Background Lung involvement is seen as frequent as 80% in AAV.Vasculitis damage index (VDI) is a validated comprehensive damage tool which has been developed as an outcome measure and consists of different types of lung damage items
Objectives To investigate lung damage in AAV by using VDI
Methods Fifty-one patients (25 female) with AAV (40 GPA, 8 MPA, 3 e-GPA) and lung involvement met ACR criteria and followed-up from a single tertiary center, between 1998-2014, were included into the study. Demographic and clinical features, smoking and exposure history, severe infections (SI) requiring hospitalization, relapse rates and glucocorticoid (GC) and cyclophosphamide (CYC) doses were recorded. Initial BVAS scores and imaging findings were noted. Pulmonary function tests, 6MWT, HRCT and VDI scores were obtained. Correlation analysis was performed for BVAS and VDI scores. VDI scores were compared between the groups stratified in accordance to the presence and recurrence of severe pulmonary infections by Mann-Whitney U test
Results Lung involvement was investigated by imaging (X-Ray/HRCT) (100%), histopathology (15%) or bronchoscopy (25%). ANCA positivity was found in 94% (66% C-ANCA/anti-PR3, 34% p-ANCA/anti-MPO). Mean age at diagnosis, time between the first symptom and diagnosis and total follow-up time were 49±13 y (med 51), 4,8±5,8 mo (med 3) and 66,5±52 mo (med 47), respectively. 28 patients were smokers. Kidney was the most frequently involved organ (78%) with lung. Initial total and lung BVAS were 22±7 (4-38) (med 23) and 4,6±2,8, respectively. The frequency of BVAS items were as follows: Nodules/cavities 80%, infiltration 52%, alveolar haemorrhage 19%, respiratory failure 11%, pleural effusion 5%, endobronchial involvement 3%. Respiratory failure developed in 11% at presentation. Cumulative total and lung VDI scores were 3,4±2,2 (0-9) (med 3), 0.4±0.8 (0-4) (med 1) respectively. The frequency of VDI lung items were 21% for impaired lung function, 7% for pulmonary fibrosis, 5% for chronic breathlesness, 1% for pulmonary hypertension.AAV patients had 1 (24%), 2 (12%) or 4 (1%) items of VDI. VDI scores of lung items in AAV subgroups were shown in Table 1. One of four patients who suffered from respiratory failure died. Severe pulmonary infections (>1 SI in 33%) were detected in 44%. VDI was higher in patients who suffered from pulmonary infection but, statistically insignificant (4.1±0.4 vs 3±1.9, p>0.05). The patients with recurrent severe pulmonary infections had significantly high VDI scores when compared to the patients with only one infection(4.8±2.7 vs 3±1.9, p=0.02). No significant correlation was found between BVAS and VDI scores.
Conclusions VDI is a useful tool to demonstrate lung damage in AAV.Lung damage has been demonstrated in almost 40% of AAV patients. Recurrent pulmonary infections seem to be an important contributor to the lung damage in AAV.
Disclosure of Interest None declared