PURPOSE: We aim to identify risk factors predicting morbidity & mortality in patients hospitalized with acute respiratory illness and Wegener's granulomatosis (WG).
METHODS: A retrospective review of patients presenting with acute respiratory illness and WG (biopsy or serology proven) for the last 10 years. The following data were collected: demographics, imaging, laboratory data (e.g., ANCA-PR3, biopsy results), interventions (e.g., intubations) and treatments, initial and 28-day ICU organ failure scores (APACHE IV & SOFA), hospital length-of-stay (LOS)/outcome. Students paired two-tailed t-tests and Pearson coefficient correlation were used for statistical analysis.
RESULTS: 20 patients (11 females, 9 males, mean age 53.1 years) were identified. Common signs/symptoms were: sepsis (70%), dyspnea (60%), severe hemoptysis (50%), fever (30%), and dry cough (25%). There were 6 new and 14 established cases (9 taking prednisone, 5 taking cytoxan). 75% had bilateral infiltrates/pleural effusion on imaging. 12 were admitted to the ICU, all (100%) had hypoxic respiratory failure. Of the ICU cases following were seen: intubation's (58%), sepsis (50%), septic shock (17%). The most common infections identified E.coli/Candida (emia) (25%), ARF (42%), HD (25%), pressors (25%), IV steroids (92%), cytoxan (25%). All received empiric antibiotics (100%). Mean hospital LOS was 16.5 days for the entire study cohort and 22.3 days among ICU cases; Similarly the mortality rate was 15% for the entire cohort and 25% for the ICU cases. 3 deaths were recorded. All were intubated, had positive cultures developed ARF, had HD and died from sepsis and renal/respiratory failure. Among living patients, respectively SOFA (initial = 5, follow-up = 2.6, p<0.001) and APACHE IV (initial = 58.5, follow-up = 43.4, p<0.001) scores significantly improved by 28 days.
CONCLUSION: A majority of patients admitted to the ICU with WG were treated empirically with broad-spectrum antibiotics and IV steroids, as a significant percentage of them had sepsis and septic shock. Development of ARF requiring HD, need for mechanical ventilation and need for pressors bode poor outcome.
CLINICAL IMPLICATIONS: The prognosis for WG patients with pulmonary manifestations in the ICU is guarded, but majority can survive with aggressive resuscitation and prompt treatment (steroids, empiric antibiotics and/or HD).
DISCLOSURE: Amir Khan, None.