■VEXAS(空泡,E1酶,X-linked,自身炎症,躯体)综合征是一种以骨髓衰竭和全身性炎症为特征的遗传性疾病,使患者面临感染的风险。这项研究全面检查了VEXAS患者的机会性感染的患病率,评估其对临床结果和潜在预防措施的影响。
■纳入VEXAS确诊患者。生存分析和逻辑回归用于确定机会性感染和死亡率之间的关联。在前瞻性8个月的观察期内,计算了肺孢子虫肺炎(PJP)和甲疱疹病毒的感染率(IR)与预防的关系。
■在94名VEXAS患者中,6%发展为PJP;15%有α疱疹病毒再激活,水痘带状疱疹病毒(VZV)是最常见的疱疹病毒;10%的人感染了非结核分枝杆菌(NTM)感染。诊断为PJP后,每月的死亡风险显着增加(风险比[HR],72.41[95%置信区间{CI},13.67-533.70])或NTM(HR,29.09[95%CI,9.51-88.79])。在有单纯疱疹病毒(HSV)再激活病史的患者中也观察到死亡几率增加(优势比[OR],12.10[95%CI,1.29-114.80]),但在VZV患者中没有(OR,0.89[95%CI,.30-2.59])。预防PJP(IR,每人每天0.001比0,P<.01)和VZV(IR,每人每天0.006比0,P=.04)显着降低了感染率,需要治疗的人数分别为4和7。
■机会性感染在VEXAS患者中很常见。患有PJP的患者,HSV,或NTM的死亡风险增加。预防PJP和VZV是非常有效的。
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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 per person-day, P = .04) markedly decreased infection rates with a number needed to treat of 4 and 7, respectively.
UNASSIGNED: Opportunistic infections are common in patients with VEXAS. Patients who develop PJP, HSV, or NTM are at increased risk for death. Prophylaxis against PJP and VZV is highly effective.