Rukavina Kresimir, Zlopasa Ozrenka, Vukovic Brinar Ivana, Dzubur Feda, Anic Branimir, Vujaklija Brajkovic Ana
Department of Internal Medicine, University Hospital Center Zagreb, Kispaticeva 12, 10000 Zagreb, Croatia.
School of Medicine, University of Zagreb, Salata 3, 10000 Zagreb, Croatia.
J Clin Med. 2024 Sep 25;13(19):5688. doi: 10.3390/jcm13195688.
ANCA-associated vasculitides (AAVs) are rare diseases with a prevalence of less than 200 cases per million persons and an incidence of less than 25 cases per million person-years. Their presenting features can vary from prodromal and nonspecific symptoms to dramatic organ-specific symptoms such as respiratory failure due to diffuse alveolar hemorrhage (DAH) and acute kidney injury (AKI). The latter two are hallmark features of pulmonary-renal syndrome, a potentially fatal condition that necessitates early recognition and treatment in intensive care units (ICUs) and rapid induction of immunosuppressive therapy. : We described three patients with newly diagnosed AAV during the treatment of critical illness. All patients had DAH and two had AKI. The initial disease severity was extremely high in patients with myeloperoxidase (MPO)-AAV, reaching Sequential Organ Failure Assessment (SOFA) scores of 15 and 14 with predicted mortality ≥ 95.2%. Both patients needed mechanical ventilation, one additional venovenous extracorporeal membrane oxygenation (VV-ECMO), and renal replacement therapy. The patient with proteinase 3 (PR3)-AAV had a less severe disease, SOFA 3, requiring only modest oxygen supplementation and exhibiting only hematuria with normal renal function parameters. Immunosuppressive therapy was initiated during the ICU stay. The patient with the most severe clinical presentation died during the ICU stay because of sepsis, and the other two patients were discharged home. : Patients with AAV presenting with pulmonary-renal syndrome necessitate various degrees of organ support. Nevertheless, these patients can be successfully treated in the early, critical stages of the disease and achieve remission.
抗中性粒细胞胞浆抗体相关性血管炎（AAV）是罕见疾病，患病率低于百万分之200例，发病率低于百万分之25例/人年。其临床表现多样，从前驱性和非特异性症状到严重的器官特异性症状，如因弥漫性肺泡出血（DAH）导致的呼吸衰竭和急性肾损伤（AKI）。后两者是肺肾综合征的标志性特征，这是一种潜在致命性疾病，需要在重症监护病房（ICU）早期识别和治疗，并迅速启动免疫抑制治疗。我们描述了3例在危重症治疗期间新诊断为AAV的患者。所有患者均有DAH，2例有AKI。髓过氧化物酶（MPO）-AAV患者初始疾病严重程度极高，序贯器官衰竭评估（SOFA）评分分别为15分和14分，预计死亡率≥95.2%。这2例患者均需要机械通气，其中1例还需要静脉-静脉体外膜肺氧合（VV-ECMO）和肾脏替代治疗。蛋白酶3（PR3）-AAV患者疾病严重程度较低，SOFA评分为3分，仅需适度吸氧，肾功能参数正常，仅表现为血尿。免疫抑制治疗在ICU住院期间启动。临床表现最严重的患者在ICU住院期间因脓毒症死亡，另外2例患者出院回家。出现肺肾综合征的AAV患者需要不同程度的器官支持。然而，这些患者在疾病的早期关键阶段可以得到成功治疗并实现缓解。
