syndrome néphrotique
TRANSCRIPT
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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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Résumé des RBPM P. 05Texte des RBPM P. 17
SOMMAIRE
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Syndrome Néphrotique
Recommandations de Bonnes Pratiques Médicales
Résumé
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1.Présentation de la maladie1.1. Dé"nition de la maladieRecommandation n°1
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3. Recommandations thérapeutiques3.1. Traitement symptomatique
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4. Modalités de suivi
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Syndrome Néphrotique
Recommandations de Bonnes Pratiques Médicales
Texte
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$cacité et de la tolérance du traitement P. 54
4.1.1. Surveillance clinique P. 544.1.2. Surveillance paraclinique P. 54
4.2. Professionnels impliqués dans le suivi P. 544.3. Date d’arrêt du traitement P. 55
5.Recommandations aux patients P. 565.1. Education thérapeutique P. 565.2. Modi"cation du mode de vie P. 56
6.Références bibliographiques P. 577.Annexes P. 63
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83
LISTE DES ABREVIATIONS
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1.Introduction 1.1. Objet19CNP>@,A>,K>O,J>KC??:=A:@DC=O,>O@,A>,?>@@J>,W,ADOICOD@DC=,A>O,IJCT>OODC==>BO,A>,
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Tableau I. Grade des recommandations
Niveau de preuve scienti!que fourni par la littérature(études thérapeutiques)
Gradedes recommandations
Niveau 1Essais comparatifs randomisés de forte puissanceAnalyse de décision basée sur des études bien menées
APreuve scienti"que
établie
Niveau 2Essais comparatifs randomisés de faible puissanceÉtudes comparatives non randomisées bien menées Études de cohorte
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1:, BD@@MJ:@LJ>, J:IICJ@>, MU:B>?>=@, L=>, IBLO, UJ:=A>, D=KDA>=K>, :L@CLJ, AL,
2.2.3. Sexe ratio
2.2.4. Age de début
2.2.5. Relation atopie-syndrome néphrotique1:,TJMVL>=K>,A>,B9:BB>JUD>,>O@,IBLO,MB>;M>,A:=O,B:,ICILB:@DC=,A>,I:@D>=@O,:@@>D=@O,
Recommandation n°2 bis
h,&KKCJA,IJCT>OODC==>BiR
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"
8m
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!$)*/0"
3H
D??CNDBDO:@DC=aR
