4. Subgrouping ANCA-Associated Vasculitis According To Antibody Specifity and Clinicopathologic Classification
Creators: name: Önay Gerçik
name: Solmaz, Dilek
name: Çakalağaoğlu, Fulya
name: Kabadayı, Gökhan
name: Soypaçacı, Zeki
name: Akar, Servet
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Objectives:</strong> In this study we aimed to evaluate the effects of classification based on clinicopathological criteria and ANCA specificity in our AAV patients, retrospectively. <strong>Methods:</strong> We reviewed the medical records of all patients diagnosed as AAV between January 2014 and December 2018 in our department. Patients were categorized as granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) according to the 2012 Chapel Hill consensus nomenclature. Patients with available ANCA ELISA results were included. Associations between mortality, renal survival and AAV subgroups were analyzed using Kaplan Meier survival curves. <strong>Results:</strong> A total of 61 AAV (45 [73.7%] GPA, 16 [26.2%] MPA, 44 [72.1%] PR3 positive and 17 [27.8%] MPO positive) patients were included. Patients’ characteristics are summarized in table 1. Overall, both the GPA and anti-PR3 subgroups exhibited renal involvement less frequently (p=0.015 and p=0.012 respectively) and ear, nose or throat involvement more frequently than did the MPA and anti-MPO subgroups (both p&lt;0.001). Renal survival was median 30 (IQR 61.75) months for MPA patients and 48 (IQR 82.5) months for GPA patients (p=0.506) during a median follow-up duration of 50 (0-184) months. Overall survival was median 38.5 (IQR 78.2) months for MPA patients and 57 (IQR 73.0) months for GPA patients (p=0,305). According to ANCA subtypes both renal and overall survival were better in PR3 positive group (p=0.018 and p=0.023 respectively). <strong>Conclusions:</strong> In this study, which included a limited number of patients, classification by ANCA subtype appears to be a stronger criterion for predicting prognosis in AAV patients. However, multicenter studies are needed to confirm this trend. <strong>Disclosures: </strong>None. <strong>Table 1</strong>. Demographic and clinical characteristics of ANCA-associated vasculitis patients GPA (n=45) MPA (n=16) Total (n=61) Sex, male (n) (%) 28 (62) 9 (56) 37 (61) Age at diagnosis, mean (SD) (years) 50.3±2.6 62.4±2.7 53.2±15.3 ANCA subtype MPO-ANCA 4 (9) 13 (81) 17 (28) PR3-ANCA 41 (93) 3 (19) 44 (72) Organ/system involvements (n) (%) Kidney 32 (71) 16 (100) 48 (79) Lung 37 (84) 15 (94) 52 (87) ENT (ear-nose-throat) 36 (82) 0 (0) 36 (60) Eye 9 (21) 0 (0) 9 (16) Cardiac 0 (0) 0 (0) 0 (0) GI 3 (7) 0 (0) 3 (5) CNS 1 (2) 0 (0) 1 (2) MNM 1 (2) 1 (6) 2 (3) FFS; (n) (%) 0 9 (24) 0 (0) 9 (17) 1 21 (55) 3 (19) 24 (44) &gt;2 8 (21) 13 (81) 21 (39) Creatinine max. (mg/dl) (median, IQR) (at diagnosis) 2.08 (5.18) 3.27 (3.53) 2.52 (4.77) ESRD development (n) (%) 9 (20) 4 (29) 13 (22) Hemodialysis at diagnosis (n) (%) 13 (42) 9 (56) 22 (39) Plasmapheresis at diagnosis (n) (%) 12 (29) 11 (69) 23 (40) Overall Mortality (n) (%) 7 (16) 6 (38) 13 (21) Follow-up duration (months), median (min-max) 57.0 (0-184) 38.5 (0-130) 50.0 (0-184)