Opportunistic Infections, Mortality Risk, and Prevention Strategies in Patients With Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic (VEXAS) Syndrome

作者：Mary M. Czech, Jennifer Cuellar‐Rodríguez, Bhavisha A. Patel, Emma M. Groarke, Edward W. Cowen, Benjamin A. Turturice, David B. Beck, Lorena Wilson, Wendy Goodspeed, Ivana Darden, Neal S. Young, Dennis D. Hickstein, Amanda K. Ombrello, Patrycjia Hoffman, E.A. Arikan, Ninet Sinaii, Londa Hathaway, Leslie Castelo‐Soccio, Alice Fike, D Kastner, Peter C. Grayson, Marcela A. Ferrada · 发表于：Open Forum Infectious Diseases · 年份：2024 · DOI：10.1093/ofid/ofae405 · 被引用次数：24 · 研究领域：Otitis Media and Relapsing Polychondritis、Spondyloarthritis Studies and Treatments、Autoimmune and Inflammatory Disorders Research

Abstract Background VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a genetic disorder characterized by bone marrow failure and systemic inflammation, putting patients at risk for infections. This study comprehensively examines the prevalence of opportunistic infections in patients with VEXAS, evaluating their impact on clinical outcomes and potential preventive measures. Methods Patients with confirmed VEXAS were included. Survival analysis and logistic regression were used to identify associations between opportunistic infections and mortality. Infection rates (IRs) for Pneumocystis jirovecii pneumonia (PJP) and alphaherpesviruses were calculated over a prospective 8-month observation period in relationship to prophylaxis. Results Of 94 patients with VEXAS, 6% developed PJP; 15% had alphaherpesvirus reactivation, with varicella zoster virus (VZV) being the most common herpesvirus; and 10% contracted a nontuberculous mycobacterial (NTM) infection. Risk of death was significantly increased per month following a diagnosis of PJP (hazard ratio [HR], 72.41 [95% confidence interval {CI}, 13.67–533.70]) or NTM (HR, 29.09 [95% CI, 9.51–88.79]). Increased odds for death were also observed in patients with a history of herpes simplex virus (HSV) reactivation (odds ratio [OR], 12.10 [95% CI, 1.29–114.80]) but not in patients with VZV (OR, 0.89 [95% CI, .30–2.59]). Prophylaxis for PJP (IR, 0.001 vs 0 per person-day, P < .01) and VZV (IR, 0.006 vs 0 p...