"@, A:=O, B>O, TCJ?>O, IJCBC=UM>OS, A>O, KC?IBDK:@DC=O, A>, B:, TLD@>, IJC@MDVL>, >@, A>O,OLNO@:=K>O,BDM>O,:L\,IJC@MD=>O,5,AM=L@JD@DC=S,O>=ODNDBD@M,:L\,D=T>K@DC=OS,:=M?D>,>@K
2.4. Diagnostic positifRecommandation n°31>,AD:U=CO@DK,ICOD@DT,>O@,J>@>=L,A>;:=@5
0=>,>=VLX@>,M@DCBCUDVL>,=MU:@D;>
&KKCJA,IJCT>OODC==>B
,2.4.1. L’existence d’un syndrome néphrotique biologique
Recommandation n°41>O,>\:?>=O,NDCBCUDVL>O,W,;DOM>,AD:U=CO@DK,OC=@,B>O,OLD;:=@O5,
&KKCJA,IJCT>OODC==>B
Recommandation n°5
'L?MJ:@DC=,TCJ?LB>,O:=ULD=>],7M?CKLB@LJ>O, a
*C=CUJ:??>,O:=ULD=,>@,LJD=:DJ>R
&KKCJA,IJCT>OODC==>B
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"
3Y
2.4.2. Une enquête étiologique négative
Recommandation n°6
&KKCJA,IJCT>OODC==>B
(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
&KKCJA,IJCT>OODC==>B
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"
38
Recommandation n°81>O,KC=@J>GD=ADK:@DC=O,A>,B:,NDCIOD>,JM=:B>,OC=@,5
,1>,J>D=,L=DVL>
.JMO>=K>,A9L=>,@L?>LJ,JM=:B>
,1>,J>D=,ICBQfQO@DVL>
&KKCJA,IJCT>OODC==>B
Recommandation n°9 :0=,,NDB:=,IJMGNDCIOD>,JM=:B>,ACD@,X@J>,IJ:@DVLM,5
&KKCJA,IJCT>OODC==>B
3 types histologiques sont possibles :
,>=,?DKJCOKCI>,CI@DVL>S,A>O,UBC?MJLB>O,IJ:@DVL>?>=@,=CJ?:L\S,
,>=,?DKJCOKCI>,MB>K@JC=DVL>S,B:,TLODC=,A>O,ID>AO,A>O,ICACKQ@>OS,
A>O, I:JCDO, K:IDBB:DJ>OS, A:=O, K>J@:D=>O, :=O>O, O>LB>?>=@, >@, A:=O, K>J@:D=O,UBC?MJLB>O,O>LB>?>=@R
,>=,?DKJCOKCI>,MB>K@JC=DVL>S,B9>TT:K>?>=@,A>O,ID>AO,A>O,ICACKQ@>OS,
.BLO, J:J>?>=@S, IJCBDTMJ:@DC=,?MO:=UD:B>, ADTTLO>, .%(, 5, =>, O9:KKC?I:U=:=@,=D, A9:B@MJ:@DC=O, A>O, I:JCDO, K:IDBB:DJ>O, =D, A>, AMIe@O, >\@J:?>=NJ:=>L\,?:DO,
L=,T:K@>LJ,IJ>AMK@DT,A>,KCJ@DKCJMODO@:=K>R5
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"
33
2.5.Diagnostic di!érentiel
Recommandation n°10
,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
&KKCJA,IJCT>OODC==>B
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!$)*/0"
34
A>,ADB:@:@DC=,?DKJC,fQO@DVL>,A>O,@LNLB>O,JM=:L\R,"BB>,>\DO@>,A<O,B:,;D>,T[@:B>,>@,
JM=:B>, OLJ;D>=@, :;:=@, 3, :=OS:KKC?I:U=M>, A>, @JCLNB>O, ADU>O@DTO, D?ICJ@:=@O,>@, A>, KC?IBDK:@DC=O, =L@JD@DC==>BB>O, >@, D=T>K@D>LO>OR, #9>O@, L=>, D=ADK:@DC=, A>,@J:=OIB:=@:@DC=,JM=:B>R,
1:,OKBMJCO>,?MO:=UD:B>,ADTTLO>
D=O>=ODNB>,W,@CLO,B>O,@J:D@>?>=@OR
2.6. Complications
=C?NJ>LO>O,KC?IBDK:@DC=OS,I:JTCDO,AJ:?:@DVL>O,5
