Primary glomerulonephritides gn ii proliferative gn
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Primary glomerulonephritides (GN) II Proliferative GN. Miroslav Merta Klinika nefrologie 1. LF a VFN. Proliferative versus neproliferative GN (glomerular capillary loop – ultrastructural changes). Subendothelial deposits. Urinary space. Acute GN. Epitheliál cells=podocytes. Basal membrane.

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Primary glomerulonephritides gn ii proliferative gn
Primary glomerulonephritides (GN)II Proliferative GN

Miroslav Merta

Klinika nefrologie 1. LF a VFN


Proliferative versus neproliferative gn glomerular capillary loop ultrastructural changes
Proliferative versus neproliferative GN(glomerular capillary loop – ultrastructural changes)

Subendothelial deposits

Urinary space

Acute GN

Epitheliál cells=podocytes

Basal membrane

Increased number and proliferation of mesangial cells

neutrophils

Capillary lumen

Proliferative GN

Subepithelial deposits

Endotehelial cells

Mesangial cells

Subendothelial deposits

Increaed number and proliferation of mesangial cells to distal parts of capillary loop

Normal glomerulus

Increased number and proliferation of mesangial cells

neutrophils

mesangial deposits of IgA

Mesangioproliferative GN

Membranoproliferative GN


Dysmorphic erythrocytes of glomerular origine are typical finding in proliferative gn
Dysmorphic erythrocytes (of glomerular origine) are typical finding in proliferative GN

Urine sediment in phase contrast

Electroscanning microscopy


Acute postreptococal gn
Acute (postreptococal) GN finding in proliferative GN

Subendotelial deposits

neutrophils

Increased number and proliferation

Subepithelial (hump-like) deposits


Acute postreptococal gn1
Acute (postreptococal) GN finding in proliferative GN

Light microscopy (LM): picture of diffuse proliferative GN with prominent influx of neutrophils and event. Other cells, (= exsudative GN)

Imunofluorescence (IF): coarsely diffusely lightening of C3, event. IgG, rarely other

Three charakteristic types:

1) mesangial

2) „star sky

3) „girlands (associated with nephrotic proteinuria)

Elektrone microscopy (EM): proof of deposits subendothelially and condensation of cytoskeleton in adjacent epithelial cytoplasma. N = neutrophils


Akutn postreptokokov gn basic characteristics
Akutní (postreptokoková GN) finding in proliferative GN- basic characteristics

  • Its frequency is decreasing in Europe, m:f 2:1, common in children.

  • Acute GN is caused by „nefritogennic“ strains of STREP. pyogenes (infections of higher respiratory airways, event. cutaneous infections). Infection precedes PSGN for several weeks.

  • Patogenesis: antigens of STREP (např. GADH)  activation of complement  development of circulatory immuno complexes (event. Their deposition), persistation of STREP infection

  • Clinical picture: acute nephritic syndrome. Always present hematuria, often swlling (90%), hypertension (80%), proteinuria (in 30% NS),  GFR (83%).

  • Laboratory findings: findings reflecting presence of STREP infection titers of ASO (2x ), positivity of STREPTOZYME panel (involving antibodies against 4 STREP antigenes). Transitory  C3 (in 90%), event. CH50.

  • Histological picture: difuse endocapillary GN with proliferation of mesangium and endothel, typically exsudation (leukocytes). Rarely complicated by RPGN, cryoglobulinemia and s.o.)


Acute postreptococal gn diagnosis treatment prognosis
Acute (postreptococal GN) finding in proliferative GN–diagnosis, treatment, prognosis

  • Renal biopsy not routinely warranted; RB indicated: atypical course (NS), long-term  C3, important  GFR

  • Treatment involves: treatment of persistating STREP infection and treatment of nefritic syndrome:

    • Treatme of persistating STREP infection: PNC 1.2 M u./7-10 days

    • Treatment of nephritic syndrome: restriction of fluids and NaCl, diuretics, rarely hemodialysis (in 20-30% adults)

    • If complicated by RPGN - corticosteroids, (CPA?)

  • Prognosis: clinical manifestation is short (2 weeks), healing during weeks, mild proteinuria (< 0.5g/l) may persist for weeks and hematuriea even for 1 year. Long-term prognosis excellent.


Iga nephropathy igan mesangioproliferative glomerulonephritis
IgA nephropathy – IGAN finding in proliferative GNmesangioproliferative glomerulonephritis

Increaing number and proliferation of mesangial cells

Mesangial deposits of IgA, rarely IgA in capilars (serious forms)


Igan histological findings
IGAN- finding in proliferative GN histological findings

Light microscopy (LM): Focal (here) or diffuse mesangial proliferation. Rarely picture of crescentic GN, more frequently picture of sclerotisating GN. Vasculitic lesions occur in HSP.

