Академический Документы
Профессиональный Документы
Культура Документы
GLOMERULONEPHRITIS
Bancha Satirapoj, MD
Renal Division
Department of Medicine
Phramongkutklao hospital
Clinical Syndrome of Glomerular Diseases
Acute glomerulonephritis (AGN) An abrupt onset of glomerular hematuria (RBC cast and/or dysmorphic
RBCs) together with two or more of the following proteinuria, azotemia,
edema, oliguria, and recent onset hypertension
Nephrotic syndrome (NS) A syndrome of massive proteinuria (>3.5 g/day), with variable edema,
hypoalbuminuria, hyperlipidemia, and lipiduria
Rapidly progressive Any glomerular disease characterized by extensive crescents (usually >50%),
glomerulonephritis (RPGN) as the primary histologic finding and a rapid loss of renal function (usually a
50% decline in GFR within 3 months)
Asymptomatic urinary Isolated proteinuria (usually <2.0 g/day) or hematuria (with or without
abnormalities proteinuria)
Chronic glomerulonephritis Slowing developing renal failure accompanied by proteinuria, hematuria, and
hypertension
Rapidly progressive glomerulonephritis
Clinical syndrome:
Rapid loss of renal function
Glomerulonephritis
Aggressive glomerulonephritis:
extensive crescentric GN
Breaks in the glomerular basement membrane
Immunopathologic categories
ANCA GN
lung hemorrhage no lung hemorrhage Microscopic polyangiitis
IgA nephropathy Wegener Granulomatosis
H-S Purpura Churg-Struss Syndrome
Lupus nephritis
Post-infectious GN
Goodpasture Syndrome
Type 1 MPGN
Type 2 MPGN
Anti GBM GN Membranous GN
Frillary GN
40
30.68
30
20
10 6.82 7.95
0
ANCA Anti GBM ANCA+antiGBM Not specific
Systemic vasculitis
Yes No
60
40
20
0
WG MPA idiopathic CSS
RPGN
ANCA negative
up to 40 % of patients with limited WG (10 percent of those
with severe disease)
30 % of all MPA patients
50 % of all CSS patients
Positive ANCA serology
Clinical presentation of RPGN
PPV at least 98 %.
Adults with hematuria, proteinuria, and a serum creatinine of
less than 1.5 mg/dL
PPV was only 47 %
Monitoring
We suggest not changing immunosuppression based on
changes in ANCA titer alone. (2D)
KDIGO. Kidney International Supplements (2012) 2, 143153
Histopathologic Classification of ANCA-Associated GN
Yes
50% globally Sclerotic Class Inclusion criteria
sclerotic glomeruli class
Yes
50% normal Focal Crescentic 50% glomeruli with cellular crescents
glomeruli class
No
Mixed
class
Focal
0.8
Renal survival
Crescentic
0.6
Mixed
0.4
Sclerotic
0.2
0
0 2 4 6 8 10 12
Month
Renal relapse
1 Daily oral
Fifty patients were included in the
Pulse
study: 0.8
Patient survival, remission rate, time of remission, relapse rate, and outcome of
renal function were not different between the 2 treatment groups (N= 22 and 25)
0.6 P<0.01
0.4
Daily oral CYC
0.2
0
0 2 4 6 8 10 12
month
0.6
0.4
Daily oral
Pulse
0.2
0
2.0 4.0 0 6.0 8.0 10.0
Time to Remission , mo
No difference in the time to remission or the percentage of remission by nine months (88
% in both groups)
de Groot K; et al. Ann Intern Med. 2009;150(10):670-80.
Pulse Versus Daily Oral Cyclophosphamide for Induction of Remission
in ANCAAssociatedVasculitis: A Randomized Trial
Pulse cyclophosphamide
Lower cumulative cyclophosphamide dose (8.2 versus 15.8 g)
Lower rate of leukopenia (26 versus 45 percent)
PE
0.2
0
0 2 4 6 8 10 12
Months from entry
0.6
Generalized vasculitis, the withdrawal of cyclophosphamide and the
substitution of azathioprine after remission did not increase the
0.0
rate of relapse. 3 6 9 12 15 18
Months from entry
0.8 Methotrexate
0.7
0.6
0.5
Azathioprine
0.4
0.3
0.2
0.1
0
0 6 12 18 24 30 36
Months since randomization
Pagnoux C; et al. N Engl J Med. 2008; 359(26):2790-803.
Induction of remission in active ANCA-
associated vasculitis with MMF in patients who
cannot be treated with cyclophosphamide.
4
Serum creatinine (mg/dL)
77.8% treated with 3.5
MMF CTX
MMF 3
47.1% treated with 2.5
CTX 2
Complete remission 1.5
with an absolute 1
difference of 30.7%. 0.5
0
0 3 mo 6 mo
Addition of plasmapheresis
Receive the same initial treatment as those with pure anti-GBM disease
Phramongkutklao Hospital
and College of Medicine