FDG-Avid Pulmonary Nodules and Tracheobronchial Mural Inflammation in IgG4-Related Disease

We report the case of a 9-year-old girl who presented with acute fevers, cough, and epistaxis with nasal swelling, and found to have a nasal mass. On 18F-FDG PET/CT, she was found to have marked nodal and pulmonary parenchymal disease with diffuse and symmetrical mural FDG uptake within the trachea and bronchial tree. The differential diagnosis was broad, and she was diagnosed with IgG4-RD (IgG4-related disease) and improved on appropriate treatment. To our knowledge, there are only 2 other pediatric cases of IgG4-related pulmonary disease in the literature, and our case serves as a unique constellation of intrapulmonary IgG4-RD imaging findings.