Immunofluorescence (IF): diffuse mesangial deposits of IgA, co-localisation of other Ig less intensive

Electrone microscopy (EM): demonstation of deposits in mesangium


Patogenesis of iga nephropathy g mez guerrero et al kidney int 2002 62 715 717
Patogenesis of IgA nephropathy finding in proliferative GNGómez-Guerrero et al., Kidney Int, 2002, 62: 715 - 717

Impairment of O-glycosylation of IgA1 causes, that exposed domain GalNAc is recognized as antigennic


Iga nephropathy basic characteristics
IgA nephropathy finding in proliferative GN- basic characteristics

  • Commonest GN in Europe (20-40% of all primary GN)

  • Typical clinical picture – asymptomatic microskopic hematuria či episodes of parainfectious macroskopic hematuria

  • Natural course of the disease is not benign – at least in 20% patients ESRD develops within 20 years

  • Histology – mesangial deposits of IgA demonstrated by IF


Prognosis of igan negative prognostic factors
Prognosis of IgAN finding in proliferative GN– negative prognostic factors

a. klinical

hypertension

proteinuria (> 1 g/24 h)

decrease of GFR at the time of diagnosis

b. histological

glomerulosclerosis

interstitial fibrosis

vascular sclerosis


Prognosis of igan dependance on renal function and proteinuria at the time of diagnosis
Prognosis of IGAN finding in proliferative GN- dependance on renal function and proteinuria at the time of diagnosis

Dependance on protenuria

Dependance on renal function (creatininemia)

Radford et al., J Am Soc Nephrol, 1997, 8: 199 - 207


Igan treatment
IgAN finding in proliferative GN - treatment

  • Strict control of hypertension achieved preferentially with ACE inhibitors (event. ABR)

  • Fish oil (unsaturated alpha omega acids) in patients with slow progression of renal insufficiency (weak effect)

  • Cortikosteroids in patients with proteinuria, which do have preserved renal functions

  • Cytotoxic agents in patients s progressing renal insufficiency


Membranoproliferative glomerulonephritis type i a ii
Membranoproliferative glomerulonephritis finding in proliferative GN– type I a II

Subendothelal deposits

Mesangial sferic deposits

New formation of BM

Increasing number of mesangial cells and their proliferation to distal parts of capillary loop

neutrophils

Increasing number of mesangial cells and their proliferation to distal parts of capillary loop

neutrophils

Intramembranus dense deposits

MPGN type I

MPGN type II


Membranoproliferative gn type i
Membranoproliferative GN type I finding in proliferative GN

Light microscopy (LM): hypercelullarity and proliferation,, lobular appearance, double contour of BM (tram track) – as consequence of interposition of mesangium),

Immunofluorescence (IF): diffuse periferal granular deposits of IgG, event.IgM, and C3 (in type II only C3)

Electrone microscopy (EM): dense material substitutes BM

Electrone microscopy (EM): demonstration of deposits subendothelially and a mesangially, double contour of BM, leucocytes


Membranoproliferatie gn mpgn
Membranoproliferatie GN (MPGN) finding in proliferative GN

Type I

  • Idiopatic

  • Secondary

    – infection

    (visceral abscesses, endocarditis, infection of atrioventricular shunts, malaria)

    - systemic diseases

    ( SLE – LN type III-IV)

    - paraproteinemias

    ( LCDD, cryoglobulinemia)

    - thrombotic microangiopathy

    ( HUS/TTP, APS)

    Type II – disease of dense deposits


Idiopatic mpgn type i basic characteristics
Idiopatic MPGN type I finding in proliferative GN- basic characteristics

  • Relatively rare disease in developed countries

  • It is found in less aged subjects

  • Clinical findings at the time of dg: usually NS and microskopic hematuria

  • Slowly progressing disease – during 10-y interval 50% patients do progress into ESRD

  • Treatment in adults is controversial (corticosteroids, CPA, anticoagulation)


Membranoproliferative gn type ii
Membranoproliferative GN type II finding in proliferative GN

Electrone microscopy (EM): dense material substitutes BM


Idiopatic MPGN type II finding in proliferative GN- basic characteristics

  • Very rare disease

  • Clinically: presence of nephritic factor with hypocomplementemia

  • More pronounced nephritic features and more agressive course of diasease

  • Efficient treatment unknown


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