Beruflich Dokumente
Kultur Dokumente
Renal Unit
Royal Hospital for Sick Children
Yorkhill Division
Please note: the following guideline has not been assessed according to the AGREE
(Appraisal of Guidelines for Research and Evaluation) criteria. This will take place at the
next guideline review.
Glomerulonephritis
Authors: Renal Clinicians Group
Revision date: Sept 2010
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 1 of 16
Issue Date: Sept 2008
Contents
1. Introduction
2. Acute Glomerulonephritis
2.1 Introduction
2.2 Investigations
2.3 Management
3. Henoch-Schonlein Purpura
3.1 Classification
3.2 Renal Involvement
3.3 Investigation
3.4 Management
3.5 Indications for Renal Biopsy
4. SLE Glomerulonephritis
4.1 Introduction
4.2 WHO Classification of Lupus Nephritis
4.3 Clinical Management
4.4 Drug Therapy
5. Mebranoproliferative Glomerulonephritis
5.1 Introduction
5.2 Management
5.3 Patients with Severe MPGN
6. Primary IGA Neuropathy
6.2 Management
7. Focal Glomerulosclerosis
7.1 Introduction
7.2 Management
8. Rapidly Progressive Glomerulonephritis
8.1 Introduction
8.2 Clinical Features
8.3 Management
9. Future Guideline Development
Glomerulonephritis
Authors: Renal Clinicians Group
Revision date: Sept 2010
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page Number(s)
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Issue Date: Sept 2008
1. Introduction
The following guideline has been developed and is regularly reviewed by clinicians
within the Renal Unit at Yorkhill. These guidelines are based on current evidence
and best practice relating to the Management of Glomerulonephritis.
This
document is intended for use by clinicians and nursing staff. For further discussion
of this guideline, please contact a consultant within the Renal Unit.
Urine
Imaging
FBC
U&Es
LFTs
Immunoglobulins
ASO titre (anti-DNAse B)
Compliment Screen
ANF
ANCA
Anti-GBM antibody titre (rarely indicated
Varicella and Hep B
Urine Protein Creatinine Ratio (PCR)
Urine Culture
Renal Ultrasound
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 3 of 16
Issue Date: Sept 2008
within a week and this is associated with blood pressure normalisation and a fall in
plasma creatinine. Frank haematuria may remain for 2-3 weeks but in the longer
term, proteinuria is usually gone by 3-6 months and microscopic haematuria by 1-2
years.
3. Henoch-Schonlein Purpura
3.1 Classification of HSP
Classification of Henoch-Schonlein Purpura Glomerulonephritis
(recommended by International Study of Kidney Disease in Childhood)
I.
II.
Minimal Changes
Pure mesangial proliferation without crescents
(a) Focal
(b) Diffuse
III.
IV.
V.
VI.
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Issue Date: Sept 2008
Nephritic
Hypertension
Haematuria
Proteinuria
Abnormal renal function
Discuss with
Nephrotic
Proteinuria > 200mg/mmol
of Creatinine
Hypoalbuminaemia
Oedema
Paediatric Nephrologist
Proteinuria (>100mg/mmol creatinine) haematuria
Hypertension
Abnormal renal function
Observe
Haematuria
Microscopic
Macroscopic
Minimal proteinuria
Management:
Glomerulonephritis
Authors: Renal Clinicians Group
Revision date: Sept 2010
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 5 of 16
Issue Date: Sept 2008
Classification of HSP
Grades I, IIa & IIb
Management
No treatment
ACE inhibitor if persistent proteinuria
Steroids as above
Cyclophosphamide 2.5mg/kg/day for 8 weeks
Consider Plasmapheresis
ACE inhibitor if persistent proteinuria
4. SLE Glomerulonephritis
4.1 Introduction
Renal manifestations are present in nearly two thirds of children with SLE and may
present with a combination of symptomatic hypertension, nephrotic syndrome or
gross haematuria. A wide range of extra renal manifestations is commonly
present and in some children the extra renal manifestations predominate. Often
the choice of therapy is influenced by potential renal or extra renal complications
of the regimens under consideration.
