the associated
    infection, autoimmune disease, or neoplasms is of
    demonstrated benefit.

56
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57
Mesangioproliferative Glomerulonephritis
  • Mesangioproliferative glomerulonephritis is
    characterized by expansion of the mesangium,
    sometimes associated with mesangial
    hypercellularity thin, single contoured
    capillary walls and mesangial immune deposits.

58
Mesangioproliferative Glomerulonephritis
  • Clinically, it can present with varying degrees
    of proteinuria and, commonly, hematuria.

59
Mesangioproliferative Glomerulonephritis
  • Mesangioproliferative disease may be seen in
  • IgA nephropathy
  • P. falciparum malaria
  • Resolving postinfectious glomerulonephritis
  • Lupus nephritis

60
Mesangioproliferative Glomerulonephritis
  • There is little agreement on treatment, but some
    clinical reports suggest benefit from use of
  • Inhibitors of the renin-angiotensin system
  • Steroid therapy
  • Cytotoxic agents

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62
THE END
  • Questions?
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