Association of antineutrophil cytoplasmic antibody (ANCA) specificity with the demographic and clinical characteristics of patients with ANCA-associated vasculitides

Wójcik, Krzysztof; Masiak, Anna; Jeleniewicz, Radosław; Jakuszko, Katarzyna; Brzosko, Iwona; Storoniak, Hanna; Kur-Zalewska, Joanna; Wisłowska, Małgorzata; Madej, Marta; Hawrot-Kawecka, Anna; Głuszko, Piotr; Kucharz, for POLVAS consortium Eugeniusz J.

doi:10.20452/pamw.16187

Cited by 6 publications

(5 citation statements)

References 16 publications

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“…Interestingly, in our data, ANCA-positive patients were older. It might be explained by the later age of vasculitic component onset [ 30 , 31 ], since in AAVs, rises of ANCA may gain on relapse of clinical symptoms at least 6–12 months [ 32 ]. However, studies concerning the role of ANCA monitoring in AAVs were restricted to GPA and MPA patients, making it unclear whether the results can be extrapolated to EGPA [ 27 ].…”

Section: Discussionmentioning

confidence: 99%

Clinical Characteristics of EGPA Patients in Comparison to GPA Subgroup with Increased Blood Eosinophilia from POLVAS Registry

Drynda,

Padjas,

Wójcik

et al. 2024

Journal of Immunology Research

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Objective. To characterize the eosinophilic granulomatosis with polyangiitis (EGPA) population from the POLVAS registry depending on ANCA status and diagnosis onset, including their comparison with the granulomatosis with polyangiitis (GPA) subset with elevated blood eosinophilia (min. 400/μl) (GPA HE) to develop a differentiating strategy. Methods. A retrospective analysis of the POLVAS registry. Results. The EGPA group comprised 111 patients. The ANCA-positive subset (n = 45 [40.54%]) did not differ from the ANCA-negative one in clinics. Nevertheless, cardiovascular manifestations were more common in ANCA-negative patients than in those with anti-myeloperoxidase (MPO) antibodies (46.97% vs. 26.92%, p = 0.045). Patients diagnosed before 2012 (n = 70 [63.06%]) were younger (median 41 vs. 49 years, p <0.01), had higher blood eosinophilia at diagnosis (median 4,946 vs. 3,200/μl, p <0.01), and more often ear/nose/throat (ENT) and cardiovascular involvement. GPA HE comprised 42 (13.00%) out of 323 GPA cases with reported blood eosinophil count. Both GPA subsets had a lower prevalence of respiratory, cardiovascular, and neurologic manifestations but more often renal and ocular involvement than EGPA. EGPA also had cutaneous and gastrointestinal signs more often than GPA with normal blood eosinophilia (GPA NE) but not GPA HE. The model differentiating EGPA from GPA HE, using ANCA status and clinical manifestations, had an AUC of 0.92, sensitivity of 96%, and specificity of 95%. Conclusion. Cardiovascular symptoms were more prevalent in the ANCA-negative subset than in the MPO-ANCA-positive one. Since EGPA and GPE HE share similarities in clinics, diagnostic misleading may result in an inappropriate therapeutic approach. Further studies are needed to optimize their differentiation and tailored therapy, including biologics.

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Section: Discussionmentioning

confidence: 99%

Clinical Characteristics of EGPA Patients in Comparison to GPA Subgroup with Increased Blood Eosinophilia from POLVAS Registry

Drynda,

Padjas,

Wójcik

et al. 2024

Journal of Immunology Research

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“…Regarding age, MPO-ANCA patients are usually older, with a median age estimated as 67 years. compared to 60 years., the median age of PR3-ANCA patients [ 5 , 6 , 7 ].…”

Section: Epidemiologymentioning

confidence: 99%

Type of ANCA May Be Indispensable in Distinguishing Subphenotypes of Different Clinical Entities in ANCA-Associated Vasculitis

