Syndrome néphrotique

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Janvier 2012

Syndrome Néphrotique

Recommandations de Bonnes Pratiques Médicales

A!ection Longue DuréeALD 34

CIM 10: N04Code ANAM : L006

Réf : 0.3.90.01

N° Dépôt Légal : 2012 MO 0957ISBN : 978 - 9954 - 0 - 8551 - 6

ISSN : 2028 - 7720

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SOMMAIRE

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3. Recommandations thérapeutiques3.1. Traitement symptomatique

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4. Modalités de suivi

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Texte

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

GROUPE DE TRAVAIL COMITE DE LECTURE

COMITE DE SUIVI

Pr. Khadija Hachim, néphrologue, CHU, Casablanca Présidente du groupe de travail

Dr. Bachir Mounib, néphrologue, secteur libéral, AgadirRapporteur du groupe de travail

Dr. Mohamed El Mehdi, néphrologue, secteur libéral, Casablanca

Dr. Asmaa Laaboudi, néphrologue, secteur public, Rabat

Dr. Rajaa Rochdi, néphrologue, secteur public, Rabat

Dr. Mia Hajji, Médecin interniste, secteur libéral, Casablanca

Pr. Saïda Sqalli, anatomopathologiste, secteur libéral, Casablanca

Pr. Mohamed BENGHANEM GHARBI, néphrologue, CHU, Casablanca

Dr. Lahcen NACIRI GAGA, Médecin interniste, secteur libéral, Agadir

Dr. Nezha SALAM, néphrologue, secteur public, Taroudant

Dr. Moha MALGHI, pédiatre, secteur public, Tiznit

Dr. Omar CHAFCHAOUNI, médecin généraliste, secteur libéral, Anza

Pr. Benyounès Ramdani, néphrologue, CHU, Casablanca Président du comité de suivi

Dr. Amal Amrani Halhoul, néphrologue, secteur public, Tanger

Pr. Yamama Amar, néphrologue, secteur libéral, Rabat

Dr. Fatima Boudribila, néphrologue, secteur libéral, Salé

Pr. Elmustapha Fatihi, néphrologue, secteur libéral, Casablanca

Dr. Fatine Hachem Reda, néphrologue, secteur libéral, Casablanca

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Liste des abréviations P. 23 1.Introduction P. 24

1.1. Objet P. 241.2. Processus/méthodologie P. 24

1.2.1. Cadre réglementaire P. 241.2.2. Méthodologie générale P. 251.2.3. Groupe de travail P. 251.2.4. Con#its d’interêt P. 251.2.5. Recherche bibliographique P. 251.2.6. Groupe de lecture P. 261.2.7. Approbation du comité de coordination de la SMSM P. 261.2.8. Validation P. 261.2.9. Approbation dé"nitive P. 271.2.10. Procédure de mise à jour P. 271.2.11. Financement P. 27

2.Présentation de la maladie P. 272.1. Dé"nition de la maladie P. 272.2. Situation épidémiologique P. 28

2.2.1. Prévalence P. 282.2.2. Incidence P. 282.2.3. Sexe ratio P. 282.2.4. Age de début P. 282.2.5. Relation atopie-syndrome néphrotique P. 282.2.6. Données épidémiologiques sur la cortico-sensibilité P. 29

2.3. Physiopathologie P. 292.4. Diagnostic positif P. 30

2.4.1. L’existence d’un syndrome néphrotique biologique P. 302.4.2. Une enquête étiologique négative P. 312.4.3. La con"rmation histologique P. 31

2.5. Diagnostic di!érentiel P. 332.6. Complications P. 34

2.6.1. L’état d’anasarque P. 342.6.2. L’hyperlipidémie P. 342.6.3. Les complications thrombo-emboliques P. 342.6.4. Les infections P. 352.6.5. Autres complications P. 35

2.7. Evolution de la maladie P. 36

SOMMAIRE

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3.Recommandations thérapeutiques : le syndrome néphrotique idiopathique P. 38

3.1. Traitement symptomatique P. 383.1.1. Le régime P. 383.1.2. Diurétiques P. 383.1.3. Traitement anti-coagulant P. 393.1.4. Vitamine D - Calcium P. 403.1.5. Statines P. 413.1.6. Prévention et traitement des infections P. 413.1.7. Hormone de croissance P. 423.1.8. Traitement anti-protéinurique P. 423.1.9. Traitement anti-hypertenseur P. 43

3.2. Traitement étiopathogénique P. 433.2.1. Formes cortico-sensibles P. 43

3.2.1.1. Première cure thérapeutique P. 433.2.1.2. Traitement des rechutes P. 443.2.1.3. Formes cortico-dépendantes P. 45

Le lévamisol P. 46Les agents alkylants P. 46La ciclosporine A P. 47Les anti-métabolites P. 48

3.2.2. Formes cortico-résistantes P. 483.2.2.1. Ciclosporine A P. 493.2.2.2. Tacrolimus P. 493.2.2.3. Sirolimus P. 503.2.2.4. Les anti-métabolites P. 503.2.2.5. Rituximab P. 503.2.2.6. Les agents alkylants P. 503.2.2.7. Autres traitements immunosuppresseurs P. 503.2.2.8. Thérapeutiques non immunosuppressives P. 50

3.2.3. Traitement étiopathogénique : conclusions P. 513.2.3.1. Dans les formes cortico-sensibles P. 513.2.3.2. Dans les formes primitives des néphroses cortico-résistantes P. 51

3.3. Implications socio-économiques P. 523.3.1. Coût approximatif du traitement médical P. 523.3.2. Coût approximatif du traitement par hémodialyse P. 533.3.3. Coût de la transplantation rénale 1ère année P. 53

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3.3.4. Implications sociales P. 533.4. Limites en contre-indications du traitement P. 53

4.Modalités du suivi P. 544.1.Evaluation de l’e$cacité et de la tolérance du traitement P. 54

4.1.1. Surveillance clinique P. 544.1.2. Surveillance paraclinique P. 54

4.2. Professionnels impliqués dans le suivi P. 544.3. Date d’arrêt du traitement P. 55

5.Recommandations aux patients P. 565.1. Education thérapeutique P. 565.2. Modi"cation du mode de vie P. 56

6.Références bibliographiques P. 577.Annexes P. 63

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83

LISTE DES ABREVIATIONS

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84

1.Introduction 1.1. Objet19CNP>@,A>,K>O,J>KC??:=A:@DC=O,>O@,A>,?>@@J>,W,ADOICOD@DC=,A>O,IJCT>OODC==>BO,A>,

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1.2.Processus/méthodologie 1.2.1.Cadre réglementaire 1:,KC=;>=@DC=,A>,I:J@>=:JD:@,ODU=M>S,B>,,Hc,r:=;D>J,,8HHjS,>=@J>,B>,%D=DO@<J>,A>,B:,+:=@MS,,B9&U>=K>,,':@DC=:B>,,A>,,B9&OOLJ:=K>,,%:B:AD>,,>@,,B:,,+CKDM@M,,%:JCK:D=>,,A>O,+KD>=K>O,,%MADK:B>O,,`+%+%aS,,>@,,D=@MUJM>,,I:J,,B>,,#C=O>DB,,':@DC=:B,,A>,,B9$JAJ>,,

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1.2.5. Recherche bibliographique

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Tableau I. Grade des recommandations

