@article {Mohammadjrheum.161224, author = {Aladdin J. Mohammad and Kristian H. Mortensen and Judith Babar and Rona Smith and Rachel B. Jones and Daiki Nakagomi and Pasupathy Sivasothy and David R.W. Jayne}, title = {Pulmonary Involvement in Antineutrophil Cytoplasmic Antibodies (ANCA)-associated Vasculitis: The Influence of ANCA Subtype}, elocation-id = {jrheum.161224}, year = {2017}, doi = {10.3899/jrheum.161224}, publisher = {The Journal of Rheumatology}, abstract = {Objective To describe pulmonary involvement at time of diagnosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), as defined by computed tomography (CT). Methods Patients with thoracic CT performed on or after the onset of AAV (n = 140; 75 women; granulomatosis with polyangiitis, n = 79; microscopic polyangiitis MPA, n = 61) followed at a tertiary referral center vasculitis clinic were studied. Radiological patterns of pulmonary involvement were evaluated from the CT studies using a predefined protocol, and compared to proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA specificity. Results Of the patients, 77\% had an abnormal thoracic CT study. The most common abnormality was nodular disease (24\%), of which the majority were peribronchial nodules, followed by bronchiectasis and pleural effusion (19\%, each), pulmonary hemorrhage and lymph node enlargement (14\%, each), emphysema (13\% ), and cavitating lesions (11\%). Central airways disease and a nodular pattern of pulmonary involvement were more common in PR3-ANCA{\textendash}positive patients (p \< 0.05). Usual interstitial pneumonitis (UIP) and bronchiectasis were more prevalent in MPO-ANCA{\textendash}positive patients (p \< 0.05). Alveolar hemorrhage, pleural effusion, lymph node enlargement, and pulmonary venous congestion were more frequent in MPO-ANCA{\textendash}positive patients. Conclusion Pulmonary involvement is frequent and among 140 patients with AAV who underwent a thoracic CT study, almost 80\% have pulmonary abnormalities on thoracic CT. Central airway disease occurs exclusively among patients with PR3-ANCA while UIP were mainly seen in those with MPO-ANCA. These findings may have important implications for the investigation, management, and pathogenesis of AAV.}, issn = {0315-162X}, URL = {https://www.jrheum.org/content/early/2017/07/26/jrheum.161224}, eprint = {https://www.jrheum.org/content/early/2017/07/26/jrheum.161224.full.pdf}, journal = {The Journal of Rheumatology} }