Clinical Characteristics of EGPA Patients in Comparison to GPA Subgroup with Increased Blood Eosinophilia from POLVAS Registry.
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Drynda Anna, Padjas Agnieszka, Wójcik Krzysztof, Dziedzic Radosław, Biedroń Grzegorz, Wawrzycka-Adamczyk Katarzyna, Włudarczyk Anna, Wilańska Joanna, Musiał Jacek, Zdrojewski Zbigniew, Czuszyńska Zenobia, Masiak Anna, Majdan Maria, Jeleniewicz Radosław, Augustyniak-Bartosik Hanna, Jakuszko Katarzyna, Krajewska Magdalena, Dębska-Ślizień Alicja, Storoniak Hanna, Bułło-Piontecka Barbara, Tłustochowicz Witold, Kur-Zalewska Joanna, Wisłowska Małgorzata, Głuszko Piotr, Madej Marta, Jassem Ewa, Damps-Konstańska Iwona, Kucharz Eugeniusz, Brzosko Marek, Milchert Marcin, Hawrot-Kawecka Anna, Miłkowska-Dymanowska Joanna, Górski Paweł, Lewandowska-Polak Anna, Makowska Joanna, Zalewska Joanna, Zaręba Lech, Bazan-Socha Stanisława
Аннотация
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.