2.6.1. L’etat d’anasarque 1>O,C>A>?>O,I>L;>=@,X@J>,@J<O,D?ICJ@:=@O,`:=:O:JVL>a,>@,>=@J:d=>J,A>O,@JCLNB>O,K:JADCGJ>OIDJ:@CDJ>O,CL,A>O,KJDO>O,ACLBCLJ>LO>O,:NAC?D=:B>OR,*BO,OC=@,BDMO,W,L=>,JM@>=@DC=,IJD?D@D;>,AL,OCADL?,>@,W,B:,N:DOO>,A>,B:,IJ>OODC=,C=KC@DVL>,,IB:O?:@DVL>,,
2.6.2. L’hyperlipidémie
AMI:OO>J,,YH,??CBgBR,"BB>,>O@,ADJ>K@>?>=@,KCJJMBM>,W,B:,KB:DJ:=K>,A>,B9:BNL?D=>R,1>O,
OM;<J>,AL,+',?:PCJ>,B>,JDOVL>,K:JADC;:OKLB:DJ>,>@,I>L@,:UUJ:;>J,B:,IJCUJ>OODC=,A>O,BMODC=O,JM=:B>O`EYa,`E8a,R
2.6.3. Les complications thromboemboliques
>O@, L=>, KC?IBDK:@DC=, ND>=, KC==L>, `Eja, S, >BB>,?>@, >=, P>L, B>, IJC=CO@DK, ;D@:BR, 1>O,
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/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,$=,I:JB>,A>,KCJ@DKCGAMI>=A:=K>,W,O>LDB,MB>;M,OD,B:,ACO>,,ICLJ,?:D=@>=DJ,B:,JM?DOODC=,
h&KKCJA,IJCT>OODC==>Bi,
2.7.2 Une corticorésistance:&NO>=K>,A>,JM?DOODC=,A:=O,B>O,4,O>?:D=>O,:IJ<O,B>,AMNL@,AL,@J:D@>?>=@,KCJ@DKCpA>,
A9D=@>J;:BB>R,h&KKCJA,IJCT>OODC==>Bi,.CLJ,K>J@:D=O,:L@>LJO,C=,=>,I:JB>,A>,KCJ@DKCG
#CJ@DKCO>=ODNDBD@M, 5, cFb, A>O, K:O, `ICLOOM>, L=DVL>, CL, J:J>, 5, YgF, ], JMKDAD;>O,?LB@DIB>O54gFa,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,,
1>, +', KCJ@DKCO>=ODNB>, :, L=, NC=, IJC=CO@DK, W, BC=U, @>J?>, ?X?>, OD, L=, UJ:=A,
D=;>JO>?>=@,IJCICJ@DC==>B,:L,@:L\,A>,B:,IJC@MD=LJD>`ca,`3ja,`4caR19M;CBL@DC=,;>JO,19*!),KC=K<J=>,8Y,W,FHb,A>O,K:O,R0=>,K>J@:D=>,KCJJMB:@DC=,>\DO@>,>=@J>,B>O,AC==M>O,
1>O,T:K@>LJO,IJMADK@DTO,A>,B:,KCJ@DKCJMODO@:=K>,O>?NB>=@,X@J>,h&KKCJA,IJCT>OODC==>Bi,50=>,ALJM>,A>,@J:D@>?>=@,k,W,YE,O>?:D=>OR
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/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
&KKCJA,IJCT>OODC==>B
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
&KKCJA,IJCT>OODC==>B
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"
3m
Recommandation n°13
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
&KKCJA,IJCT>OODC==>B
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, `ICLJ, 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=>,
@%)-* $#-* =(01#-* -2<G0#-* I
IB:VL>@@>Og?BS,>O@,J>KC??:=AMR
@502#*/5*&0%'#)&*I*PLOVL9W,ADOI:JD@DC=,A>O,T:K@>LJO,A>,JDOVL>R
&KKCJA,IJCT>OODC==>B
Recommandation n°15 : Traitement curatif des thromboses
,.LDO,J>B:D,J:IDA>,I:J,:=@D;D@:?D=>,n,,>@,>=,;DO:=@,L=,*'!,>=@J>,3,>@,4
,7MI:JD=>,ILDO,:=@D,;D@:?D=>,nR,ADOKL@>J,>=,O>J;DK>,OIMKD:BDOM,B9:K@D;:@>LJ,@DOOLB:DJ>,A>,IB:O?D=CU<=>,:OOCKDM,CL,=C=,W,B9&)***R
&KKCJA,IJCT>OODC==>B
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"
4H
