Purpose or Case Report: The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of autoimmune diseases characterized by vascular inflammation requiring immunosuppressive treatment. Involvement is most common of the kidneys and lungs, although there is a paucity of literature reporting the chest imaging findings in children. Our goal is to describe the salient chest imaging findings of pediatric AAV to promote recognition by radiologists.
Methods & Materials: A retrospective review of the electronic medical records of all patients with the diagnosis of AAV from 2008-2019 at our institution was performed and details regarding clinical presentation, management and outcome were extracted. The pulmonary and extrapulmonary findings on all chest computed tomography (CT) scans were recorded by a board-certified pediatric radiologist.
Results: AAV was diagnosed in 49 pediatric patients (female/male: 38/11). Mean age at presentation was 11.85+/- 4.94 and mean age at diagnosis was 12.55 +/- 4.7 years. Thirty-six (73.5%) patients presented with thoracic manifestations including cough (n=25), pulmonary hemorrhage (n=23), infection (n=11), shortness of breath (n=12), chest pain (n=8) and respiratory failure (n=8). Sixty-eight CT scans (66 helical/volumetric, 2 axial noncontiguous) of 30 patients were available for review. The most prevalent pulmonary findings on the 68 CT scans were pulmonary nodules in 28 (41%) with the nodules being poorly-defined in 89%, consolidation in 26 (38%) and ground-glass opacities in 26 (38%). Other findings included pulmonary cysts in 9 (13%), septal thickening in 9 (13%), fibrosis in 3 (4%), crazy-paving in 1 (1%) and bronchiectasis in 1 (1%). On the 66 helical/volumetric CT scans, the most common extrapulmonary finding was hilar/mediastinal lymphadenopathy in 18 (27%), followed by pleural effusion in 7 (11%), pleural thickening in 7 (11%), and pericardial effusion in 5 (8%). All patients were treated with intravenous corticosteroids and other immunosuppressants and immunomodulators resulting in clinical recovery, corroborated by improvement in the CT findings in all 16 patients with follow-up CT imaging.
Conclusions: Thoracic involvement is common in pediatric AAV. Typical manifestations on chest CT include poorly defined pulmonary nodules, pulmonary air space opacities and lymphadenopathy. Follow-up CT demonstrates improvement in the chest imaging findings in response to therapy.