Protocol of-rpgn2014 (1)

1. MANSOURA UNIVERSITY CHILDREN’S HOSPITAL
2014
Protocols of management
of Rapidly Progressive
Glomerulonephritis
Pediatric Nephrology Unit

2. ‫المنصــورة‬ ‫جامعــة‬Mansoura University
‫األطفـال‬ ‫مستشفـي‬Children's Hospital
‫الكلي‬ ‫أمراض‬ ‫وحدة‬Nephrology Unit
Updated April 2014
Definitions and inclusion criteria
Definitions:
A clinico-pathologic condition that is characterized by a
rapid deterioration of renal function (>50% decrease in
GFR) and demonstration of ‘crescents’ affecting at least
50% of the glomeruli in an adequatebiopsy specimen.
Inclusion criteria:
*Rapid deterioration of kidney function:
 Duplicationof creatinine within 24 hs. or
 Creatinine >50% of normal.or
 Serum cretinine > 1.8 mg/dl (1st
encountered or follow up).
*Crescents affecting at least 50% of the glomeruli.
Classification:
(Pauci –immune crescentric GN )
DrugsIdiopathic

3. ‫المنصــورة‬ ‫جامعــة‬Mansoura University
‫األطفـال‬ ‫مستشفـي‬Children's Hospital
‫الكلي‬ ‫أمراض‬ ‫وحدة‬Nephrology Unit
Updated April 2014
RPGN without crescents:
 Hemolytic uremic syndrome.
 Acute interstitial nephritis.
 Diffuse proliferative GN.
Investigations:
 CBC (blood film and RC if HUS is suspected).
 Creatinine/24 hs.
 Serum albumin,ABG, Na, K.
 Urine analysis, urinary protein /creatinine .
 Streptococcal antibodytiters (ASO).
 Culture of throat and skin lesions
 C3, C4.
 Renal ultrasound.
 If diagnosisof APSGN ruled out do further investigations:
 Viral hepatitismarkers.
 ANA, antidsDNA.
 ANCA.
 Anti-GBM antibodytiter (if pulmonary involvement).
 Renal biopsy.
Treatment: “Aim”
 Induction phase: to control inflammationand associated
immune response.
 Maintenance phase: prevent further renal damage and
relapses.

4. ‫المنصــورة‬ ‫جامعــة‬Mansoura University
‫األطفـال‬ ‫مستشفـي‬Children's Hospital
‫الكلي‬ ‫أمراض‬ ‫وحدة‬Nephrology Unit
Updated April 2014
Crescentic glomerulonephritis
Granular immune complex deposits
C3 normal or low; ANCA negative
Scarce or absent immune deposits
ANCA positive; C3 normal
Linear anti-GBM antibodies
ANCA negative; C3 normal
Other Postinfectious GN
IgA nephropathy
Lupus nephritis
Henoch schonlein purpura
Membranoproliferative GN
Microscopic polyangitis
Wegener’s granulomatosis
Renal limited vasculitis
Churg Strauss syndrome
Goodpasture’s syndrome
Anti GBM nephritis
IV/oral corticosteroids
IV/oral
Cyclophosphamide
Treat primary condition
Treat infection
Plasma exchange
Protocol II
Protocol
III
Protocol IV
Induction:
IV/oral corticosteroids
IV/oral Cyclophosphamide
Plasma exchange
Maintenance:
Oral corticosteroids
Azathioprine , alternatively
MMF, methotrexate.
Monitor for relapses.
Plasma exchange.
IV/oral corticosteroids
Oral
Cyclophosphamide
APSG
N
Protocol I
IV/oral
corticosteroids
±
IV/oral
Cyclophosphamide
Plasma exchange.

5. ‫المنصــورة‬ ‫جامعــة‬Mansoura University
‫األطفـال‬ ‫مستشفـي‬Children's Hospital
‫الكلي‬ ‫أمراض‬ ‫وحدة‬Nephrology Unit
Updated April 2014
Protocol of RPGN
Protocol I (APSGN)
1. Steroid therapy:
Protocol Date Dose
1- Methylprednisolone (MP):
15-20mg/kg/dose (not exceed 1gm) IV daily 3-6
doses.
1st dose
2nd dose
3rd dose
4th dose
5th dose
6th dose
/ /
/ /
/ /
/ /
/ /
/ /
……………...
……………...
……………...
……………...
……………...
……………...
2- Followed by oral prednisolone:
2mg/kg/day (on 3 divided doses) for 1 month / / …………tab/day
3-Revaluation when result of biopsy is available clinical and laboratory + higher stuff
consultation to give Endoxan or not.
Clinical:
Biopsy result:
Urine analysis:
Serum creatinine: C3:
Consultant: Decision: Date:
4- Then taper oral steroid as follow:
1.5 mg/kg/day for 1 month
1. mg/kg/day for 1 month
0.5 mg/kg/day for 1 month
0.5 mg/kg/EOD for 3 months
1st month
2nd month
3rd month
4th month
5th month
6th month
/ /
/ /
/ /
/ /
/ /
/ /
………tab/EOD
………tab/EOD
……..tab/EOD
……..tab/EOD
……..tab/EOD
……..tab/EOD
2. Cyclophosphamide: (after biopsy result, re-evalution and consultation)
1st
month 2nd
month 3rd
month
Dose:……….……………………
Date:…………/..………/………
Dose:……….……………………
Date:…………/..………/………
Dose:……….……………………
Date:…………/..………/………
4th
month 5th
month 6th
month
Dose:……….……………………
Date:…………/..………/………
Dose:……….……………………
Date:…………/..………/………
Dose:……….……………………
Date:…………/..………/………
The dose should be adjusted to maintain a nadir leukocyte count,
2 weeks post treatment, of 3000 to 4000/mm3
3. Plasma exchange.

