233. ALVEOLAR HEMORRHAGE IN ANCA-ASSOCIATED VASCULITIS: LONG TERM OUTCOME AND MORTALITY PREDICTORS. A RETROSPECTIVE LONG-TERM STUDY ON 106 ITALIAN PATIENTS Free

Background: Alveolar Hemorrhage (AH) is a severe and rare manifestation of AAV. It is not clear whether prognosis in these patients is determined by renal involvement (that is often associated with AH) or by AH itself. The aim of our study was to identify predictors of outcome in this particular subset of patients.

Methods: We performed a retrospective study on consecutive patients diagnosed with AAV-AH from 28 Italian Centers. Univariate and multivariate analysis was performed to examine the predictive role of baseline clinical characteristics and treatments in AAV-AH outcome. In case of collinearity, variables were used in multivariate analysis on the basis of both clinical relevance and stronger association.

Results: 106 patients were included; their characteristics are reported in Table 1. The majority were ANCA-positive (PR3 57.1%, MPO 33.7%) and 72.6% had renal involvement together with AE. Respiratory support was required in 46.1% of cases. Interventions are shown in Table 2. Median follow-up was 37 months [IQR 13-77]. At the end of follow-up, 19/106 patients (17.9%) were dead. The leading cause of death in the first 3 months after AE onset was active disease (4/7). Afterward, infections were the main cause of death. At Cox regression, age > 65 years at onset (p = 0.03), > 1 comorbidity (p = 0.01) > 2 cardiovascular comorbidities (p = 0.02) and infections (p = 0.004) were statistically associated with death, as was respiratory failure (p = 0.02) and the need for respiratory support (RS) (p = 0.005). Cyclophosphamide (Cyc) cumulative dose > 6 grams was found to be protective (the higher the dose, the lowest the risk, p = 0.03). By stepwise regression analysis, age > 65 years (p = 0.008) and the need in RS (p = 0.007) at DAH onset were confirmed to be predictive of mortality.

Conclusion: We have shown that the outcome of patients with AH is influenced by both factors that are an expression of intrinsic fragility (CV comorbidities, risk of infection and age at onset), and factors that point to a more severe involvement of the lung parenchyma by the capillaritis (the presence of respiratory failure and need for respiratory support at onset). As a consequence, treatment must be Individualized and keep into account, alongside AH severity, the patient’s baseline characteristics, fragility and risk of infection. This is true of both induction therapy and maintenance treatment, as justified by the fact that infection is the main cause of death in the follow-up period.

Disclosures: None