Figure S1: Multivariable Analysis with Mortality of Matched General Population Subtracted

Figure S1: Multivariable Analysis with mortality of matched general population subtracted

Figure S2 a: Patient survival (Kaplan-Meier) according to clinical diagnosis; Wegener’s granulomatosis (Wegener) and Microscopic Polyangiitis (MPA), respectively

Figure S2 b: Patient survival (Kaplan-Meier) according to type of ANCA; Proteinase3-ANCA (PR3), Myeloperoxidase-ANCA (MPO), negative ANCA (Negative) and double positive (MP3)

Excluding patients with missing ANCA (N=8),

Figure S2 c: Patient survival (Kaplan-Meier) according to age at baseline, categorised into quartiles

Figure S2 d: Patient survival (Kaplan-Meier) according to renal function at baseline; Chronic Kidney Disease (CKD) stages trichotomised

Figure S2 e: Patient survival (Kaplan-Meier) according to BVAS1 quartiles

Table S1: Results from the multivariable Cox-regression analysis using patients only

Manuscript LTFU patient survival – appendix version 19th September, 2010. 1

Covariate / Univariate analysis / Multivariable analysis
without time dependency / Multivariable analysis with time dependency for Hb, WBC, and platelets
Coeff. / HR / 95%CI / p-value / Coeff. / HR / 95%CI / p-value / Coeff. / HR / 95%CI / p-value
Entry diagnosis WG / 0 / 1 / - / reference / 0 / 1 / - / reference / 0 / 1 / - / reference
Entry diagnosis MPA / 0.93 / 2.53 / 1.77-3.62 / <0.00001 / 0.264393 / 1.30 / 0.828-2.05 / 0.25 / 0.287 / 1.33 / 0.837-2.12 / 0.23
CKD trichot A / 0 / 1 / - / reference / 0 / 1 / - / reference / 0 / 1 / - / reference
CKD trichot B / 0.865 / 2.38 / 1.23-4.58 / 0.0097 / -0.062785 / 0.939 / 0.462-1.91 / 0.86 / 0.101 / 1.11 / 0.535-2.29 / 0.79
CKD trichot C / 2.062 / 7.86 / 4.30-14.4 / <0.00001 / 0.919867 / 2.51 / 1.21-5.19 / 0.013 / 1.03 / 2.80 / 1.30-6.03 / 0.0084
Age / 0.0781 / 1.08 / 1.06-1.10 / <0.00001 / 0.061662 / 1.06 / 1.04-1.08 / <0.00001 / 0.0592 / 1.06 / 1.04-1.08 / <0.00001
Sex female / 0 / 1 / - / reference / 0 / 1 / - / reference / 0 / 1 / - / reference
Sex male / 0.292 / 1.34 / 0.946-1.90 / 0.10 / 0.275819 / 1.32 / 0.915-1.90 / 0.14 / 0.244 / 1.28 / 0.878-1.86 / 0.20
BVAS / 0.0197 / 1.02 / 1.00-1.04 / 0.054 / 0.022924 / 1.02 / 0.999-1.05 / 0.059 / 0.028 / 1.03 / 1.01-1.05 / 0.023
EntryANCA PR3 / 0 / 1 / - / reference / 0 / 1 / - / reference / 0 / 1 / - / reference
EntryANCA neg / 0.159 / 1.17 / 0.423-3.25 / 0.76 / -0.049288 / 0.952 / 0.332-2.73 / 0.93 / 0.0361 / 1.04 / 0.364-2.96 / 0.95
EntryANCA MPO / 0.924 / 2.52 / 1.75-3.62 / <0.00001 / 0.414821 / 1.51 / 0.977-2.35 / 0.063 / 0.367 / 1.44 / 0.928-2.24 / 0.1
EntryANCA PR3 and MPO / 0.336 / 1.40 / 0.436-4.49 / 0.57 / 0.875636 / 2.40 / 0.730-7.89 / 0.15 / 0.851 / 2.34 / 0.703-7.79 / 0.17
Hb (without time dependency) / -0.172 / 0.842 / 0.772-0.919 / 0.00011 / -0.035911 / 0.965 / 0.864-1.08 / 0.52
Hb (with time dependency) / -0.520 / 0.594 / 0.538-0.656 / <0.00001 / -0.281 / 0.755 / 0.667-0.854 / <0.00001
Hb log time / 0.242 / 1.27 / 1.21-1.35 / <0.00001 / 0.199 / 1.22 / 1.14-1.30 / <0.00001
WBC (without time dependency) / 0.0136 / 1.01 / 0.976-1.05 / 0.49 / 0.012896 / 1.01 / 0.970-1.06 / 0.56
WBC (with time dependency) / 0.0187 / 1.02 / 0.989-1.05 / 0.22 / 0.0106 / 1.01 / 0.974-1.05 / 0.58
WBC log time / -0.0457 / 0.955 / 0.943-0.968 / <0.00001 / -0.0444 / 0.957 / 0.941-0.972 / <0.00001
Platelets (without time dependency) / -0.00222 / 0.998 / 0.997-0.999 / 0.00045 / -0.000438 / 1.00 / 0.998-1.00 / 0.53
Platelets (with time dependency) / 0.000896 / 1.00 / 0.999-1.00 / 0.27 / -0.000012 / 1.00 / 0.998-1.00 / 0.99
Platelets log time / -0.002046 / 0.998 / 0.997-0.999 / <0.00001 / -0.000136 / 1.00 / 0.999-1.00 / 0.74
CRP / -0.000008 / 1.00 / 0.999-1.00 / 0.99 / -0.000433 / 1.00 / 0.997-1.00 / 0.70 / -0.000173 / 1.00 / 0.998-1.00 / 0.88