Niveau de preuve scienti!que fourni par la littérature(études thérapeutiques)

Gradedes recommandations

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APreuve scienti"que

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1.2.11. Financement

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2.2.6. Données épidémiologiques sur la cortico-sensibilité1>O,AC==M>O,A>,B:,BD@@MJ:@LJ>,?C=@J>=@,A>O,@:L\,A>,KCJ@DKCJMODO@:=K>,>=@J>,8b,>@,

2.3. Physiopathologie1>,+',>O@,K:J:K@MJDOM,I:J,B>,I:OO:U>,A:=O,B9LJD=>,A>,IJC@MD=>O,A>,ICDAO,?CBMKLB:DJ>,OLIMJD>LJ,W,jH,n(:,>=,VL:=@D@M,D?ICJ@:=@>R,(>L\,?MK:=DO?>O,I>L;>=@,>\IBDVL>J,K>,I:OO:U>,5

>=, ?DKJCOKCID>, CI@DVL>R, 1:, IJC@MD=LJD>, >O@, :BCJO, OMB>K@D;>, `KC=O@D@LM>,IJD=KDI:B>?>=@,A9:BNL?D=>aR,#>,?>K:=DO?>,>O@,D?IBDVLM,A:=O,B>,+',W,BMODC=O,UBC?MJLB:DJ>O,?D=D?>OR

>=,?DKJCOKCID>,CI@DVL>,>=@J:d=:=@,B>,I:OO:U>,A>,IJC@MD=>O,A>,ICDAO,?CBMKLB:DJ>,OLIMJD>LJ,W,YFH,n(:R,1:,IJC@MD=LJD>,>O@,:BCJO,=C=,OMB>K@D;>R,#>,?MK:=DO?>,>O@,D?IBDVLM,A:=O,B>O,:L@J>O,@QI>O,A>,+'R

A>,KCJ@DKCJMODO@:=K>R

1:,TLD@>,?:OOD;>,A>,IJC@MD=>O,A:=O,B>O,LJD=>O,:LJ:,KC??>,KC=OMVL>=K>O,5

0=>,AD?D=L@DC=,A>,B:,IJ>OODC=,C=KC@DVL>,J>OIC=O:NB>,>=,I:J@D>,A>O,C>A<?>OS,

T:K@>LJO,5,

@:L\,A>,IB:VL>@@>Oa,

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

3H

D??CNDBDO:@DC=aR

"@, A:=O, B>O, TCJ?>O, IJCBC=UM>OS, A>O, KC?IBDK:@DC=O, A>, B:, TLD@>, IJC@MDVL>, >@, A>O,OLNO@:=K>O,BDM>O,:L\,IJC@MD=>O,5,AM=L@JD@DC=S,O>=ODNDBD@M,:L\,D=T>K@DC=OS,:=M?D>,>@K

2.4. Diagnostic positifRecommandation n°31>,AD:U=CO@DK,ICOD@DT,>O@,J>@>=L,A>;:=@5

0=>,>=VLX@>,M@DCBCUDVL>,=MU:@D;>

&KKCJA,IJCT>OODC==>B

,2.4.1. L’existence d’un syndrome néphrotique biologique

Recommandation n°41>O,>\:?>=O,NDCBCUDVL>O,W,;DOM>,AD:U=CO@DK,OC=@,B>O,OLD;:=@O5,


Recommandation n°5

'L?MJ:@DC=,TCJ?LB>,O:=ULD=>],7M?CKLB@LJ>O, a

*C=CUJ:??>,O:=ULD=,>@,LJD=:DJ>R


!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

3Y

2.4.2. Une enquête étiologique négative

Recommandation n°6


(GIM=DKDBB:?D=>,S,?:B:AD>,A97CAUfD=S,IDVsJ>,A9D=O>K@>,>@K Ra

ICBQOMJD@>S,AD:N<@>S,@L?>LJ,>@K^,

"=, TC=K@DC=, A>O, AC==M>O, A>, B9>=VLX@>, M@DCBCUDVL>, IJMBD?D=:DJ>, , B>O, >\:?>=O,NDCBCUDVL>O, KC?IBM?>=@:DJ>O, A>, AMNJCLDBB:U>, O>JC=@, A>?:=AMO, h&KKCJA,IJCT>OODC==>B,i,5,,

0=,NDB:=,D??L=CBCUDVL>,5,M@LA>,AL,KC?IBM?>=@,OMJDVL>,>@,A>,O>O,KC?ICO:=@OS,B>O,,:=@DKCJIO,:=@DG=LKBM:DJ>OS,B>O,:=@DKCJIO,:=@D,('&S,>@K^,

IJC@MD=>,?C=CKBC=:B>R

(9:L@J>O,>\:?>=O,I>L;>=@,X@J>,A>?:=AMO,>=,TC=K@DC=,AL,KC=@>\@>

2.4.3. La con"rmation histologiqueRecommandation n°7(>;:=@,@CL@,+',B:,NDCIOD>,JM=:B>,>O@,D=ADVLM>5

+',D?ILJS,+',KCJ@DKCJMODO@:=@IJMO>=K>,A>,ODU=>O,>\@J:,JM=:L\,&U>k,,Y:=,CL,l,YY,:=O&;:=@,AM?:JJ:U>,A>,B:,KDKBCOICJD=>,&R


!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

38

Recommandation n°81>O,KC=@J>GD=ADK:@DC=O,A>,B:,NDCIOD>,JM=:B>,OC=@,5

,1>,J>D=,L=DVL>

.JMO>=K>,A9L=>,@L?>LJ,JM=:B>

,1>,J>D=,ICBQfQO@DVL>


Recommandation n°9 :0=,,NDB:=,IJMGNDCIOD>,JM=:B>,ACD@,X@J>,IJ:@DVLM,5


3 types histologiques sont possibles :

,>=,?DKJCOKCI>,CI@DVL>S,A>O,UBC?MJLB>O,IJ:@DVL>?>=@,=CJ?:L\S,

,>=,?DKJCOKCI>,MB>K@JC=DVL>S,B:,TLODC=,A>O,ID>AO,A>O,ICACKQ@>OS,

A>O, I:JCDO, K:IDBB:DJ>OS, A:=O, K>J@:D=>O, :=O>O, O>LB>?>=@, >@, A:=O, K>J@:D=O,UBC?MJLB>O,O>LB>?>=@R

,>=,?DKJCOKCI>,MB>K@JC=DVL>S,B9>TT:K>?>=@,A>O,ID>AO,A>O,ICACKQ@>OS,

.BLO, J:J>?>=@S, IJCBDTMJ:@DC=,?MO:=UD:B>, ADTTLO>, .%(, 5, =>, O9:KKC?I:U=:=@,=D, A9:B@MJ:@DC=O, A>O, I:JCDO, K:IDBB:DJ>O, =D, A>, AMIe@O, >\@J:?>=NJ:=>L\,?:DO,

