Objective  To explore clinical features and risk factors of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with pulmonary involvement. Methods  A retrospectively study of clinical data of 113 AAV patients with pulmonary involvement was conducted in the First Affiliated Hospital of Soochow University from January 2015 to December 2020. The differences in general characteristics, treatment and prognosis of different types of AAV with pulmonary involvement were compared. In addition, the clinical characteristics and survival status between the pulmonary involvement group and the non-pulmonary involvement group (n=69) were analyzed. Multivariate logistic regression model was used to analyze the risk factors. Results  A total of 113 patients (57 males and 56 females) of AAV with pulmonary involvement were enrolled, including 86 cases of microscopic polyangiitis (MPA), 21 cases of granulomatosis polyangiitis (GPA), 6 cases of eosinophilia granulomatosis (EGPA). The average age was (67±11) years old. There was no significant difference in the age and gender distribution. The clinical manifestations were non-specific. Interstitial lung disease was common imaging feature of MPA, multiple nodules or mass was common in GPA, the incidence of sinusitis in EGPA was significantly higher (P<0.05). Seventy-three patients were complicated with extrapulmonary involvement. The most common organ involved was the kidney, followed by the cardiovascular and nervous system. The most important organs involved in MPA, EGPA and GPA were kidney, heart, and ear, nose, throat respectively. Compared with the non-pulmonary involvement group, the proportions of Birmingham vasculitis activity score (BVAS) ≥15 points, higher antineutrophil cytoplasmic antibody titer and lower complement C3 or C4, pulmonary infection, mechanical ventilation and plasmapheresis in the pulmonary involvement group were significantly higher (P<0.05). Forty patients died during the follow-up. One-year cumulative survival was further calculated using the Kaplan-Meier method, which demonstrated that pulmonary involvement was a risk factor for higher mortality in AAV patients. Compared with the survival group, the proportions of coronary heart disease, multiple organs involvement (n≥3), BVAS≥15 points, serum creatinine≥500 μmol/L, hemoglobin≤90 g/L, C-reactive protein≥10 mg/L, pulmonary infection, requiring mechanical ventilation, continuous renal replacement therapy and plasmapheresis in the death group were significantly higher (P<0.05). Conclusions  AAV with pulmonary involvement is more common in the elderly, the morbidity is similar between male and female, and the clinical manifestations are usually non-specific. The chest imaging manifestations are mainly pulmonary interstitial changes, multiple nodules and masses. Multiple organs involvement occurs more often. BVAS≥15 points is independent risk factor for pulmonary involvement in AAV patients. The prognosis of AAV patients with pulmonary involvement is relatively poor. Combined with coronary heart disease, pulmonary infection and CRP≥10 mg/L are independent risk factors of poor prognosis.

Citation： ZUO Tingting, CHEN Yanbin. Analysis of clinical features and risk factors of antineutrophil cytoplasmic antibody-associated vasculitis with pulmonary involvement. Chinese Journal of Respiratory and Critical Care Medicine, 2023, 22(2): 96-103. doi: 10.7507/1671-6205.202211043 Copy