Clinical and laboratory markers to distinguish VEXAS from Schnitzler's syndrome: data from the AIDA network registries.
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Caggiano Valeria, Sbalchiero Jessica, Frassi Micol, Martín-Nares Eduardo, Hinojosa-Azaola Andrea, Sikora Mariusz, Jahnz-Różyk Karina, Crisafulli Francesca, Franceschini Franco, Airò Paolo, Guaracha-Basañez Guillermo Arturo, Torres-Ruiz Jiram, Sfriso Paolo, Bindoli Sara, Baggio Chiara, Hernández-Rodríguez José, Caverzaschi Verónica Gómez, Espinosa Gerard, Mayrink Giardini Henrique A, Alves Cordeiro Rafael, González-García Andrés, Peña Rodríguez Mercedes, Lopalco Giuseppe, Iannone Florenzo, Viapiana Ombretta, Tufan Abdurrahman, Kucuk Hamit, Hissaria Pravin, Beecher Mark, De Paulis Amato, Mormile Ilaria, Dagna Lorenzo, Campochiaro Corrado, Gidaro Antonio, La Cava Leyla, Bugatti Serena, Milanesi Alessandra, Ruiz-Irastorza Guillermo, Piga Matteo, Conti Fabrizio, Moscato Paolo, Opris-Belinski Daniela, Vitetta Rosetta, Chighizola Cecilia, Recke Andreas, Tornero-Romero Fernando, Prete Marcella, Govoni Marcello, Emmi Giacomo, Kawakami-Campos Perla Ayumi, Triggianese Paola, Gurnari Carmelo, Ragab Gaafar, Balistreri Alberto, Ziȩtkiewicz Marcin, Wiesik-Szewczyk Ewa, Frediani Bruno, Fabiani Claudia, Sicuranza Anna, Bocchia Monica, Cantarini Luca, Vitale Antonio
Аннотация
METHODS: Data from male-only patients with Schnitzler's syndrome or VEXAS syndrome were obtained from international AIDA Network registries. Subjects with Schnitzler's syndrome were compared to VEXAS patients with urticarial skin manifestations resembling cutaneous features typically observed in Schnitzler's syndrome.
RESULTS: A total of 19 VEXAS patients and 18 patients with Schnitzler's syndrome were enrolled. At univariate binary logistic regression, the diagnosis of VEXAS syndrome was associated with the age at disease onset (OR = 1.08, 95% CI. 1.01-1.16, p = 0.02), hemoglobin levels (OR = 0.44, 95% CI. 0.26-0.77, p = 0.003), anemia (OR = 13.9, 95% CI. 3.4-5.7, p = 0.02), leucocytosis (OR = 0.04, 95% CI. 0.06-0.22, p < 0.001), lymphadenopathy (OR = 7.8, 95% CI. 1.41-45.4, p = 0.02), and thrombocytopenia (OR = 13.5, 95% CI. 1.47-123.7, p = 0.02). In the multivariable logistic regression analysis with the stepwise forward selection approach, the diagnosis of VEXAS syndrome was significantly associated with the age at disease onset (OR: 1.13, 95% CI: 1.02-1.30, p = 0.04) and the presence of lymphadenopathy (OR: 67.49, 95% CI: 5.36-3284.89, p = 0.007), while thrombocytopenia showed a trend toward statistical significance (OR: 12.02, 95% CI: 1.07-315.86, p = 0.06).
CONCLUSIONS: Patients with lymphadenopathy, thrombocytopenia, anemia, particularly in older age and in the absence of leucocytosis, are more likely to be affected by VEXAS syndrome rather than Schnitzler's syndrome.