L=,T:K@>LJ,IJ>AMK@DT,A>,KCJ@DKCJMODO@:=K>R5

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

33

2.5.Diagnostic di!érentiel


,A>O,:L@J>O,K:LO>O,A>,OQ=AJC?>O,C>AM?:@>L\,5

>@,B>O,IMJDK:JAD@>O,KC=O@JDK@D;>O,VLD,I>L;>=@,MU:B>?>=@,O9:KKC?I:U=>J,A9L=>,IJC@MD=LJD>R


D=T>K@D>LO>S,,@L?CJ:B>,CL,@C\DVL>,>@K R

L=,+',>O@,AD@,IJD?D@DT, OD, B9>=VLX@>,M@DCBCUDVL>,O>, JM;<B>,=MU:@D;>R,1>,+',>O@,

?>?NJ:=>LO>53FbS, 1MODC=O, UBC?MJLB:DJ>O, ?D=D?>O, 5, YFbS, 7Q:BD=CO>,

YHba

IJCBDTMJ:@D;>,a

AL,K:AJ>,A>,K>@@>,J>KC??:=A:@DC=],K>O,TCJ?>O,OC=@,J:J>O,>@,B:,NDCIOD>,JM=:B>,Q,>O@,

ADTTLO>,5

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

34

A>,ADB:@:@DC=,?DKJC,fQO@DVL>,A>O,@LNLB>O,JM=:L\R,"BB>,>\DO@>,A<O,B:,;D>,T[@:B>,>@,

JM=:B>, OLJ;D>=@, :;:=@, 3, :=OS:KKC?I:U=M>, A>, @JCLNB>O, ADU>O@DTO, D?ICJ@:=@O,>@, A>, KC?IBDK:@DC=O, =L@JD@DC==>BB>O, >@, D=T>K@D>LO>OR, #9>O@, L=>, D=ADK:@DC=, A>,@J:=OIB:=@:@DC=,JM=:B>R,

1:,OKBMJCO>,?MO:=UD:B>,ADTTLO>

D=O>=ODNB>,W,@CLO,B>O,@J:D@>?>=@OR

2.6. Complications

=C?NJ>LO>O,KC?IBDK:@DC=OS,I:JTCDO,AJ:?:@DVL>O,5

2.6.1. L’etat d’anasarque 1>O,C>A>?>O,I>L;>=@,X@J>,@J<O,D?ICJ@:=@O,`:=:O:JVL>a,>@,>=@J:d=>J,A>O,@JCLNB>O,K:JADCGJ>OIDJ:@CDJ>O,CL,A>O,KJDO>O,ACLBCLJ>LO>O,:NAC?D=:B>OR,*BO,OC=@,BDMO,W,L=>,JM@>=@DC=,IJD?D@D;>,AL,OCADL?,>@,W,B:,N:DOO>,A>,B:,IJ>OODC=,C=KC@DVL>,,IB:O?:@DVL>,,

2.6.2. L’hyperlipidémie

AMI:OO>J,,YH,??CBgBR,"BB>,>O@,ADJ>K@>?>=@,KCJJMBM>,W,B:,KB:DJ:=K>,A>,B9:BNL?D=>R,1>O,

OM;<J>,AL,+',?:PCJ>,B>,JDOVL>,K:JADC;:OKLB:DJ>,>@,I>L@,:UUJ:;>J,B:,IJCUJ>OODC=,A>O,BMODC=O,JM=:B>OÈYa,È8a,R

2.6.3. Les complications thromboemboliques

>O@, L=>, KC?IBDK:@DC=, ND>=, KC==L>, Èja, S, >BB>,?>@, >=, P>L, B>, IJC=CO@DK, ;D@:BR, 1>O,

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

3F

J>;L>,A>, B:, BD@@MJ:@LJ>, @JCL;>,L=,oU>,?MAD:=,A>,YH,:=O,:;>K,A>O,oU>O,:BB:=@,A>,

J>IJMO>=@>=@,VL>,8H,W,8Fb,`F3a,R,1>O,8,BCK:BDO:@DC=O,;>D=>LO>O,B>O,IBLO,TJMVL>=@>O,OC=@,B>O,;>D=>O,JM=:B>O,>@,B>O,;>D=>O,IJCTC=A>O,A>O,?>?NJ>OR

I:J@DKLBD>J, , >=, K:O, A>, +', D=@>=O>, `:BNL?D=M?D>, k8H, UgB, ;CDJ>, 8F, UgBa, `FFa, `FEa,`FcaR1>,JDOVL>,A9>?NCBD>,ILB?C=:DJ>,>O@,:BCJO,I:J@DKLBD<J>?>=@,MB>;MR,1>O,TCJ?>O,

OC=@,IBLO,TJMVL>=@>O,VL>,B>O,TCJ?>O,JM;MBM>O,I:J,A>O,ODU=>O,KBD=DVL>O,5,ACLB>LJO,BC?N:DJ>OS, 7M?:@LJD>, ?:KJCOKCIDVL>S, :UUJ:;:@DC=, NJL@:B>, A>, B:, IJC@MD=LJD>,

Y8bR,

OIBM=CICJ@:B>S,:\DBB:DJ>S,OD=LO,KMJMNJ:L\a,C=@,M@M,MU:B>?>=@,AMKJD@>O,`FFaR

2.6.4. Les infections

:B@>J=>O,AL,KC?IBM?>=@,`FmaR,1>,JDOVL>,A9D=T>K@DC=,I:J,B>O,N:K@MJD>O,>=K:IOLBM>O,

I=>L?CKCVL>,M@:D@,:L@J>TCDO,L=>,K:LO>,D?ICJ@:=@>,A>,?CJ@:BD@M

2.6.5. Autres complications 1>O,@:L\,IB:O?:@DVL>O,A9L=,K>J@:D=,=C?NJ>,A>,IJC@MD=>O,A>,BD:DOC=,OC=@,:N:DOOMO,

OC=@,AD?D=LM>O,5

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

3E

1:, I>J@>, A>, B:, @J:=OT>JJD=>, I>L@, KC=ALDJ>, W, L=>, :=M?D>, ?DKJCKQ@:DJ>,

1:, N:DOO>, A>, B9:BNL?D=>, OMJDVL>, >O@, J>OIC=O:NB>, A>, B9:LU?>=@:@DC=, A>, B:,TJ:K@DC=,BDNJ>,A>O,?MADK:?>=@OS,=C@:??>=@,A>O,:=@D,;D@:?D=>O,nS,A>O,&*'+^1>, JDOVL>, A>, OLJACO:U>, >@, A9>TT>@, @C\DVL>, >O@, :LU?>=@MR, , 1:, ?:B=L@JD@DC=,

2.7. Evolution de la maladie

`?CD=O,A>,YHb,A>O,K:OaS,M;>=@L:BD@M,VLD,,=>,ACD@,I:O,ADOI>=O>J,AL,AM?:JJ:U>,A>,B:,

KCJ@DKCO>=ODNDBD@M,OCD@,I:J,L=>,KCJ@DKCJMODO@:=K>R

2.7.1 Une corticosensibilité :

2.7.1.1 - avec rémission complète ?8, CL, @J:K>O, W, B:, N:=A>B>@@>, LJD=:DJ>, , I>=A:=@, 3, PCLJO, KC=OMKL@DTOR, h&KKCJA,IJCT>OODC==>Bi

2.7.1.2 - avec corticosensibilité partielle IJC@MD=LJD>gKJ>:@D=D=LJD>, >=@J>, Y, >@, 3, >=,UgU, a, >@, L=>, :BNL?D=M?D>,l, 8F, UgBR, h,&KKCJA,IJCT>OODC==>B,i

2.7.1.3 - avec une ou plusieures rechutes :

