Supplemental Table : Demographic and Treatment Data, Outcome

Supplemental Table : Demographic and treatment data, outcome

Patient Sex Age Pre-treatment Treatment 6 Reason for inclusion Organ ANCA PR 3 MPO Crea CRP BVAS VDI Outcome Treatment [years] history with months prior to involvement IF [μmol/l] [mg/dl] (entry) (entry) during follow cumulative CYC entry (mg/day po up dose unless otherwise stated), all on OCS 1 M 70.4 CYC 34g, MMF, CYC 125, CYC 75 active disease while Ch, R neg neg neg 162 6 11 6 CR AZA MTX, IFX on CYC 2 M 40.1 CYC 23.9g, MMF 1.5g high cumulative dose Ch neg neg neg 315 2 6 3 WD after 5th cycle CYC, PE, IFX MMF of CYC disease progression 3 M 62.6 CYC 12.1g, MMF 1g, MMF 2g active disease on Ch pos neg 88 3.1 6 2 CR AZA MMF MMF 4 M 51.3 CYC 110g, AZA CYC 150 high cumulative dose Ch neg neg neg 100 8.8 5 0 CR AZA of CYC 5 M 43.3 CYC 39.6g, AZA CYC 100 active disease while E,R c pos neg 138 3 18 5 PR AZA on CYC 6 F 37.1 CYC 11.1g, MMF 2g, CYC did not tolerate CYC C, Ch, R c pos neg 88 5.2 6 0 WD after 3 cycles in AZA, CYC on MMF, AZA 600iv 2weekly CR due to AE, REL relapse 7 F 35.7 CYC 50.3g, AZA 150 high cumulative dose E c pos neg 55 108 8 5 PR,REL MMF AZA, MTX of CYC 8 F 35.2 CYC 75g, MTX, MTX 10/week high cumulative dose E, Ch c pos neg 63 50 15 9 PR OCS only AZA of CYC 9 F 47.5 CYC 117g, AZA CYC 100 active disease while C, R, E c pos neg 62 4 14 5 WD due to AE after MTX on CYC 4 th cycle after CR 10 M 45.3 CYC 85g, MMF MMF 1g high cumulative dose E, Ch, R c pos neg 97 7 25 7 CR AZA of CYC 11 M 49.6 CYC 43g, MTX CYC 100, CYC active disease while E, Ch, c pos neg 90 112 11 2 WD cycle 3 in PR, MTXk 1000 iv 3 weekly, on CYC pituitary REL granuloma 12 M 66.5 CYC 200g, MTX, MTX 26/week iv active disease on E, Ch c pos neg 67 49 13 1 PR MTX LEF MTX, h/o haemorrhagic cystitis 13 M 41.7 CYC 45g, AZA, LEF 20 active disease on R, E, Ch p neg pos 151 3 21 4 PR MMF LEF LEF, h/o haemorrhagic cystitis 14 F 59.7 CYC 123g, MTX, AZA 1200 iv 3 no response to CYC E, retro- c neg neg 52 13.3 12 2 PR AZA LEF, IFX, ETC, weekly orbital RTX, pulse AZA granuloma 15 F 39.1 CYC 36.6g, CYC 150, MMF 2g active disease on R, C, Ch c pos neg 128 17 19 3 PR MMF MTX, AZA, MMF CYC 16 M 20.3 CYC 20g, MTX MTX 15/week active disease on R, Ch, C c pos neg 100 18 21 5 PR, WD 6th cycle MMF, IVIG MTX after relapse, died 17 M 50.3 CYC 100g, AZA, MMF 2g, MTX h/o bladder ca retro-orbital c neg neg 95 3.4 12 9 CR AZA MTX, MMF, IVIG 15/week granuloma, E 18 F 62.7 CYC 360g, AZA, PRED only h/o haemorrhagic E, Ch c pos neg 107 29 14 14 CR AZA MMF, MTX, CsA cystitis 19 M 77.3 CYC 36g, CsA, CsA 200 h/o bladder carcinoma E, Ch c neg neg 197 9.5 16 13 PR MMF AZA 20 M 58.6 CYC 28g, MTX, AZA 150, AZA 100 h/o haemorrhagic C,E, R c neg neg 105 23 16 4 CR AZA AZA cystitis 21 F 19.4 CYC 7.4g, IVIG, CYC 100, MMF relapse within 6 E, Ch, R c pos neg 63 36 23 1 PR,REL MMF MMF, PE 2g, IVIG 37g 3 months of CYC days, MMF 3g 22 M 62.5 CYC 18g, AZA, MMF 2g severe lymphopaenia E, Ch c pos neg 120 2 4 4 CR,REL AZA ALZ x3, MMF, IFX 23 F 68.8 CYC 4.8g, MMF CYC 800 iv 3 relapse within 3 E c neg neg 73 19 8 4 CR,REL MMF, DSG weekly, MMF 2g months of CYC after REL 24 M 60.3 CYC 2.5g, AZA AZA 150 infections on CYC E, M c pos neg 79 7 12 6 PR,REL AZA 25 M 53.5 CYC 9g, AZA AZA 150 relapse