Article Figures & Data
Figures
- Figure 1.
Renal survival by Kaplan-Meier curves according to serology type (139 patients with renal disease at diagnosis; 67 with PR3-ANCA+ and 72 with MPO-ANCA+). PR3: proteinase 3; ANCA: antineutrophil cytoplasmic antibodies; MPO: myeloperoxidase.
- Figure 2.
Patient survival by Kaplan-Meier curves according to serology type (183 patients; 98 with PR3-ANCA+ and 85 with MPO-ANCA+). PR3: proteinase 3; ANCA: antineutrophil cytoplasmic antibodies; MPO: myeloperoxidase.
Tables
- Table 1.
Demographic, laboratory, and clinical characteristics of 183 patients with ANCA-associated vasculitis and ANCA positivity measured by ELISA.
PR3-ANCA, n = 98 MPO-ANCA, n = 85 p Sex, F/M 39/59 48/37 0.024 Diagnosis: GPA/MPA/EGPA 73/25/0 17/64/4 Age at diagnosis, yrs 63.2 ± 15.9 68.7 ± 14.4 0.016 Diagnosis delay, mos 2 (1–4) 2 (1–5) 0.063 Laboratory results C-reactive protein, mg/dl 11.65 (6.5–19.1) 3.4 (0.9–12.4) < 0.001 ESR, mm/h 77 ± 30 58 ± 35 0.004 Hemoglobin, g/dl 11.1 ± 1.82 10.6 ± 1.77 0.004 Thrombocyte count, × 109/l 408 (333–517) 324 (261–400) < 0.001 White blood cell count, × 109/l 11.9 (9.9–15) 9.6 (7.1–13) 0.002 Creatinine, mg/dl 1.46 (0.81–3.03) 2.67 (1.21–5.19) 0.004 Patients with ≥ 3 organ systems involved, n (%) 75 (76) 48 (56) 0.004 Deaths during followup, n (%) 36 (37) 36 (42) 0.438 Organ systems involved at diagnosis General, n (%) 92 (94) 72 (85) 0.042 ENT, n (%) 58 (59) 16 (19) < 0.001 Chest, n (%) 51 (52) 34 (40) 0.103 Nervous, n (%) 14 (14) 8 (9) 0.312 Cutaneous, n (%) 5 (5) 6 (7) 0.579 Mucocutaneous and eyes, n (%) 10 (10) 2 (2) 0.032 Cardiovascular, n (%) 4 (4) 4 (5) 0.837 Abdominal, n (%) 5 (5) 8 (9) 0.258 Renal, n (%) 67 (68) 72 (85) 0.01 -
Results are mean ± SD (normally distributed variables) or median and interquartile range (not normally distributed variables), or number and percentage. GPA: granulomatosis with polyangiitis (formerly Wegener); MPA: microscopic polyangiitis; EGPA: eosinophilic granulomatosis with polyangiitis (Churg-Strauss); ENT: ear-nose-throat; ESR: erythrocyte sedimentation rate; ANCA: antineutrophil cytoplasmic antibody; PR3: proteinase 3; MPO: myeloperoxidase.