A>,ICDAO a, >@gCL,NDCBCUDVL>, , CL,I:J, B:, JM:II:JD@DC=,A9L=>,IJC@MD=LJD>, DOCBM>, `,

>=,Ug??CB,a,IBLO,A>,3,O>?:D=>OR,h&KKCJA,IJCT>OODC==>Bi,R,

0=>,IJC@MD=LJD>,I>L@,JM:II:J:d@J>,=C@:??>=@,:L,AMKCLJO,A9MIDOCA>O,D=T>K@D>L\,N:=:L\R,+D,B:,IJC@MD=LJD>,=>,O9:KKC?I:U=>,I:O,A>,IJDO>,A>,ICDAO,=D,A>,OQ=AJC?>,

VLD,I>L@,OLJ;>=DJ,>=,8G3,O>?:D=>O,:;>K,L=>,OLJ;>DBB:=K>,A>,B:,IJC@MD=LJD>,>@,A>,B9:BNL?D=M?D>,L=>,TCDO,I:J,O>?:D=>,>@,L=,@J:D@>?>=@,:K@DT,A>,@CL@,TCQ>J,D=T>K@D>L\R,R,h&KKCJA,IJCT>OODC==>Bi,

,K>BB>O,OLJ;>=:=@,IBLO,A>,3,?CDO,:IJ<O,B9:JJX@,AL,@J:D@>?>=@

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

3j

>=,E,?CDO,CL,A>,E,TCDO,>=,Yc,?CDOR,R,h&KKCJA,IJCT>OODC==>Bi

2.7.1.4 - avec corticodépendance :A>,B:,JMALK@DC=,A>O,KCJ@DKCpA>O,CL,A:=O,B>O,3,?CDO,OLD;:=@O,B9:JJX@,AL,@J:D@>?>=@R,$=,I:JB>,A>,KCJ@DKCGAMI>=A:=K>,W,O>LDB,MB>;M,OD,B:,ACO>,,ICLJ,?:D=@>=DJ,B:,JM?DOODC=,

h&KKCJA,IJCT>OODC==>Bi,

2.7.2 Une corticorésistance:&NO>=K>,A>,JM?DOODC=,A:=O,B>O,4,O>?:D=>O,:IJ<O,B>,AMNL@,AL,@J:D@>?>=@,KCJ@DKCpA>,

A9D=@>J;:BB>R,h&KKCJA,IJCT>OODC==>Bi,.CLJ,K>J@:D=O,:L@>LJO,C=,=>,I:JB>,A>,KCJ@DKCG

#CJ@DKCO>=ODNDBD@M, 5, cFb, A>O, K:O, ÌCLOOM>, L=DVL>, CL, J:J>, 5, YgF, ], JMKDAD;>O,?LB@DIB>O54gFa,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,

1>, +', KCJ@DKCO>=ODNB>, :, L=, NC=, IJC=CO@DK, W, BC=U, @>J?>, ?X?>, OD, L=, UJ:=A,

D=;>JO>?>=@,IJCICJ@DC==>B,:L,@:L\,A>,B:,IJC@MD=LJD>`ca,`3ja,`4caR19M;CBL@DC=,;>JO,19*!),KC=K<J=>,8Y,W,FHb,A>O,K:O,R0=>,K>J@:D=>,KCJJMB:@DC=,>\DO@>,>=@J>,B>O,AC==M>O,

1>O,T:K@>LJO,IJMADK@DTO,A>,B:,KCJ@DKCJMODO@:=K>,O>?NB>=@,X@J>,h&KKCJA,IJCT>OODC==>Bi,50=>,ALJM>,A>,@J:D@>?>=@,k,W,YE,O>?:D=>OR

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

3c

3.Recommandations thérapeutiques pour le syndrome néphrotique idiopathique3.1. Traitement symptomatique

B>O, ?>OLJ>O, OQ?I@C?:@DVL>O, O9:;<J>=@, D=ADOI>=O:NB>O, ICLJ, IJM;>=DJ, B>O,

A>, B:, TC=K@DC=, JM=:B>S, A9:?MBDCJ>J, B:, VL:BD@M, A>, ;D>, A>O, I:@D>=@OS, >@, A9M;D@>J, B>O,

I:@D>=@OR

3.1.1. Le régime

Recommandation n°111>,JMUD?>,IJMKC=DOM,ACD@,X@J>5,

3H?Ug?8gPa,ILDO,J>O@J>D=@,>=,OCADL?,I:J,B:,OLD@>R'CJ?CIJC@DADVL>R

B>O,:IICJ@O,>=,>:L,OC=@,W,:A:I@>J,>=,TC=K@DC=,A>,B9M@:@,KBD=DVL>,`C>A<?>OS,IJDO>,A>,ICDAOa,A>,B:,=:@J>?D>,>@,A>,B:,ADLJ<O>RB9:IICJ@,A>,IC@:OODL?,=9>O@,I:O,=MK>OO:DJ>,B>,IBLO,OCL;>=@R


3.1.2. Les diurétiques

Recommandation n°121>O,ADLJM@DVL>O,ACD;>=@,X@J>,L@DBDOMO,:;>K,IJLA>=K>,>=,K:O,A9C>A<?>O,D?ICJ@:=@O5

5,FH,W,YFH,?U,gP,>=,IJ>?D<J>,D=@>=@DC=,A9:L@:=@,IBLO,VL>,B:,=:@JDLJ<O>,>O@,>TTC=AJM>M5

M;>=@L>BB>?>=@,I:J,I>JTLODC=,A9:BNL?D=>,>@,:;>K,L=>,OLJ;>DBB:=K>,A>,B:,f:BDM?D>R


!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

3m


A>,IMJD@C=D@>S, OCLO, TCJ?>,A9:BNL?D=>,W,8Hb,`YUgfUa,>=,I>JTLODC=, B>=@>,:;>K,L=,KC=@JeB>,A>,B:,IJ>OODC=,:J@MJD>BB>R


3.1.3. Traitement anticoagulant

Recommandation n°14 : Traitement préventif des thromboses.CLJ,@CLO,B>O,I:@D>=@O,5,%CNDBDO>J,B>O,I:@D>=@OS,I:O,A>,J>ICO,:L,BD@

I>JTLODC=O,A9&BNL?D=>,W,ADOKL@>J,.JCOKJDJ>, 5, B>O,IC=K@DC=O,A9:J@<J>O,>@,A>,;>D=>O,IJCTC=A>O, ÌCLJ, B>O,NDB:=O,CL,

D=@J:;>D=>LO>,D=L@DB>R

@%)-* $#-* =(01#-* 1(/20G#-* I, VLD, OC=@, K>BB>O, UM=MJ:B>?>=@, CNO>J;M>OS, L=>,

@%)-* $#-* =(01#-* -2<G0#-* I

IB:VL>@@>Og?BS,>O@,J>KC??:=AMR

@502#*/5*&0%'&#1#)&*I*PLOVL9W,ADOI:JD@DC=,A>O,T:K@>LJO,A>,JDOVL>R


Recommandation n°15 : Traitement curatif des thromboses

,.LDO,J>B:D,J:IDA>,I:J,:=@D;D@:?D=>,n,,>@,>=,;DO:=@,L=,*'!,>=@J>,3,>@,4

,7MI:JD=>,ILDO,:=@D,;D@:?D=>,nR,ADOKL@>J,>=,O>J;DK>,OIMKD:BDOM,B9:K@D;:@>LJ,@DOOLB:DJ>,A>,IB:O?D=CU<=>,:OOCKDM,CL,=C=,W,B9&)***R