Glomerulonephritis
Authors: Renal Clinicians Group
Revision date: Sept 2010
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 6 of 16
Issue Date: Sept 2008
the
child
or
adolescent
2
with
severe
lupus
nephritis,
intravenous
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Issue Date: Sept 2008
6-12 months
12-24 months
36-48 months
48-60 months
2
2
2
At the end of each six month period the child should be assessed for treatment
failure as defined above and if present steroids should be withdrawn.
Glomerulonephritis
Authors: Renal Clinicians Group
Revision date: Sept 2010
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 8 of 16
Issue Date: Sept 2008
Current Guidelines
Suggested therapy is 6 months prednisolone @40mg/m2 alternate
days; if patients improve prednisolone should be weaned over a
period of up to 3 years.
The symptomatic treatment of proteinuria and/or hypertension with
ACE-I and/or ARB therapy is recommended although there is little
direct proof of efficacy in this paediatric patient group.
In addition the use of aspirin and dipyridamole should be considered
in adolescent patients.
Glomerulonephritis
Authors: Renal Clinicians Group
Revision date: Sept 2010
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 9 of 16
Issue Date: Sept 2008
of relapse
hypocomplementaemia
haematuria (return of/worsening)
worsening proteinuria
4. Other Therapies
a. Aspirin and dipyridamole
Antiplatelet therapy may be of benefit in adults patients, it could be
considered for adolescents [10]
b. ACE-I/ARBs
Prescription of these therapies is currently recommended in the
guideline. Although there is no direct proof of long term efficacy in
this paediatric patient group; there are however strong theoretic
reasons for prescription.
c. MMF/tacrolimus
The use of these immunosuppressant agents should be reserved for
the most difficult and severe cases. We have anecdotal evidence of
good efficacy; there is little literature. [11;12]
Reference List
1. Tarshish P, Bernstein J, Tobin JN, Edelmann CM, Jr. (1992) Treatment of
mesangiocapillary glomerulonephritis with alternate-day prednisone--a report
of the International Study of Kidney Disease in Children. Pediatr Nephrol 6
(2) : 123-130
2. Garcia-de la PS, Orozco-Loza IL, Zaltzman-Girshevich S, de Leon BB (2008)
Prognostic factors in children with membranoproliferative glomerulonephritis
type I. Pediatr Nephrol
Glomerulonephritis
Authors: Renal Clinicians Group
Revision date: Sept 2010
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 10 of 16
Issue Date: Sept 2008
Glomerulonephritis
Authors: Renal Clinicians Group
Revision date: Sept 2010
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 11 of 16
Issue Date: Sept 2008
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 12 of 16
Issue Date: Sept 2008
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 13 of 16
Issue Date: Sept 2008
Glomerulonephritis
Authors: Renal Clinicians Group
Revision date: Sept 2010
IgA nephropathy
Membranous GN
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 14 of 16
Issue Date: Sept 2008
Systemic Diseases
PSAGN
Shunt nephritis
Infective endocarditis
SLE
HSP
PAN
Wegeners granulomatosis
Cryglobulinaemia
be
classified
by
three
Immune
complex:
PSAGN,
HSPGN,
SLE,
MPGN,
MGN
and
Mixed
Cryoglobulinaemia. These conditions have granular immune deposition on IF and
this is the commonest observed pattern.
Pauci-immune: Vasculitis e.g. PAN, Wegeners and Idiopathic. These conditions
are typified by the absence of immune deposits on IF and ANCA positivity.
Anti-GBM antibody disease: This condition is typified by linear GBM staining and
the presence of circulating anti-GBM antibody and may be associated with antialveolar basement membrane antibody staining and pulmonary haemorrhage.
Complement levels
Hypocomplementaemia is present in SLE and MPGN but normal in other underlying
conditions.
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 15 of 16
Issue Date: Sept 2008
The following regime should be considered in patients who are ANCA positive and
negative
2
Glomerulonephritis
Authors: Renal Clinicians Group
Revision date: Sept 2010
Version: 1.2
Authorised by: Dr Jim Beattie
Q-Pulse ref: YOR-REN-017
Page 16 of 16
Issue Date: Sept 2008