Objective The aim of this study was to clarify the clinical characteristics and long-term outcomes of ANCA-associated vasculitis (AAV) patients with pulmonary involvement from a single Chinese cohort. Methods Newly diagnosed AAV patients with pulmonary involvement, as defined by CT, were recruited from January 2010 to June 2020. Clinical data and CT images were collected retrospectively. Baseline CTs were evaluated and re-classified into four categories: interstitial lung disease (ILD), airway involvement (AI), alveolar hemorrhage (AH), and pulmonary granuloma (PG). Results A total of 719 patients were newly diagnosed with AAV, 366 (50.9%) of whom combined with pulmonary involvement at baseline. Among the AAV cases with pulmonary involvement, 55.7% (204/366) had ILD, 16.7% (61/366) had AI alone, 14.8% (54/366) had PG, and 12.8% (47/366) had AH alone. During follow-up of a median duration of 42.0 months, 66/366 (18.0%) patients died, mainly died from infections. Survival, relapse, and infection were all significantly different based on the radiological features. Specifically, the ILD group tends to have a poor long-term prognosis, the PG group is prone to relapse, and the AI group is apt to infection. The AH group has a high risk of both early infection and relapse, thus a poor short-term prognosis. Conclusion AAV patients with diverse radiological features have different clinical characteristics and outcomes. Therefore, the intensity of immunosuppressive therapy must be carefully valued by considering the baseline CT findings among AAV patients with pulmonary involvement.

Several retrospectivee described the association of interstitial lung disease (ILD) and ANCA-associated vasculitis (AAV). However, the relationship between the ILD and mortality in AAV patients have not been established so far. This study aims to estimate the relevance of AAV-associated-ILD (AAV-ILD) and mortality risk by conducting a systematic review and meta-analysis.A comprehensive systematic review was conducted in accordance with the guidelines of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-analyses). PubMed, Embase.com and the Cochrane Library (Wiley) were searched for original observational studies. Summary estimates were derived with a random-effects model and reported as risk ratio (RR), tested for publication bias and heterogeneity. Ten retrospective cohort studies were included, comprising 526 AAV-ILD patients enrolled from 1974 to 2018. Meta-analysis yielded a pooled RR of 2.90 (95% confidence interval 1.77–4.74) for death among those with AAV-ILD compared to control group. UIP pattern was associated with an even poorer prognosis in comparison to non-UIP pattern (RR 4.36, 95% confidence interval 1.14–16.78). Sensitivity analysis suggested that the meta-RR result was not skewed by a single dominant study. ILD might be associated with a higher mortality risk in AAV patients.

<b><i>Background:</i></b> Immune checkpoint inhibitors (ICIs) can lead to one of the common and quite serious immune-related adverse events (irAEs) in a real-world setting, namely, checkpoint inhibitor pneumonitis (CIP). <b><i>Objective:</i></b> We aimed to investigate the potential risk factors for CIP in cancer patients treated by ICIs and quantify the association. <b><i>Methods:</i></b> We conducted a systematic literature review in PubMed, EMBASE, and Web of Science from January 1, 2000, to March 20, 2022, with no study design restrictions. Studies evaluating the risk factors for CIP in cancer patients treated with ICIs-containing regimes were included. Odds ratios (ORs) with 95% confidence intervals (CIs) were calculated for risk factors of CIP by using random-effect model. Heterogeneity was assessed by sensitivity analysis and subgroup analysis regarding CIP severity, geographical regions, cancer types, treatment regimes, and ICI types. <b><i>Results:</i></b> A total of 35 studies comprising 142,703 patients were eventually included. The incidence of grade I–V and grade III–V CIP in cancer patients was 0.16 (95% CI: 0.14–0.18) and 0.06 (95% CI: 0.05–0.08), respectively. When combining the adjusted ORs, the following risk factors were significantly associated with the development of CIP: squamous cell carcinoma (OR: 1.31, 95% CI: 1.18–1.45), previous thoracic radiotherapy (OR: 2.07, 95% CI: 1.34–3.19), preexisting radiation-induced pneumonitis (OR: 3.62, 95% CI: 1.53–8.58), preexisting respiratory disease (OR: 2.43, 95% CI: 1.45–4.07), preexisting interstitial lung disease (OR: 5.78, 95% CI: 3.08–10.85), preexisting ground glass attenuation (OR: 11.48, 95% CI: 1.13–116.74), preexisting honeycombing (OR: 6.11, 95% CI: 2.37–15.79), preexisting pulmonary emphysema (OR: 2.72, 95% CI: 1.00–7.36), use of pembrolizumab (OR: 2.89, 95% CI: 1.56–5.35, <i>I</i>² = 0%), high PD-L1 expression (OR: 3.59, 95% CI: 1.23–10.50), and hypoalbuminemia (OR: 0.3, 95% CI: 0.14–0.64). When including the crude ORs, smoking history (OR: 1.39, 95% CI: 1.14–1.71), neutrophil-lymphocyte ratio (OR: 1.04, 95% CI: 1.01–1.08), and c-reactive protein (OR: 1.08, 95% CI: 1.01–1.16) were also risk factors for CIP. <b><i>Conclusion:</i></b> Histological characteristics, specific previous lung diseases and treatment history, treatment regimen, PD-L1 expression level, and serological biomarkers are all closely associated with the development of CIP. These findings would be useful for oncologists to optimize the appropriate options of ICIs and close monitoring during immunotherapy treatments, particularly for patients identified as having a higher risk for CIP.