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F(50*/R%5&0#-*%52-*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=>,(, `ACO>,=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, $!1S, IMJD@C=D@>S, D=T>K@DC=, W, I=>L?CKCVL>S,CO@MC?Q>BD@>S,?M=D=UD@>RRR
D)=#>&'()-*)()*:%>&20'#))#-
IJM;>=@DT,>=,K:O,A>,KC=@:U>,5,:KQKBC;DJ,I>J,CO,3H?UgfUgP,I>=A:=@,F,PCLJOa,
7>JI<O, ;DJLO, >=, K:O, A>, ICLOOM>, OCLO, @J:D@>?>=@, KCJ@DKCpA>, CL,D??L=COLIIJ>OO>LJ,5,:KQKBC;DJ,I>J,COR,
&KKCJA,IJCT>OODC==>B
Recommandation n°19 : Vaccinations B>O, ;:KKD=O, @LMO, I>L;>=@, X@J>, T:D@O, W, ADO@:=K>, A9L=>, ICLOOM>, CL, ICLJ, B>O,>=T:=@O,VLD,J>O@>=@,BC=U@>?IO,OCLO,KCJ@DKCpA>O,BCJOVL>,B:,ACO>,A>,IJ>A=DOC=>,>O@,T:DNB>,`k,Y,?UgfU,L=,PCLJ,OLJ,A>L\a,,
1>O,:OOCKD:@DC=O,A>,;:KKD=OS,ACD;>=@,X@J>,M;D@M>O,K:J,>BB>O,OC=@,OLOK>I@DNB>O,A>,
&KKCJA,IJCT>OODC==>B
K>I>=A:=@,J:DOC==:NB>,A>,=>,I:O,;:KKD=>J,I>=A:=@,L=>,ICLOOM>,CL,PLO@>,:IJ<OS,I>=A:=@,L=,@J:D@>?>=@,W,TCJ@>,ACO>,A>,KCJ@DKCpA>O,>@,OCLO,D??L=COLIIJ>OO>LJOR,
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/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
&KKCJA,IJCT>OODC==>B
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
&KKCJA,IJCT>OODC==>B
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"
43
1>O, *"#, >@, B>O,&!&**, C=@, L=, JeB>, :APL;:=@, =C=, =MUBDU>:NB>, :;>K, L=, >TT>@, :=@D,
OQO@<?>,JM=D=>,:=UDC@>=OD=>,JMALD@,B9:ICI@CO>,ICACKQ@:DJ>,`3jaR,19:OOCKD:@DC=,*"#G
3.1.9. Traitement anti-hypertenseur
Recommandation n°22 :
A>, ;>DBB>J, W, B>LJO, KC=@J>GD=ADK:@DC=, J>OI>K@D;>OS, =M:=?CD=O, B9L@DBDO:@DC=, A9*"#,
IJ>?D<J>,D=@>=@DC=&KKCJA,IJCT>OODC==>B
3.2. Traitement étiopathogénique3.2.1. Formes cortico-sensibles 3.2.1.1. Première cure thérapeutique
Recommandation n°23 :
H)=%)&.J>A=DOC=>,EH,?Ug?qgP,\,4,O>?:D=>OILDOS,EH,?Ug?qg8P,\,4,O>?:D=>OSILDOS,4H,?Ug?qg8P,\,4,O>?:D=>OSILDOS,3H,?Ug,?qg8P,\,4,O>?:D=>O>=OLD@>,JMALK@DC=,IJCUJ>OOD;>,I:J,I:BD>JO,A>,F,?U,OLJ,L=>,ALJM>,A>,3,?CDOALJM>,@C@:B>,5,E,W,j,?CDO
9/5$&#.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
Recommandation n°24 :
&KKCJA,IJCT>OODC==>B
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"
44
Recommandation n°25 : Rémission complète
A>O,@J:K>O,A>,IJC@MD=LJD>,W,B:,N:=A>B>@@>,LJD=:DJ>,,I>=A:=@,3,PCLJO,KC=OMKL@DTO
&KKCJA,IJCT>OODC==>B
3.2.1.2. Traitement des rechutesRecommandation n°26 :
NDCBCUDVL>,CL,I:J,B:,JM:II:JD@DC=,A9L=>,IJC@MD=LJD>,DOCBM>,`,IJC@MD=LJD>,lCL,