!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

4H

F(50* >#0&%')-* %5&#50-J

N9?O*(5*32+%0')#*/#*:%-*+('/-*1($2>5$%'0#PQ*%)&'A%8028%)&-*+$%45#&&%'0#-*>=,

F(50*/R%5&0#-*%5&#50-*I

&BNL?D=>,k,8H,UgB

(GAD?<J>O,l,YHHH,?Ug?B

*F02>%5&'()*IA>,.JC@MD=>,#,>@,+,I:J,B:,t:JT:JD=>S,OLJ;>=:=@,:;:=@,J>O@:LJ:@DC=,A>,B9&)***,I>L@,

AL,@J:D@>?>=@R,(C=KS,B:,?DO>,>=,JCL@>,IJCUJ>OOD;>,AL,@J:D@>?>=@,>O@,J>KC??:=AM>R,

F02>%5&'()I,1CJO,A>,B:,=MU:@D;:@DC=,A>,B:,IJC@MD=LJD>S,B:,I>J@>,A9&),***,;:,K>OO>JS,AC=K,DB,>O@,J>KC??:=AM,A9:JJX@>J,B>,@J:D@>?>=@,A<O,B:,=MU:@D;:@DC=,A>,B:,IJC@MD=LJD>,

7#-* 32+%0')#-* /#* :%-* +('/-* 1($2>5$%'0#, I>L;>=@, KC=O@D@L>J, L=>, :B@>J=:@D;>,

3.1.4. Vitamine D - CalciumRecommandation n°16 :

, OLIIBM?>=@:@DC=,>=,;D@:?D=>,(, ÀCO>,=CJ?:B>,gPCLJ, 5, 4HH,W,cHH,0gPa, PLOVL9W,

, OLIIBM?>=@:@DC=,K:BKDVL>,>=, TC=K@DC=,A>O,:IICJ@O,:BD?>=@:DJ>O,>=,;DO:=@,A>O,:IICJ@O,=CJ?:L\,ICLJ,B9oU>

KBD=DVL>O,>@,CL,NDCBCUDVL>O&KKCJA,IJCT>OODC==>B

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

4Y

3.1.5. Statines

Recommandation n°17 : 19L@DBDO:@DC=,A>O,O@:@D=>O,I>J?>@,A>,IJM;>=DJ,B>O,KC?IBDK:@DC=O,K:JADC;:OKLB:DJ>O,

3.1.6. Prévention et traitement des infections

Recommandation n°18 : Infections D)=#>&'()-*:%>&20'#))#-

>@, L=>, OLJ;>DBB:=K>, :@@>=@D;>, ICLJ, AMIDO@>J, B>O, KC?IBDK:@DC=O, D=T>K@D>LO>O, AL,

L=>, KC?IBDK:@DC=, D=T>K@D>LO>, 5, $!1S, IMJD@C=D@>S, D=T>K@DC=, W, I=>L?CKCVL>S,CO@MC?Q>BD@>S,?M=D=UD@>RRR

D)=#>&'()-*)()*:%>&20'#))#-

IJM;>=@DT,>=,K:O,A>,KC=@:U>,5,:KQKBC;DJ,I>J,CO,3H?UgfUgP,I>=A:=@,F,PCLJOa,

7>JI<O, ;DJLO, >=, K:O, A>, ICLOOM>, OCLO, @J:D@>?>=@, KCJ@DKCpA>, CL,D??L=COLIIJ>OO>LJ,5,:KQKBC;DJ,I>J,COR,


Recommandation n°19 : Vaccinations B>O, ;:KKD=O, @LMO, I>L;>=@, X@J>, T:D@O, W, ADO@:=K>, A9L=>, ICLOOM>, CL, ICLJ, B>O,>=T:=@O,VLD,J>O@>=@,BC=U@>?IO,OCLO,KCJ@DKCpA>O,BCJOVL>,B:,ACO>,A>,IJ>A=DOC=>,>O@,T:DNB>,`k,Y,?UgfU,L=,PCLJ,OLJ,A>L\a,,

1>O,:OOCKD:@DC=O,A>,;:KKD=OS,ACD;>=@,X@J>,M;D@M>O,K:J,>BB>O,OC=@,OLOK>I@DNB>O,A>,


K>I>=A:=@,J:DOC==:NB>,A>,=>,I:O,;:KKD=>J,I>=A:=@,L=>,ICLOOM>,CL,PLO@>,:IJ<OS,I>=A:=@,L=,@J:D@>?>=@,W,TCJ@>,ACO>,A>,KCJ@DKCpA>O,>@,OCLO,D??L=COLIIJ>OO>LJOR,

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

48

1CJOVL9L=>,;:KKD=:@DC=,>O@,PLUM>,=MK>OO:DJ>S,B>O,;:KKD=O,@LMO,I>L;>=@,X@J>,T:D@O,W,ADO@:=K>,A9L=>,ICLOOM>,CL,ICLJ,B>O,>=T:=@O,VLD,J>O@>=@,BC=U@>?IO,OCLO,KCJ@DKCpA>O,BCJOVL>,B:,ACO>,A>,IJ>A=DOC=>,>O@,T:DNB>,`k,Y,?UgfU,L=,PCLJ,OLJ,A>L\a,>@,B>,;:KKD=,:=@DUJDII:B,I>L@,X@J>,IJCICOMR

B>O, ;:KKD=:@DC=O, =C=, D??MAD:@>?>=@, =MK>OO:DJ>OS, C=, IJCICO>, :K@L>BB>?>=@, A>,;:KKD=>J,W,ADO@:=K>,A9L=>,ICLOOM>S,OD,ICOODNB>,Y,:=,:IJ<O,B9:JJX@,AL,@J:D@>?>=@R

*B, >O@, IJMTMJ:NB>, A9M;D@>J, B>O, :OOCKD:@DC=O, A>, ;:KKD=O, @>B, B>, @>@J:KCVS, K:J, >BB>O,

3.1.7. Hormone de croissance

Recommandation n°20 : 1>O,>=T:=@O,VLD,JMIC=A>=@,:L\,KJD@<J>O,A9D=KBLODC=,OLD;:=@OS,ACD;>=@,X@J>,@J:D@MO,

B9D=KBLODC=(CO>,A>,IJ>A=DOC=>,A9:L,?CD=O,HS8,?UgfUgPCLJ,`HS4,?UgfU,Y,PCLJ,OLJ,8a"=T:=@O,D?ILN<J>O,CL,ILN>J@M,AMNL@:=@>

HS4E,?UgfUgO>?:D=>Y,D=P>K@DC=,OCLOGKL@:=M>,@CLO,B>O,PCLJO


3.1.8. Traitement anti-protéinurique

Recommandation n°21 : 1>, @J:D@>?>=@, W, ;DOM>, :=@DIJC@>D=LJDVL>, `CNP>K@DT, >OO>=@D>B, AL, @J:D@>?>=@,

OLIIJ>OO>LJO,CL,>=,K:O,A>,JM?DOODC=,I:J@D>BB>,AL,+'R


!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

43

1>O, *"#, >@, B>O,&!&**, C=@, L=, JeB>, :APL;:=@, =C=, =MUBDU>:NB>, :;>K, L=, >TT>@, :=@D,

OQO@<?>,JM=D=>,:=UDC@>=OD=>,JMALD@,B9:ICI@CO>,ICACKQ@:DJ>,`3jaR,19:OOCKD:@DC=,*"#G

3.1.9. Traitement anti-hypertenseur

Recommandation n°22 :

