Evaluation of Pulmonary Involvement and Prognosis in Vasculitis Associated with Anti-Neutrophil Cytoplasma Antibodies
DOI:
https://doi.org/10.5281/zenodo.15834958Keywords:
Interstitial Lung Disease (ILD), Alveolar Hemorrhage (AH), Vasculitis Damage Index (VDI), Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitides (AAV), Anti-Neutrophil Cytoplasmic Antibody (ANCA)Abstract
Objective: No study has evaluated AAV's lung prognosis and the Vasculitis Damage Index (VDI). This study investigated lung involvement in AAV-associated disease activity, morbidity, and mortality rate.
Method: This retrospective analysis included 51 cases who were followed up in our institution's rheumatology outpatient clinic and diagnosed with AAV according to ARC and/or CHCC criteria. The patients were supposed to have lung (pulmonary) involvement at the beginning of the disease. Initial Birmingham Vasculitis Activity Scores (BVAS) and imaging findings were noted. Respiratory function test (PFT), 6-minute walk test (6MWT), thorax CT findings, and vasculitis damage index (VDI) scores were recorded.
Results: ANCA positivity was detected in 94% of patients with AAV (66% C-ANCA/anti-PR3, 34% p-ANCA/anti-MPO). The total follow-up period was recorded as 66.5±52 months. Initial total and BVAS were calculated as 22±7 and 4.6±2.8. BVAS findings were determined as 80% nodule/cavity, 56% infiltration, 24% AH/massive hemoptysis, 11% respiratory failure, 5% pleural effusion/pleurisy, and 3% endobronchial involvement. Radiological improvement was seen in 35%, regression in 12%, progression in 12%, and sequela changes in 30% of the patients. Cumulative VHI and lung VHI scores were calculated as 3.5±2.3 and 0.5±0.8. The frequency of VHI findings was 22% lung function disorder, 8% lung fibrosis, 6% chronic dyspnea, 4% chronic asthma, and 2% pulmonary hypertension. Serious pulmonary infections were seen in 44% (27% had >1 severe infection). VHI was higher in those with serious pulmonary infections (p=0.006).
Conclusion: Lung involvement with AAV causes high rates of lung damage and increased mortality in long-term follow-up.
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