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13° CONVEGNOPATOLOGIA IMMUNE E
               13°CONVEGNO PATOLOGIA IMMUNE E
               MALATTIE ORFANE, Torino 21-23/01/10
                                       21-




FARMACI BIOLOGICI NELLE VASCULITI
           ESPERIENZA MONOCENTRICA CMID


                              MIRELLA ALPA


 Dipartimento di Malattie Rare, Immunologiche, Ematologiche ed Immunoematologiche
   Centro di Ricerche di Immunopatologia e Documentazione su Malattie Rare (CMID)
Struttura Complessa a Direzione Universitaria di Immunologia Clinica, Ospedale G. Bosco
CLINICAL FEATURES AND               OUTCOME OF         PSV PTS TREATED WITH ANTICD20 MoAb
 PATIENT,                             ACTIVE ORGAN                     URINARY    HEMATURIA
              FAILING THERAPY                             CREATININE                          VAS SCALE   DIAGNOSIS
AGE/GENDER                            INVOLVEMENT                      PROTEINS
1/39/M         MMF, MTX, SP,        K, PNS, J , F, S, W       0.7        0.4         50          9          mPa
                IVIgG, PE
   C
 2/67/F            MMF, SP               K, F, W             1.1         1.1         50          7          mPa
    I
3/63/M          Oral CYC, SP             K, J, W             2.4         1.3         40          7           mPa
   R
 4/71/F         Oral CYC, SP            PNS, J, F            0.9         0.5           0         6           mPa
   R
5/69/M             MMF, SP           K, PNS, J, F, W         1.4         1.4          15         6           WG
relapsing

 6/62/F             MMF,            K, L, F,W, PNS, J,       2.1         2.2          20         9           WG
    I             SP, IVIgG               M, ent
7/60/M              CYC,             PNS, S, J, H ,W         1.2         0.3           0         9           CS
   R              SP, IVIgG

8/35/M             Steroid            L, F, W, J, ent        0.9        0.1           0          8           CS
   C
9/50/M        Steroid, Mtx, anti-    PNS, W, J, ent          0.8        0.1           5          7           WG
                     TNF
   R
10/49/F       SP, CYC, AZA, CyA     K, W, J, PNS, ent        1.1        3.4           5          8           WG
   R
11/68/F      SP, Oral CYC, IVIgG,     K, ent, CNS, L          2         0.9          10          9           WG
                      PE
   R
PATIENTS AND PROTOCOL

             11 PTS: 5 WOMEN, 6 MEN (5 WG, 4 MPA, 2 CS)
                PTS:                   WG, MPA, CS)
             WITH INVOLVEMENT OF
             KIDNEY #7
             PNS #7
             ENT #5
             LUNG #3
             RETROORBITAL OR SINUS GRANULOMATA #2
             CNS   #1

RITUXIMAB 375 mg/sm DAYS # 0, 7, 14, 21 plus 2 DOSES AT 1 AND 2
MTHS
35



30   29

     28

     26    B-VAS
25



      20
20



      15           16
15

     12
     11
10



                   6
5

                   3
                   2
                   2

0
      T0           T12

                        p<0.01 at 12 months
20

18   18

16

14
     13        VDI
12

10
     9
 8   8
      7
 6    6               6

 4    4
                      4
                      3
 2                    2
                      1
 0        0           0
          T0         T12

                       p<0.01 at 12 months

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Alpa Mirella Torino 13° Convegno Patologia Immune E Malattie Orfane 21 23 Gennaio 2010 [Modalità Compati

  • 1. 13° CONVEGNOPATOLOGIA IMMUNE E 13°CONVEGNO PATOLOGIA IMMUNE E MALATTIE ORFANE, Torino 21-23/01/10 21- FARMACI BIOLOGICI NELLE VASCULITI ESPERIENZA MONOCENTRICA CMID MIRELLA ALPA Dipartimento di Malattie Rare, Immunologiche, Ematologiche ed Immunoematologiche Centro di Ricerche di Immunopatologia e Documentazione su Malattie Rare (CMID) Struttura Complessa a Direzione Universitaria di Immunologia Clinica, Ospedale G. Bosco
  • 2. CLINICAL FEATURES AND OUTCOME OF PSV PTS TREATED WITH ANTICD20 MoAb PATIENT, ACTIVE ORGAN URINARY HEMATURIA FAILING THERAPY CREATININE VAS SCALE DIAGNOSIS AGE/GENDER INVOLVEMENT PROTEINS 1/39/M MMF, MTX, SP, K, PNS, J , F, S, W 0.7 0.4 50 9 mPa IVIgG, PE C 2/67/F MMF, SP K, F, W 1.1 1.1 50 7 mPa I 3/63/M Oral CYC, SP K, J, W 2.4 1.3 40 7 mPa R 4/71/F Oral CYC, SP PNS, J, F 0.9 0.5 0 6 mPa R 5/69/M MMF, SP K, PNS, J, F, W 1.4 1.4 15 6 WG relapsing 6/62/F MMF, K, L, F,W, PNS, J, 2.1 2.2 20 9 WG I SP, IVIgG M, ent 7/60/M CYC, PNS, S, J, H ,W 1.2 0.3 0 9 CS R SP, IVIgG 8/35/M Steroid L, F, W, J, ent 0.9 0.1 0 8 CS C 9/50/M Steroid, Mtx, anti- PNS, W, J, ent 0.8 0.1 5 7 WG TNF R 10/49/F SP, CYC, AZA, CyA K, W, J, PNS, ent 1.1 3.4 5 8 WG R 11/68/F SP, Oral CYC, IVIgG, K, ent, CNS, L 2 0.9 10 9 WG PE R
  • 3. PATIENTS AND PROTOCOL 11 PTS: 5 WOMEN, 6 MEN (5 WG, 4 MPA, 2 CS) PTS: WG, MPA, CS) WITH INVOLVEMENT OF KIDNEY #7 PNS #7 ENT #5 LUNG #3 RETROORBITAL OR SINUS GRANULOMATA #2 CNS #1 RITUXIMAB 375 mg/sm DAYS # 0, 7, 14, 21 plus 2 DOSES AT 1 AND 2 MTHS
  • 4.
  • 5.
  • 6. 35 30 29 28 26 B-VAS 25 20 20 15 16 15 12 11 10 6 5 3 2 2 0 T0 T12 p<0.01 at 12 months
  • 7. 20 18 18 16 14 13 VDI 12 10 9 8 8 7 6 6 6 4 4 4 3 2 2 1 0 0 0 T0 T12 p<0.01 at 12 months