A>, ;>DBB>J, W, B>LJO, KC=@J>GD=ADK:@DC=, J>OI>K@D;>OS, =M:=?CD=O, B9L@DBDO:@DC=, A9*"#,

IJ>?D<J>,D=@>=@DC=&KKCJA,IJCT>OODC==>B

3.2. Traitement étiopathogénique3.2.1. Formes cortico-sensibles 3.2.1.1. Première cure thérapeutique


H)=%)&.J>A=DOC=>,EH,?Ug?qgP,\,4,O>?:D=>OILDOS,EH,?Ug?qg8P,\,4,O>?:D=>OSILDOS,4H,?Ug?qg8P,\,4,O>?:D=>OSILDOS,3H,?Ug,?qg8P,\,4,O>?:D=>O>=OLD@>,JMALK@DC=,IJCUJ>OOD;>,I:J,I:BD>JO,A>,F,?U,OLJ,L=>,ALJM>,A>,3,?CDOALJM>,@C@:B>,5,E,W,j,?CDO

9/5$&#.J>A=DOC=>,YSF,?UgfUgP,\,H8,?CDOSILDOS,YSF,?UgfUg8P,\,H8,?CDOS>=OLD@>,JMALK@DC=,IJCUJ>OOD;>,OLJ,L=>,ALJM>,A>,8,W,3,?CDOALJM>,@C@:B>,5,E,W,j,?CDO



!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

44

Recommandation n°25 : Rémission complète

A>O,@J:K>O,A>,IJC@MD=LJD>,W,B:,N:=A>B>@@>,LJD=:DJ>,,I>=A:=@,3,PCLJO,KC=OMKL@DTO


3.2.1.2. Traitement des rechutesRecommandation n°26 :

NDCBCUDVL>,CL,I:J,B:,JM:II:JD@DC=,A9L=>,IJC@MD=LJD>,DOCBM>,`,IJC@MD=LJD>,lCL,

IBLO,A>,3,O>?:D=>OR,&L,AMKCLJO,A9MIDOCA>O,D=T>K@D>L\,N:=:L\SOD,B:,IJC@MD=LJD>,=>,O9:KKC?I:U=>,I:O,

A9:@@>=AJ>,L=>,JM?DOODC=,OIC=@:=M>,VLD,I>L@,OLJ;>=DJ,>=,8G3,O>?:D=>O,:;>K,L=>,OLJ;>DBB:=K>,A>,B:,IJC@MD=LJD>,>@,A>,B9:BNL?D=M?D>,L=>,TCDO,I:J,O>?:D=>,>@,L=,@J:D@>?>=@,:K@DT,A>,@CL@,TCQ>J,D=T>K@D>L\R,R



IJ>?D<J>,KLJ>,#>I>=A:=@S, BCJOVL>, B>,I:@D>=@, >O@, KC==L,:;>K,L=, O>LDB,A>, KCJ@DKCO>=ODNDBD@M,

AMKJCDOO:=K>, IBLO, J:IDA>, :;>K, A>O, I:BD>JO, A>, 8, O>?:D=>O, O>LB>?>=@, ICLJ,

I:@D>=@R

ACO>,A>,YF,?Ug?8,L=,PCLJ,OLJ,A>L\,I>=A:=@,Y8,W,Yc,?CDOR


1>O,ILNBDK:@DC=O,A>,B:,BD@@MJ:@LJ>,KC=KCJA>=@,VL:=@,W,B9D=@MJX@,A>O,KCJ@DKCpA>O,>=,

`3ma,`4caR7#-*7STB:,?:PCJD@M,A>O,K:O,mHGmFb,>@,>BB>,>O@,?LB@D,JMKDAD;:=@>,A:=O,FH,W,EHb,A>O,K:O,`3a,R

OCL;>=@,N>OCD=,A9L=,@J:D@>?>=@,A>,A>L\D<?>,D=@>=@DC=R

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

4F

>O@, D=;>JO>?>=@,IJCICJ@DC==>B,W, B:,ALJM>,AL, @J:D@>?>=@, `Ya`3a`3Ya], B9DAM:B, O>J:D@,4,O>?:D=>O,A>,ACO>,VLC@DAD>==>,OLD;D>O,A>,E,?CDO,A>,@J:D@>?>=@,:B@>J=:@DT, ], B>,JDOVL>,A9>TT>@O,O>KC=A:DJ>O,=>,O9>=,@JCL;>=@,I:O,?:PCJM,`3YaR(:=O, B:, BD@@MJ:@LJ>, , 8H, W, 44b, A>O,ECK, OC=@, MU:B>?>=@, KCJ@DKCO>=ODNB>O, :;>K,

O>=ODNB>O,W,L=>,O>KC=A>,KLJ>,A>,KCJ@DKCpA>O,O>BC=, B>O,?X?>O,?CA:BD@MO,VL>, B:,IJ>?D<J>`4caR

3.2.1.3 Formes corticodépendantesRecommandation n°28 :

$=, I:JB>, A>, KCJ@DKCA>I>=A:=K>, A>;:=@, L=, +', D=D@D:B>?>=@, KCJ@DKCO>=ODNB>,

OLD;:=@O,B9:JJX@,AL,@J:D@>?>=@R$=,I:JB>,A>,KCJ@DKCGAMI>=A:=K>,W,O>LDB,MB>;M,OD, B:,ACO>, ,ICLJ,?:D=@>=DJ, B:,

B9:ALB@>

Recommandation n°29 : "=,K:O,A>,KCJ@DKCAMI>=A:=K>SB>O,@J:D@>?>=@O,L@DBDOMO,,OC=@,KB:OOMO,>=,3,=D;>:L\,`*,W,***aR1:,IJ>A=DOC=>,W,B:,ACO>,A>,HS8H,?UgfU,g8P,>O@,:OOCKDM>R,+:LT,>\K>I@DC=S,DBO,ACD;>=@,X@J>,>=;DO:UMO,A:=O,B9CJAJ>,OLD;:=@51M;:?DOCB>,,`=D;>:L,*a,5,1:,ACO>,>O@,A>,8SF,?UgfU,L=,PCLJ,OLJ,A>L\,>=,Y,IJDO>,R,

IJCT>OODC==>Bi1>O,:U>=@O,:BfQB:=@O,`=D;>:L,**a5,

1:,KDKBCOICJD=>,&,`=D;>:L,***a5,.COCBCUD>,5,,FGE?UgfUgP,,>=,8,IJDO>O,VLC@DAD>==>O,R,1:,KDKBCOICJD=M?D>,O>J:,

O>J:,I:O,:LU?>=@M>R(LJM>,AL,@J:D@>?>=@,5Y8,W,84,?CDOR,"=OLD@>,N:DOO>,IJCUJ>OOD;>,OLJ,3,W,E,?CDOR,

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

4E

"=,K:O,A>,KCJ@DKCAMI>=A:=K>S,B>,B>;:?DOCB,CL,B>O,*??L=COLIIJ>OO>LJO,:OOCKDMO,

1:,KB:OO>,A>O,*??L=COLIIJ>OO>LJO,KC?IJ>=A,B>O,:U>=@O,:BfQB:=@O`=D;>:L,**a,,,>@,B:, KDKBCOICJD=>, `=D;>:L, ***aR, 1>LJO, D=ADK:@DC=O, J>B<;>=@, A>, ODU=>O, A9D=@C\DK:@DC=,O@MJCpAD>==>, UJ:;>O, `:=C?:BD>O, A>, B:, KJCDOO:=K>, OLJ@CL@S, =MKJCO>, COO>LO>S,