Dp double positive

Table S2: Results from the multivariable Cox-regression where the mortality of an age, sex, year and country matched cohort was used as offset

Covariate / Univariate analysis / Multivariable analysis
without time dependency / Multivariable analysis with time dependency for Hb, WBC, and platelets
Coeff. / HR / 95%CI / p-value / Coeff. / HR / 95%CI / p-value / Coeff. / HR / 95%CI / p-value
Entry diagnosis WG / 0 / 1 / - / reference / 0 / 1 / - / reference / 0 / 1 / - / reference
Entry diagnosis MPA / 0.430 / 1.54 / 1.08-2.19 / 0.018 / 0.194 / 1.21 / 0.779-1.89 / 0.39 / 0.303 / 1.35 / 0.852-2.15 / 0.20
CKD trichot A / 0 / 1 / - / reference / 0 / 1 / - / reference / 0 / 1 / - / reference
CKD trichot B / 0.0147 / 1.01 / 0.527-1.95 / 0.97 / -0.256 / 0.774 / 0.377-1.59 / 0.49 / -0.0210 / 0.811 / 0.386-1.70 / 0.58
CKD trichot C / 1.41 / 4.08 / 2.23-7.46 / <0.00001 / 1.10 / 3.02 / 1.48-6.14 / 0.0023 / 1.09 / 2.96 / 1.39-6.30 / 0.0048
Age / -0.0116 / 0.988 / 0.970-1.01 / 0.22 / -0.0240 / 0.976 / 0.957-0.996 / 0.019 / -0.0293 / 0.971 / 0.952-0.991 / 0.0045
Sex female / 0 / 1 / - / reference / 0 / 1 / - / reference / 0 / 1 / - / reference
Sex male / -0.356 / 0.700 / 0.495-0.990 / 0.044 / -0.192 / 0.825 / 0.566-1.20 / 0.32 / -0.378 / 0.686 / 0.464-1.01 / 0.058
BVAS / 0.0342 / 1.03 / 1.01-1.06 / 0.0013 / 0.0325 / 1.03 / 1.01-1.06 / 0.014 / 0.0434 / 1.04 / 1.02-1.07 / 0.0014
EntryANCA PR3 / 0 / 1 / - / reference / 0 / 1 / - / reference / 0 / 1 / - / reference
EntryANCA neg / 0.302 / 1.35 / 0.488-3.75 / 0.56 / -0.0499 / 0.951 / 0.321-2.82 / 0.93 / 0.297 / 1.35 / 0.467-3.88 / 0.58
EntryANCA MPO / 0.517 / 1.68 / 1.17-2.41 / 0.0051 / 0.607 / 1.84 / 1.21-2.79 / 0.0044 / 0.544 / 1.72 / 1.12-2.65 / 0.013
EntryANCA PR3 + MPO / 0.302 / 1.35 / 0.421-4.34 / 0.61 / 0.727 / 2.07 / 0.612-7.00 / 0.24 / 0.892 / 2.44 / 0.720-8.26 / 0.15
Hb (without time dependency) / -0.195 / 0.823 / 0.752-0.901 / <0.00001 / -0.107 / 0.899 / 0.803-1.01 / 0.063
Hb (with time dependency) / -0.877 / 0.416 / 0.379-0.457 / <0.00001 / -0.718 / 0.488 / 0.434-0.547 / <0.00001
Hb log time / 0.413 / 1.51 / 1.44-1.59 / <0.00001 / 0.395 / 1.49 / 1.40-1.58 / <0.00001
WBC (without time dependency) / 0.0497 / 1.05 / 1.01-1.09 / 0.011 / 0.0400 / 1.04 / 0.995-1.09 / 0.084
WBC (with time dependency) / 0.0880 / 1.09 / 1.06-1.12 / <0.00001 / 0.0714 / 1.07 / 1.03-1.12 / 0.00021
WBC log time / -0.0579 / 0.944 / 0.932-0.955 / <0.00001 / -0.0619 / 0.940 / 0.925-0.955 / <0.00001
Platelets (without time dependency) / -0.000679 / 1.00 / 0.998-1.00 / 0.27 / -0.000346 / 1.00 / 0.998-1.00 / 0.64
Platelets (with time dependency) / 0.00636 / 1.01 / 1.01-1.01 / <0.00001 / 0.000347 / 1.00 / 0.998-1.00 / 0.73
Platelets log time / -0.00383 / 0.996 / 0.995-0.997 / <0.00001 / -0.000238 / 1.00 / 0.999-1.00 / 0.63
CRP / 0.00110 / 1.00 / 0.999-1.00 / 0.12 / -0.00195 / 0.998 / 0.996-1.00 / 0.14 / -0.00131 / 1.00 / 0.996-1.00 / 0.36

Dp-double positiv

Manuscript LTFU patient survival – appendix version 19th September, 2010. 1

Manuscript LTFU patient survival – appendix version 19th September, 2010. 1