Konstantouli

Λιούλιος

Stai

et al. 2022

Life

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The traditional nomenclature system for classifying antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) based on clinical phenotype describes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA) as distinct clinical entities. This classification has proved its expedience in clinical trials and everyday clinical practice; yet, a substantial overlap in clinical presentation still exists and often causes difficulties in prompt definition and clinical distinction. Additionally, new insights into the AAV pathogenesis point out that PR3 and MPO-AAV may not represent expressions of the same disease spectrum but rather two distinct disorders, as they display significant differences. Thus, it is supported that a classification based on ANCA serotype (PR3-ANCA, MPO-ANCA or ANCA-negative) could be more accurate and also closer to the nature of the disease compared to the phenotype-based one. This review aims to elucidate the major differences between PR3 and MPO-AAV in terms of epidemiology, pathogenesis, histological and clinical manifestations and response to therapeutic approaches.

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“…ANCA is a useful diagnostic marker, and it may have a specific pathogenic mechanism in vasculitis. MPO-and PR3-ANCA are found in the three clinical phenotypes of AAV, with PR3-positive ANCA often associated with GPA and MPO-positive ANCA associated with MPA [6] [7] [8] [9]. Studies have shown that MPO-and PR3-ANCA vasculitis are genetically different, and disease classification based on the ANCA subtype helps to differentiate clinical phenotypes and better predict AAV recurrence [10].…”

Section: Introductionmentioning

confidence: 99%

Clinical Characteristics and Prognosis of Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis According to Serotype: A Retrospective Study of 349 Chinese Patients

Xu,

Feng,

Lin

et al. 2023

JBM

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Objective: According to clinical phenotypic classification, there is a significant overlap of clinical features between different anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), and disease classification based on ANCA subtype helps to differentiate clinical phenotypes. We investigated the clinical features and outcomes of patients based on ANCA serotype classification. Methods: Clinical and laboratory data were collected retrospectively. We compared clinical manifestations and organ involvement based on serotype. The risk factors for death and renal survival were investigated with univariate and multivariate Cox regression models. Results: Patients with MPO-ANCA were predominant, whose median age and lung involvement at diagnosis was higher than that of the PR3-ANCA patients. Compared to the AAV patients without renal involvement, those with renal involvement have older, anemia, low complement C3, and hypoproteinemia, more likely to have cardiovascular and abdominal involvement, and have less lung involvement. Multivariate Cox analysis revealed that age ≥ 65 years (HR = 2.611, p < 0.001), serum creatinine (SCR) ≥ 500 μmol/L (HR = 1.546, p = 0.019), BVAS ≥ 15 (HR = 1.943, p = 0.001), low C3 (HR = 1.696, p = 0.008), and hypoproteinemia (HR = 1.438, p = 0.044) were associated with mortality. SCR ≥ 500 μmol/L (HR = 13.583, p < 0.001), BVAS ≥ 15 (HR = 1.660, p = 0.020), low C3 (HR = 1.506, p = 0.049) were independent detrimental factors for renal survival, and immunosuppressive treatment was a protective factor for renal survival (HR = 0.523, p = 0.003). Conclusions: Clinical manifestations varied by AAV categories. Age, SCR, BVAS, low C3 and hypoproteinemia at diagnosis were

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Association of antineutrophil cytoplasmic antibody (ANCA) specificity with the demographic and clinical characteristics of patients with ANCA-associated vasculitides

Abstract: Association of antineutrophil cytoplasmic antibody (ANCA) specificity with the demographic and clinical characteristics of patients with ANCA-associated vasculitides

Cited by 6 publications

References 16 publications

Clinical Characteristics of EGPA Patients in Comparison to GPA Subgroup with Increased Blood Eosinophilia from POLVAS Registry

Clinical Characteristics of EGPA Patients in Comparison to GPA Subgroup with Increased Blood Eosinophilia from POLVAS Registry

Type of ANCA May Be Indispensable in Distinguishing Subphenotypes of Different Clinical Entities in ANCA-Associated Vasculitis

Clinical Characteristics and Prognosis of Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis According to Serotype: A Retrospective Study of 349 Chinese Patients

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