on AZA R, M c pos neg 104 26 17 2 PR AZA 26 M 44.7 CYC 24.6g, CYC 1500 iv 3 active disease despite Ch, E, M c neg neg 89 6 15 3 PR,REL AZA AZA, T/S, IFX, weekly, AZA 150 CYC IVIG, PE 27 M 33.5 CYC 16.2g, CYC 1200 iv 3 active disease despite E, R p neg pos 92 2 10 2 PR,REL AZA MMF weekly, MMF 1.5g CYC 28 M 61.5 CYC ?, IVIG, MMF 1g relapse on MMF R, C, M c pos neg 119 41 19 6 PR MMF AZA, MMF 29 M 67.7 CYC >87g, ATG, AZA 150 high cumulative dose Ch, E p pos neg 95 2 13 4 CR AZA MTX, IFX of CYC 30 F 51.6 CYC 80g, AZA, MMF 2g high cumulative dose E c neg neg 75 9 7 5 PR,REL MMF, DSG ALZ, MTX, IFX of CYC after REL 31 M 40.0 CYC 29g, MMF MMF 2g high cumulative dose E, Ch c pos neg 89 0 17 7 CR MMF of CYC 32 M 38.6 CYC 26g, MTX CYC 200, MTX active disease despite E, Ch neg pos neg 80 2 9 2 PR,REL AZA 10/week CYC 33 M 59.8 MTX, AZA MTX 10/week, poor response to R c neg neg 163 0 10 3 CR MMF AZA 150 MTX, AZA 34 M 43.2 CYC 43g, AZA, MMF 1.5g, MMF high cumulative dose R, E, Ch, M c pos neg 79 128 15 4 PR,REL MMF, DSG MMF 3g of CYC after REL 35 F 44.7 CYC 60g, AZA, MMF 2g high cumulative dose E p neg neg 82 13 7 11 CR, WD 5th cycle, MMF IVIG, MMF, IFX of CYC breast cancer, REL 36 M 59.9 CYC 20.25g, MTX 15/week partial response to E, Ch c neg neg 83 14 8 4 CR,REL AZA; DSG AZA, MTX 3months after REL CYC+6months MTX, hypogonadism on CYC 37 F 36.5 CYC 32g, MTX, MTX 30/week, IFX active disease while E c neg neg 72 6 7 12 PR MTX ALZ, IVIG, AZA, 400 iv 6 weekly on MTX, h/o CsA, MMF, IFX, haemorrhagic cystitis αCD18, LEF 38 F 25.5 MTX MTX 15/week active disease while C, E p neg pos 115 8 11 5 PR, died on MTX 39 M 67.8 CYC 6g, AZA, MTX 40/week active disease while E, Ch c pos neg 137 11 10 9 CR,REL AZA MTX on MTX 40 M 51.6 CYC 16g, MTX, MTX 15/week active disease while E, Ch neg pos neg 75 14 10 6 CR AZA IVIG, AZA on MTX 41 M 52.9 CYC 35g, AZA, MTX 20/week active disease while E, R c pos neg 268 7 20 5 CR,REL AZA MTX on MTX 42 M 65.4 CYC >12g, LEF, MMF 2g h/o malignancy Ch, R c pos neg 279 17 13 14 WD 1st cycle, MMF, IVIG, consent OHCL 43 F 62.8 CYC 12g, MTX, CsA 125, MMF active disease while M, E, N neg neg neg 102 15 17 6 PR, WD due to AE MMF MMF, CsA 1.5g, MTX on MTX, renal after 3 cycles 15/week transplant 44 F 53.9 CYC 22g, MMF OCS only severe leukopaenia E c pos neg 56 10 3 CR MMF on CYC 45 F 33.5 CYC 18g, MMF MMF 1g active disease after E p neg pos 80 10 4 0 CR,REL MMF 6month CYC

F, female; M, male; CYC, cyclophosphamide; MTX, methotrexate; MMF, mycophenolate mofetil; AZA, azathioprine; LEF, leflunomide; OHCL, hydroxychloroquine; CsA, cyclosporine A; T/S, trimethoprim/sulfamethoxazole; OCS, oral corticosteroids; IVIG, intravenous immunoglobulins; ALZ, alemtuzumab; ATG, anti-thymocyte globulin; ETC, etanercept; IFX, infliximab; RTX, rituximab; αCD18, anti-CD18 monoclonal antibody; DSG, deoxyspergualin; ANCA-IF, antineutrophil cytoplasm antibodies pattern on immuno-fluorescence; MPO, anti myeloperoxidase antibodies; PR3, anti proteinase 3 antibodies; CRP, c-reactive protein; Crea, creatinine; BVAS, Birmingham Vasculitis Activity Score; VDI, Vasculitis Damage Index; C, skin; E, ear/nose/throat; M, eyes; Ch, chest; R, kidney; N, nervous system; CR, complete remission; PR, partial remission; REL, relapse; WD, withdrawn; AE, adverse event; h/o, history of