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PR3-ANCA n = 67 MPO-ANCA n = 72 p Sex, F/M 24/43 36/36 0.092 Diagnosis: GPA/MPA/EGPA 42/25/0 9/62/1 Age at diagnosis, yrs 64.8 ± 16.4 70.1 ± 14.3 0.043 Diagnosis delay, mos 2 (1–3) 2 (1–4) 0.206 Laboratory results C-reactive protein, mg/dl 11.8 (8.8–17.7) 4.6 (0.9–12.6) < 0.001 ESR, mm/h 80.1 ± 29.6 64.2 ± 36.0 0.047 Hemoglobin, g/dl 10.8 ± 1.89 10.2 ± 1.68 0.05 Thrombocyte count, × 109/l 387 (314–483) 316 (260–386) 0.001 White blood cell count, × 109/l 12.5 (10–15) 9.3 (7.1–12.5) 0.001 Creatinine at diagnosis, mg/dl 2.64 (1.37–4.10) 3.05 (1.73–5.77) 0.062 Creatinine at last followup*, mg/dl 1.19 (0.85–1.60) 1.39 (1.01–1.84) 0.187 Patients with GFR < 50 ml/min at last followup*, n (%) 12/35 (34) 13/27 (48) 0.243 Patients developed ESRD, n (%) 10 (15) 27 (38) 0.003 Patients with ≥ 3 organ systems involved, n (%) 50 (75) 42 (58) 0.042 Death, n (%) 29 (43) 35 (49) 0.529 Organ system involved at diagnosis General, n (%) 64 (96) 60 (83) 0.021 ENT, n (%) 31 (46) 9 (13) < 0.001 Chest, n (%) 32 (48) 29 (40) 0.374 Nervous, n (%) 7 (10) 4 (6) 0.286 Cutaneous, n (%) 4 (6) 3 (4) 0.627 Mucocutaneous and eyes, n (%) 5 (7) 1 (1) 0.078 Cardiovascular, n (%) 2 (3) 3 (4) 0.709 Abdominal, n (%) 4 (6) 8 (11) 0.281 Renal histology findings, n (%) Diffuse crescentic GN, pauciimmune 15 (22) 11 (15) 0.282 Diffuse crescentic GN, IF not done 2 (3) 3 (4) 0.585 Diffuse crescentic GN, granular IgG/C3 1 (1.5) 2 (3) 0.602 Focal necrotizing/crescentic GN, pauciimmune 24 (36) 22 (31) 0.509 Focal necrotizing/crescentic GN, IF not done 3 (4.5) 8 (11) 0.147 Pauciimmune small vessel vasculitis without GN 0 (0) 3 (4) 0.091 Miscellaneous or insufficient material for diagnosis 5 (8) 8 (11) 0.46 No renal biopsy performed 17 (25) 15 (21) 0.525 -
↵* Data on creatinine and GFR < 50 ml/min at last followup (May 2012), included only patients alive and with native kidney function at last followup. All other demographic, clinical, and laboratory data are based on 139 patients with renal involvement. Results are reported as mean ± SD (normally distributed variables) or median and interquartile range (not normally distributed variables), or number and percentage. AAV: ANCA-associated vasculitis; GPA: granulomatosis with polyangiitis (formerly Wegener); MPA: microscopic polyangiitis; EGPA: eosinophilic granulomatosis with polyangiitis (Churg-Strauss); ESRD: endstage renal disease; ENT: ear-nose-throat; GN: glomerulonephritis; ESR: erythrocyte sedimentation rate; ANCA: anti-neutrophil cytoplasmic antibody; PR3: proteinase 3; MPO: myeloperoxidase; GFR: glomerular filtration rate; IF: immunofluorescence.
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HR 95% CI p MPO+ 2.64 1.25–5.58 0.011 Age at diagnosis 1.01 0.98–1.03 0.405 Sex (male) 0.96 0.68–1.34 0.815 S-creatinine 3.17 1.99–5.04 < 0.001 -
Cox regression analysis. MPO: myeloperoxidase.
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- Table 4.
Summary of studies on renal and patient survival in patients with AAV based on ANCA serotype (C-/PR3-ANCA and P-/MPO-ANCA).
Study (reference) Year No. Patients Renal Involvement Specialty Comparison Case Identification ESRD Death Geffriaud-Ricouard, et al16 1993 83 92% Nephrology PR3 vs MPO Single center No difference No difference Franssen, et al18 1995 92 75% “Mixed” PR3 vs MPO Teaching hospitals No difference No difference Hogan, et al13 1996 107 100% Nephrology C vs P-ANCA Specialized units
(Glomerular Disease
Collaborative Network)No difference Worse for C-ANCA Westman, et al19 1998 123 100% Nephrology PR3 vs MPO Single center Worse for PR3# No difference Franssen, et al10 1998 92 75% “Mixed” PR3 vs MPO Teaching hospitals NR No difference Vizjak, et al24 2003 135 100% “Mixed” PR3 vs MPO Single center Worse MPO No difference Weidner, et al12 2004 80 100% Nephrology PR3 vs MPO Single center No difference Worse for PR3 Rihova, et al25 2005 61 100% Nephrology C-vs P-ANCA Single center No difference No difference Flossmann, et al26 2011 535 100% Mixed PR3 vs MPO Multicenter clinical trials NR Worse for MPO Lionaki, et al5 2012 502 97% Nephrology PR3 vs MPO Specialized units
(Glomerular Disease
Collaborative Network)Worse for MPO Worse for MPO de Joode, et al20 2013 212 100% Nephrology MPO vs PR3 Single center Worse for MPO Worse for MPO* Present study 2013 183 76% “Mixed” PR3 vs MPO Population-based Worse for MPO No difference
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