PT  - JOURNAL ARTICLE
AU  - Aladdin J. Mohammad
AU  - Kristian H. Mortensen
AU  - Judith Babar
AU  - Rona Smith
AU  - Rachel B. Jones
AU  - Daiki Nakagomi
AU  - Pasupathy Sivasothy
AU  - David R.W. Jayne
TI  - Pulmonary Involvement in Antineutrophil Cytoplasmic Antibodies (ANCA)-associated Vasculitis: The Influence of ANCA Subtype
AID  - 10.3899/jrheum.161224
DP  - 2017 Oct 01
TA  - The Journal of Rheumatology
PG  - 1458--1467
VI  - 44
IP  - 10
4099  - http://www.jrheum.org/content/44/10/1458.short
4100  - http://www.jrheum.org/content/44/10/1458.full
SO  - J Rheumatol2017 Oct 01; 44
AB  - 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–positive patients (p < 0.05). Usual interstitial pneumonitis (UIP) and bronchiectasis were more prevalent in MPO-ANCA–positive patients (p < 0.05). Alveolar hemorrhage, pleural effusion, lymph node enlargement, and pulmonary venous congestion were more frequent in MPO-ANCA–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.