(L,T:D@,A>,B9D??L=COLIIJ>OODC=,VL9DBO,D=ALDO>=@S,DBO,>\ICO>=@,W,A>O,KC?IBDK:@DC=O,D=T>K@D>LO>OR#>O, @J:D@>?>=@O, OC=@, MU:B>?>=@, W, ADOKL@>J, A:=O, B>O, TCJ?>O, KCJ@DKCO>=ODNB>O,I:J@D>BB>O

W,B9M@LA>R

7#*7#<%1'-($

T:DNB>O,ACO>O,A>,IJMA=DOC=>,AC=@, DB,I>J?>@,L=,>TT>@,A9MI:JU=>R,#C?ICJ@:=@,L=,

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:,>L,3,ICLOOM>O,:L,?CD=O,CL,BCJOVL>,B:,KCJ@DKCAMI>=A:=K>,:,L=,O>LDB,MB>;MR

+D,K>,NL@,=9>O@,I:O,:@@>D=@,:L,NCL@,A>,E,W,Y8,?CDOS,B>,@J:D@>?>=@,>O@,:JJX@MR*B, T:L@, OLJ;>DBB>J, B:, =L?MJ:@DC=, TCJ?LB>, O:=ULD=>, `JDOVL>, A>, =>L@JCIM=D>a,@CL@>O,B>O,8,O>?:D=>O,ILDO,@CL@>O,B>O,3,O>?:D=>O,>=,TC=K@DC=,A>,B:,@CBMJ:=K>R,0=>,=>L@JCIM=D>,k,8HHH,D?ICO>,B9:JJX@,AL,@J:D@>?>=@R,1:,J>IJDO>,:IJ<O,=CJ?:BDO:@DC=,A>, B:, =L?MJ:@DC=, TCJ?LB>, O:=ULD=>S, :;>K, L=>, OLJ;>DBB:=K>, :KKJL>, >@, W, IBLO,T:DNB>O,ACO>O,>O@,IJCICOM>,I:J,K>J@:D=OR,1:,OLJ;>=L>,A9L=,A>L\D<?>,MIDOCA>,A>,

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!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

4j

AL, @J:D@>?>=@, `FHaR, *B, T:L@, OLJ;>DBB>J, B:, =L?MJ:@DC=, TCJ?LB>, O:=ULD=>S, , @CL@>O,

R,*B,I>L@,X@J>,J>IJDO,:IJ<O,=CJ?:BDO:@DC=,A>,B:,=L?MJ:@DC=,TCJ?LB>,O:=ULD=>R,1>,

J>O@>,W,ADOKL@>J,AL,T:D@,AL,KL?LB,A>O,ACO>O,@C\DVL>O,`FHaR

ADOKC=@D=LR,&IJ<O,B>,A>L\D<?>,?CDO,A>,@J:D@>?>=@S,>BB>,>O@,J:IDA>?>=@,AD?D=LM>,OLJ,L=,?CDO,PLOVL9W,B9:JJX@,`FHaR

7%*>'>$(-+(0')#*9

ALJ:NB>,A:=O,IBLO,A>,jFb,A>O, K:O,>@,I:J@D>BB>,A:=O,j,W,YHb,A>O, K:O, `Yaa`FaÈa`ca,CN@>=L>,:IJ<O,8G4,O>?:D=>O,A>,@J:D@>?>=@,I:JTCDO,IBLO,Ò:;CDJ,:@@>=AJ>,4,?CDOa,ÈaR,

W,F,?CDO,`3aR,0=, @J:D@>?>=@,I:J, KDKBCOICJD=>, >O@, D=ADVLM, >=, K:O,A>, KCJ@DKCA>I>=A:=K>,:IJ<O,

1>, @J:D@>?>=@, >O@, ICLJOLD;D, Yc, W, 84, ?CDO`YHaR, &IJ<O, K>, AMB:DS, >=, B9:NO>=K>, A>,

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1:, OLJ;>DBB:=K>, KC?ICJ@>J:, L=, DC=CUJ:??>, O:=ULD=S, LJM>, OMJDVL>S, KJM:@D=D=>,OMJDVL>S,IJC@DAM?D>, `:BNL?D=M?D>aS, KB>:J:=K>,A>, B:,KJM:@D=D=>,>@, @:L\,JMODAL>BO,

KDKBCOICJD=M?D>,W,)C,>=@J>,YHH,>@,YFH,uUgB,O:=U,@C@:B

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4c

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3.2.2. Formes corticorésistantes




IJCT>OODC==>Bi,

19:OOCKD:@DC=,A9L=>,T:DNB>,ACO>,A>,KCJ@DKCpA>O,`HR8H,?UgfUgPa,>O@,IJMKC=DOM>R,


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4m


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;%>0($'15-I

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Recommandation n°33 : 1>O, :L@J>O, @J:D@>?>=@O, 5, :U>=@O, :BfQB:=@OS, +DJCBD?LOS, !D@L\D?:NS, =>, OC=@, I:O,

K>,KC=@>\@>,B>O,@J:D@>?>=@O,L@DBDOMO,O>JC=@,W,N:O>,A>,5

7%*>'>$(-+(0')#*9

1:, KDKBCOICJD=>, :LJ:D@, L=, >TT>@, :=@DIJC@MD=LJDVL>, D=AMI>=A:=@, A>, B9>TT>@,D??L=COLIIJ>OO>LJ, K>, VLD, >\IBDVL>J:D@, B>O, JM?DOODC=O, I:J@D>BB>O, `maR, +CLO,KDKBCOICJD=>,W,BC=U,@>J?>S,B:,TC=K@DC=,JM=:B>,O9:?MBDCJ>,:L,BD>L,A>,AMKBD=>J,`YHaR,

KC??>=K>J,W,N:DOO>J,B>O,KCJ@DKCpA>O,PLOVL9W,B:,ACO>,B:,IBLO,T:DNB>,?:D=@>=:=@,B:,JM?DOODC=,`HR8H,?U,gfUg,8Pa`3aR,1:,ALJM>,@C@:B>,AL,@J:D@>?>=@,I:J,KDKBCOICJD=>,ACD@,X@J>,A>,8,:=O,`YHa`YYaR&,B9DOOL>,A>,K>@@>,IMJDCA>,8H,W,8Fb,A>O,JM?DOODC=O,J>O@>=@,ALJ:NB>O,:IJ<O,:JJX@`3a

A>O,K:O,OC=@,JMODO@:=@O,W,L=,8<?>,@J:D@>?>=@,,I:J,#DKBCOICJD>,&`8HaR,19LO:U>,A>,B:,

7#*;%>0($'15-1:,ALJM>, @C@:B>,AL, @J:D@>?>=@,A:=O, B>O,>OO:DO,>O@,A>,8, O>?:D=>O,W,Yc,?CDO, `Y8a,S,?:DO, OC=, L@DBDO:@DC=, A:=O, K>@@>, D=ADK:@DC=, >O@, >=KCJ>, BD?D@M>`3aR, +C=, >TT>@, >O@,O>?NB:NB>,W,B:,KQKBC,&,:;>K,L=>,?CD=AJ>,@C\DKD@M,JM=:B>,`Y3a`4FaR,*B,:,M@M,I:JTCDO,