IBLO,A>,3,O>?:D=>OR,&L,AMKCLJO,A9MIDOCA>O,D=T>K@D>L\,N:=:L\SOD,B:,IJC@MD=LJD>,=>,O9:KKC?I:U=>,I:O,
A9:@@>=AJ>,L=>,JM?DOODC=,OIC=@:=M>,VLD,I>L@,OLJ;>=DJ,>=,8G3,O>?:D=>O,:;>K,L=>,OLJ;>DBB:=K>,A>,B:,IJC@MD=LJD>,>@,A>,B9:BNL?D=M?D>,L=>,TCDO,I:J,O>?:D=>,>@,L=,@J:D@>?>=@,:K@DT,A>,@CL@,TCQ>J,D=T>K@D>L\R,R
&KKCJA,IJCT>OODC==>B
Recommandation n°27 :
IJ>?D<J>,KLJ>,#>I>=A:=@S, BCJOVL>, B>,I:@D>=@, >O@, KC==L,:;>K,L=, O>LDB,A>, KCJ@DKCO>=ODNDBD@M,
AMKJCDOO:=K>, IBLO, J:IDA>, :;>K, A>O, I:BD>JO, A>, 8, O>?:D=>O, O>LB>?>=@, ICLJ,
I:@D>=@R
ACO>,A>,YF,?Ug?8,L=,PCLJ,OLJ,A>L\,I>=A:=@,Y8,W,Yc,?CDOR
&KKCJA,IJCT>OODC==>B
1>O,ILNBDK:@DC=O,A>,B:,BD@@MJ:@LJ>,KC=KCJA>=@,VL:=@,W,B9D=@MJX@,A>O,KCJ@DKCpA>O,>=,
`3ma,`4caR7#-*7STB:,?:PCJD@M,A>O,K:O,mHGmFb,>@,>BB>,>O@,?LB@D,JMKDAD;:=@>,A:=O,FH,W,EHb,A>O,K:O,`3a,R
OCL;>=@,N>OCD=,A9L=,@J:D@>?>=@,A>,A>L\D<?>,D=@>=@DC=R
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/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!$)*/0"
4E
"=,K:O,A>,KCJ@DKCAMI>=A:=K>S,B>,B>;:?DOCB,CL,B>O,*??L=COLIIJ>OO>LJO,:OOCKDMO,
1:,KB:OO>,A>O,*??L=COLIIJ>OO>LJO,KC?IJ>=A,B>O,:U>=@O,:BfQB:=@O`=D;>:L,**a,,,>@,B:, KDKBCOICJD=>, `=D;>:L, ***aR, 1>LJO, D=ADK:@DC=O, J>B<;>=@, A>, ODU=>O, A9D=@C\DK:@DC=,O@MJCpAD>==>, UJ:;>O, `:=C?:BD>O, A>, B:, KJCDOO:=K>, OLJ@CL@S, =MKJCO>, COO>LO>S,
(L,T:D@,A>,B9D??L=COLIIJ>OODC=,VL9DBO,D=ALDO>=@S,DBO,>\ICO>=@,W,A>O,KC?IBDK:@DC=O,D=T>K@D>LO>OR#>O, @J:D@>?>=@O, OC=@, MU:B>?>=@, W, ADOKL@>J, A:=O, B>O, TCJ?>O, KCJ@DKCO>=ODNB>O,I:J@D>BB>O
W,B9M@LA>R
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JDOVL>,A>,JMKDAD;>,>=,KC?I:J:DOC=,:L,IB:K>NC,CL,W,B:,IJ>A=DOC=>,`3Ya,`FYaR#9>O@,L=,IJCALD@,I>L,KeL@>L\,,@>O@M,OLJ,A>O,ALJM>O,:BB:=@,A>,4,W,Y8,?CDO`FYaR,#>,@J:D@>?>=@,:,
:,>L,3,ICLOOM>O,:L,?CD=O,CL,BCJOVL>,B:,KCJ@DKCAMI>=A:=K>,:,L=,O>LDB,MB>;MR
+D,K>,NL@,=9>O@,I:O,:@@>D=@,:L,NCL@,A>,E,W,Y8,?CDOS,B>,@J:D@>?>=@,>O@,:JJX@MR*B, T:L@, OLJ;>DBB>J, B:, =L?MJ:@DC=, TCJ?LB>, O:=ULD=>, `JDOVL>, A>, =>L@JCIM=D>a,@CL@>O,B>O,8,O>?:D=>O,ILDO,@CL@>O,B>O,3,O>?:D=>O,>=,TC=K@DC=,A>,B:,@CBMJ:=K>R,0=>,=>L@JCIM=D>,k,8HHH,D?ICO>,B9:JJX@,AL,@J:D@>?>=@R,1:,J>IJDO>,:IJ<O,=CJ?:BDO:@DC=,A>, B:, =L?MJ:@DC=, TCJ?LB>, O:=ULD=>S, :;>K, L=>, OLJ;>DBB:=K>, :KKJL>, >@, W, IBLO,T:DNB>O,ACO>O,>O@,IJCICOM>,I:J,K>J@:D=OR,1:,OLJ;>=L>,A9L=,A>L\D<?>,MIDOCA>,A>,
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R,*B,I>L@,X@J>,J>IJDO,:IJ<O,=CJ?:BDO:@DC=,A>,B:,=L?MJ:@DC=,TCJ?LB>,O:=ULD=>R,1>,
J>O@>,W,ADOKL@>J,AL,T:D@,AL,KL?LB,A>O,ACO>O,@C\DVL>O,`FHaR
