This document outlines treatment protocols for rapidly progressive glomerulonephritis (RPGN) in children at Mansoura University Children's Hospital in Egypt. It defines RPGN and lists inclusion criteria. Four treatment protocols are provided: Protocol I involves steroids, Protocol II adds cyclophosphamide, Protocol III uses lower doses of cyclophosphamide and adds azathioprine, and Protocol IV focuses on plasma exchange with steroids and cyclophosphamide. Each protocol provides details on drug doses, administration schedules, and monitoring of patients. The goal is to control inflammation during induction and prevent further kidney damage and relapses during maintenance therapy.

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)