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Comparative Study
. 2024 Apr 25:2024:4283928.
doi: 10.1155/2024/4283928. eCollection 2024.

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

Anna Drynda  1 , Agnieszka Padjas  2 , Krzysztof Wójcik  2 , Radosław Dziedzic  3 , Grzegorz Biedroń  2 , Katarzyna Wawrzycka-Adamczyk  2 , Anna Włudarczyk  2 , Joanna Wilańska  2 , Jacek Musiał  2 , Zbigniew Zdrojewski  4 , Zenobia Czuszyńska  4 , Anna Masiak  4 , Maria Majdan  5 , Radosław Jeleniewicz  5 , Hanna Augustyniak-Bartosik  6 , Katarzyna Jakuszko  6 , Magdalena Krajewska  6 , Alicja Dębska-Ślizień  7 , Hanna Storoniak  7 , Barbara Bułło-Piontecka  7 , Witold Tłustochowicz  8 , Joanna Kur-Zalewska  8 , Małgorzata Wisłowska  9 , Piotr Głuszko  9 , Marta Madej  10 , Ewa Jassem  11 , Iwona Damps-Konstańska  11 , Eugeniusz Kucharz  12 , Marek Brzosko  13 , Marcin Milchert  13 , Anna Hawrot-Kawecka  14 , Joanna Miłkowska-Dymanowska  15 , Paweł Górski  15 , Anna Lewandowska-Polak  16 , Joanna Makowska  16 , Joanna Zalewska  17 , Lech Zaręba  18 , Stanisława Bazan-Socha  2
Affiliations
Comparative Study

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

Anna Drynda et al. J Immunol Res. .

Abstract

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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Conflict of interest statement

All authors have declared no conflicts of interest.

Figures

Figure 1
Figure 1
Clinical manifestations in MPO-ANCA-positive and ANCA-negative EGPA subsets. Categorical variables are presented as percentages. Abbreviations: ANCA, anti-neutrophil cytoplasmic antibodies; MPO-ANCA, anti-myeloperoxidase antibodies; and ENT, ear/nose/throat.
Figure 2
Figure 2
Classification tree for differentiating EGPA and GPA HE. The highest row in every node is the diagnosis; the middle row is the number of classified patients/total number of patients in the node; the lowest row is the percentage of the entire sample. Abbreviations: EGPA, eosinophilic granulomatosis with polyangiitis; GPA HE, granulomatosis with polyangiitis and elevated blood eosinophil count.
Figure 3
Figure 3
The receiver operating characteristic curve for the model differentiating patients with EGPA and GPA HE.
See this image and copyright information in PMC

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