ADOKC=@D=LR,&IJ<O,B>,A>L\D<?>,?CDO,A>,@J:D@>?>=@S,>BB>,>O@,J:IDA>?>=@,AD?D=LM>,OLJ,L=,?CDO,PLOVL9W,B9:JJX@,`FHaR
7%*>'>$(-+(0')#*9
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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>,
&;:=@, B>, AMNL@, AL, @J:D@>?>=@S, DB, T:L@, O9:OOLJ>J, A>, B:, =CJ?:BD@M, A>, B:, TC=K@DC=,UBC?MJLB:DJ>,`KB>:J:=K>,A>,B:,KJM:@D=D=>aR,0=>,NDCIOD>,JM=:B>,>O@,=MK>OO:DJ>,ICLJ,O9:OOLJ>J,AL,AD:U=CO@DKS,I>J?>@@J>,B:,KC?I:J:DOC=,:;>K,A>O,NDCIOD>O,LB@MJD>LJ>O,>@,
:IJ<O,8,:=O,A>,@J:D@>?>=@,>@,>=,K:O,A9:B@MJ:@DC=,A>,B:,TC=K@DC=,UBC?MJLB:DJ>R
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
1:,ACO>,A>,KDKBCOICJD=>,O>J:,AD?D=LM>,A>,8H,W,FH,b,>=,K:O,5A>,N:DOO>,A>,B:,KB>:J:=K>,A>,B:,KJM:@D=D=>,A>,IBLO,A>,3Hb
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A:=O,?CD=O,A>,8Hb,A>O,K:O,`8YaR,(:=O,L=>,:L@J>,M@LA>,`88aS,DB,Q,:,>L,:NO>=K>,A>,@C\DKD@M,:IJ<O,F,:=OR
7#-*%)&'12&%:($'&#-*N*TTK*,*9V9;EDMF"DWHA*TDVM"DXDWH*P
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3.2.2. Formes corticorésistantes
Recommandation n°30 :
h&KKCJA,IJCT>OODC==>Bi
Recommandation n°31 :
IJCT>OODC==>Bi,
19:OOCKD:@DC=,A9L=>,T:DNB>,ACO>,A>,KCJ@DKCpA>O,`HR8H,?UgfUgPa,>O@,IJMKC=DOM>R,
h&KKCJA,IJCT>OODC==>Bi
!"#$%%&'(&)*$'+,("+,-$''"+,.!&)*/0"+,%"(*#&1"+&1(,'2,34,5,+6'(!$%",'".7!$)*/0"
4m
Recommandation n°32 :
A>,@C\DKD@M,W,B:,KDKBCOICJD=>,&S,B>O,@J:D@>?>=@O,:B@>J=:@DTO,OLD;:=@O,O>JC=@,L@DBDOMO5
;%>0($'15-I
9)&'12&%:($'&#-
!*S0%/#*B*6
(5
!*S0%/#*B*6
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,
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3.3.2. Coût approximatif du traitement par hémodialyse
3.3.3. Coût approximatif de la transplantation rénale
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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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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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6.Références bibliographiques
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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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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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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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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