!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"

FH

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F3

3.3.2. Coût approximatif du traitement par hémodialyse

3.3.3. Coût approximatif de la transplantation rénale

3.3.4. Implications sociales(:=O,B:,?:PCJD@M,A>O,K:O,B>,@J:D@>?>=@,?MADK:B,>@,B>,OLD;D,OC=@,KC?I:@DNB>O,:;>K,L=>,

3.4. limites et contre-indications du traitement1>O,BD?D@>O,AL,@J:D@>?>=@,OC=@,KC=O@D@LM>O,I:J,B>O,>TT>@O,O>KC=A:DJ>O,UM=MJMO,I:J,

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HTA, Diabète, Obésité,Retard staturo-pondéral, Ostéonécrose aseptique, Glaucome, Cataracte, Vergetures, Troubles du comportement, Infection, Ostéoporose, Troubles digestifs.

Leucopénie, Aplasie, Stérilité, Cystite, Alopécie, Néoplasie, Lymphomes

HTA, Néphrotoxicité, Hypertrichose, Hypertrophie gingivale, Paresthésies

Recommandation n°34 : 1>O,KC=@J>GD=ADK:@DC=O,:L,@J:D@>?>=@,OC=@5

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F4

4.Modalités de suivi4.1. Evaluation de l’e$cacité et de la tolérance du traitement4.1.1. Surveillance cliniqueRecommandation n°35 :

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4.1.2. Surveillance paraclinique

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4.2. Professionnels impliqués dans le suivi

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ETAPES DE LA PRISE EN CHARGE PROFESSIONNELS IMPLIQUES

DIAGNOSTIC CLINIQUE ET BIOLOGIQUE

Néphrologues,Néphrologues pédiatres,Pédiatres,Anatomopathologistes,Médecins internistes,Médecins généralistes,

CONFIRMATION HISTOLOGIQUE

Néphrologues,Néphrologues pédiatres,UrologuesMédecins internistes,Anatomopathologistes,

ETAPE THERAPEUTIQUE

Néphrologues,Néphrologues pédiatres,Pédiatres,Médecins internistes,Médecins généralistes,Nutritionnistes,

ETAPE DU SUIVI

Néphrologues,Néphrologues pédiatres,Pédiatres,Médecins internistes,Médecins généralistes,

4.3. Date d’arrêt du traitementRecommandation n°37 :

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5.Recommandations aux patients5.1. Education thérapeutique"BB>,AMNL@>,A<O,B:,IJ>?D<J>,;DOD@>R,*B,I>J?>@,A>,?D>L\,KC?IJ>=AJ>,B:,?:B:AD>,:L,I:@D>=@R

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6.Références bibliographiques

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E3

Annexe 1: Liste des prestataires concernés par la prise en charge et le suivi

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Annexe 2: Liste des médicaments, dispositifs médicaux et actes professionnels nécessaires à la prise en charge

Médicaments DCI Présentation et dosage

Prednisone

Comprimé : 1 mgComprimé : 5 mgComprimé : 20 mg

Prednisolone Comprimé : 5 mgComprimé : 20 mg

Methylprednisolone

Poudre et solvant intra-veineuse : 20 mgPoudre et solvant intra-veineuse : 40 mgPoudre et solvant intra-veineuse : 120 mgPoudre et solvant intra-veineuse : 500 mg

LévamisolComprimé : 300 mgComprimé : 150 mg

Ciclosporine

Capsule : 25 mgCapsule : 50 mgCapsule : 100 mgSolution buvable : 100 mg/ml

Chlorambucil Comprimé : 2 mg

Azathioprine Comprimé : 50 mg

Cyclophosphamide Comprimé : 50 mg

Mycophénolate MofetilGélule : 250 mgCapsule : 500 mg

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E4

TacrolimusGélule :1 mgGélule :5 mg

Rituximab Solution pour perfusion intra-veineuse : 100 mg/mlSolution pour perfusion intra-veineuse : 500 mg/ml

Captopril Comprimé : 25 mg

Cilazapril Comprimé : 2,5 mgComprimé : 5 mg

Périndopril Comprimé : 4 mgComprimé : 8 mg

Quinapril Comprimé : 5 mgComprimé : 20 mg

Ramipril

Comprimé : 1,25 mgComprimé : 2,5 mgComprimé : 5 mgComprimé : 10 mg

Trandolapril Comprimé :0,5 mgComprimé : 2 mg

CandesartanComprimé : 4 mgComprimé : 8 mgComprimé : 16 mg

Irbésartan Comprimé : 150 mgComprimé : 300 mg

Losartan Comprimé : 50 mgComprimé : 100 mg

ValsartanComprimé : 40 mgComprimé : 80 mgComprimé : 160 mg

Atorvastatine Comprimé : 10 mgComprimé : 20 mg

Fluvastatine Comprimé : 80 mg

Pravastatine Comprimé : 20 mg

SimvastatineComprimé : 10 mgComprimé : 20 mgComprimé : 40 mg

Dipyridamole Comprimé : 75 mg

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EF

Aspirine et salicylés

Gélule : 325 mgPoudre pour solution buvable : 100 mgPoudre pour solution buvable : 160 mgPoudre pour solution buvable : 250 mgPoudre pour solution buvable : 300 mgPoudre pour solution buvable : 500 mg

Acénocoumarol Comprimé : 4 mg

CalciumComprimé : 500 mg ;1000 mgPoudre : 500 mg ;1000 mg ;1540 mgSirop : 250 mg par cas

Vitamine D 3 Solution buvable : 300 UI par goutteAmpoule buvable : 200 000 UI par ampoule

Albumine humaine Flacon de 100 ml ;250 ml ;500 ml

Hormone de croissance Cartouche :4UI ; 16UI ;32UIFlacon :4UI ; 12UI ; 24UI

Dispostifs médicaux

Trocard de ponction biopsie rénale

Actes professionnels

Ponction biopsie rénale

Ponction biopsie rénale avec repérage échographique

Ponction biopsie rénale sous guidage échographique

Biopsie rénale par voie trans-jugulaire

Biopsie rénale chirurgicale

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EE

Annexe 3 : Composition du groupe de travail et liste des participants à l’élaboration des RBPM (con#it d’intérêt)

Nom & Prénom Spécialité Secteur Institution Con"its d’intérêt

Khadija Hachim

(Présidente)Néphrologie CHU

CHU Ibn Rochd

Casblanca

Absence de con#it

Bachir Mounib

(Rapporteur)Néphrologie Libéral Cabinet privé

AgadirAbsence de

con#it

Mohamed El Mehdi Néphrologie Libéral Cabinet privé

CasablancaAbsence de

con#it

Mia Hajji Médecine Interne Libéral Cabinet privé Casablanca

Absence de con#it

Asmaa Laaboudi Néphrologie Publique Hôpital de

TemaraAbsence de

con#it

Rajaa Rochdi Néphrologie PubliqueHôpital

Aïn Chock Casablanca

Absence de con#it

Saïda Sqalli Anatomopathologie Libéral Casablanca Absence de con#it

athe

na com

Documents

Syndrome néphrotique