6. ‫المنصــورة‬ ‫جامعــة‬Mansoura University
‫األطفـال‬ ‫مستشفـي‬Children's Hospital
‫الكلي‬ ‫أمراض‬ ‫وحدة‬Nephrology Unit
Updated April 2014
Protocol of RPGN
Protocol II
1. Steroid therapy:
Protocol Date Dose
1- Methylprednisolone (MP):
15-20mg/kg/dose (not exceed 1gm) IV daily 3-6
doses.
1st dose
2nd dose
3rd dose
4th dose
5th dose
6th dose
/ /
/ /
/ /
/ /
/ /
/ /
……………...
……………...
……………...
……………...
……………...
……………...
2- Followed by oral prednisolone:
2mg/kg/day (on 3 divided doses) for 1 month / / ………tab/day
4- Then taper oral steroid as follow:
1.5 mg/kg/day for 1 month
1. mg/kg/day for 1 month
0.5 mg/kg/day for 1 month
0.5 mg/kg/EOD for 3 months
1st month
2nd month
3rd month
4th month
5th month
6th month
/ /
/ /
/ /
/ /
/ /
/ /
……tab/EOD
……tab/EOD
……tab/EOD
……tab/EOD
……tab/EOD
……tab/EOD
2. Cyclophosphamide: at the beginning of oral steroid therapy.
Intravenous: 500-750mg/m2
IV monthly:
1st
month 2nd
month 3rd
month
Dose:……….……………………
Date:…………/..………/………
Dose:……….……………………
Date:…………/..………/………
Dose:……….……………………
Date:…………/..………/………
4th
month 5th
month 6th
month
Dose:……….……………………
Date:…………/..………/………
Dose:……….……………………
Date:…………/..………/………
Dose:……….……………………
Date:…………/..………/………
The dose should be adjusted to maintain a nadir leukocyte count,
2 weeks post treatment, of 3000 to 4000/mm3
3. Plasma exchange.

7. ‫المنصــورة‬ ‫جامعــة‬Mansoura University
‫األطفـال‬ ‫مستشفـي‬Children's Hospital
‫الكلي‬ ‫أمراض‬ ‫وحدة‬Nephrology Unit
Updated April 2014
Protocol of RPGN
Protocol III
1. Steroid therapy:
Protocol Date Dose
1- Methylprednisolone (MP):
15-20mg/kg/dose (not exceed 1gm) IV daily 3-6
doses.
1st dose
2nd dose
3rd dose
4th dose
5th dose
6th dose
/ /
/ /
/ /
/ /
/ /
/ /
……………...
……………...
……………...
……………...
……………...
……………...
2- Followed by oral prednisolone:
2mg/kg/day (on 3 divided doses) for 1 month / / ………tab/day
3- Then taper oral steroid as follow:
1.5 mg/kg/day for 1 month
1. mg/kg/day for 1 month
0.5 mg/kg/day for 1 month
0.5 mg/kg/EOD for 3 months
1st month
2nd month
3rd month
4th month
5th month
6th month
/ /
/ /
/ /
/ /
/ /
/ /
……tab/EOD
……tab/EOD
……tab/EOD
……tab/EOD
……tab/EOD
……tab/EOD
2. Cyclophosphamide: at the beginning of oral steroid therapy.
Oral : 2 mg/kg/day for 3 months (Endoxan 50 mg/tablet)
Intravenous: 500-750mg/m2
IV monthly for 3 months:
1st
month 2nd
month 3rd
month
Dose:……….……………………
Date:…………/..………/………
Dose:……….……………………
Date:…………/..………/………
Dose:……….……………………
Date:…………/..………/………
The dose should be adjusted to maintain a nadir leukocyte count,
2 weeks post treatment, of 3000 to 4000/mm3
3. Azathioprine (after endoxan) 2mg/kg/day for 3 months (Immuran
50mg/tablet):
4. Plasma exchange.

8. ‫المنصــورة‬ ‫جامعــة‬Mansoura University
‫األطفـال‬ ‫مستشفـي‬Children's Hospital
‫الكلي‬ ‫أمراض‬ ‫وحدة‬Nephrology Unit
Updated April 2014
Protocol of RPGN
Protocol IV
1. Plasma exchange.
2. Steroid therapy(concomitant with plasmapheresis):
Protocol Date Dose
1- Methylprednisolone (MP):
15-20mg/kg/dose (not exceed 1gm) IV daily 3-6
doses.
1st dose
2nd dose
3rd dose
4th dose
5th dose
6th dose
/ /
/ /
/ /
/ /
/ /
/ /
……………...
……………...
……………...
……………...
……………...
……………...
2- Followed by oral prednisolone:
2mg/kg/day (on 3 divided doses) for 1 month / / ………tab/day
3- Then taper oral steroid as follow:
1.5 mg/kg/day for 1 month
1. mg/kg/day for 1 month
0.5 mg/kg/day for 1 month
0.5 mg/kg/EOD for 3 months
1st month
2nd month
3rd month
4th month
5th month
6th month
/ /
/ /
/ /
/ /
/ /
/ /
……tab/EOD
……tab/EOD
……tab/EOD
……tab/EOD
……tab/EOD
……tab/EOD
3.Cyclophosphamide: at the beginning of oral steroid therapy.
Oral : 2 mg/kg/day for 6 months (Endoxan 50 mg/tablet)