<b><i>Background:</i></b> Interstitial lung disease (ILD) is a common pulmonary manifestation of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). <b><i>Objectives:</i></b> We aimed to clarify the clinical predictors of mortality in a cohort of patients with AAV-related ILD (AAV-ILD). <b><i>Method:</i></b> We retrospectively identified AAV-ILD patients seen at Peking University First Hospital from January 2010 to June 2020 and manually screened for study inclusion. Baseline computed tomography (CT) images were further classified as nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), and unclassified ILD. Disease characteristics and other pulmonary findings including pulmonary function test and bronchoalveolar lavage (BAL) were also evaluated. Multivariable Cox regression analysis was performed to identify clinical predictors of mortality. <b><i>Results:</i></b> The cohort included 204 patients with AAV-ILD, 152 had UIP on CT (AAV-UIP), 39 had NSIP on CT (AAV-NSIP), 3 had OP, and 10 had unclassified ILD. Microscopic polyangiitis was more prevalent in patients with UIP, while granulomatosis with polyangiitis was more common in the NSIP and OP groups, and eosinophilic granulomatosis with polyangiitis was more frequent in patients with unclassified ILD. ILD diagnosis before AAV was more common in patients with either UIP or NSIP patterns. During the median follow-up of 40 months, 44 (21.6%) patients died. One- and 5-year overall survival rates were 88.2% (95% CI, 83.7–92.7%) and 81.0% (95% CI, 74.9–87.1%) for the entire cohort. Patients with UIP patterns had the worst prognosis, while those with NSIP patterns had the best long-term outcome. Specifically, patients with UIP patterns had an approximately 5-fold risk of death compared to those with NSIP. After controlling for potential confounding factors, we observed that each 10% increase in the BAL fluid neutrophil percentage was associated with nearly a 20% increased risk of death (HR 1.195, 95% CI 1.018–1.404). <b><i>Conclusions:</i></b> Clinical characteristics and survival differ between subgroups defined by CT patterns. BAL fluid neutrophilia is an independent predictor of mortality among AAV-ILD patients, and therefore, the clinical utility of BAL at the time of AAV diagnosis should be considered.

Objective: The aim of this study was to clarify the clinical characteristics and long-term outcomes of ANCA-associated vasculitis (AAV) patients with pulmonary involvement from a single Chinese cohort. Methods: Newly diagnosed AAV patients with pulmonary involvement, as defined by CT, were recruited from January 2010 to June 2020. Clinical data and CT images were collected retrospectively. Baseline CTs were evaluated and re-classified into four categories: interstitial lung disease (ILD), airway involvement (AI), alveolar hemorrhage (AH), and pulmonary granuloma (PG). Results: A total of 719 patients were newly diagnosed with AAV, 366 (50.9%) of whom combined with pulmonary involvement at baseline. Among the AAV cases with pulmonary involvement, 55.7% (204/366) had ILD, 16.7% (61/366) had AI alone, 14.8% (54/366) had PG, and 12.8% (47/366) had AH alone. During follow-up of a median duration of 42.0 months, 66/366 (18.0%) patients died, mainly died from infections. Survival, relapse, and infection were all significantly different based on the radiological features. Specifically, the ILD group tends to have a poor long-term prognosis, the PG group is prone to relapse, and the AI group is apt to infection. The AH group has a high risk of both early infection and relapse, thus a poor short-term prognosis.Conclusion: AAV patients with diverse radiological features have different clinical characteristics and outcomes. Therefore, the intensity of immunosuppressive therapy must be carefully valued by considering the baseline CT findings among AAV patients with pulmonary involvement.