Zeyad Ahmed Metwalli MD, Presenter: Nothing to Disclose

R. Paul Guillerman MD, Abstract Co-Author: Nothing to Disclose

Petek Bayindir MD, Abstract Co-Author: Nothing to Disclose

Claire Langston, Abstract Co-Author: Nothing to Disclose

Pulmonary capillaritis is an immune-mediated small-vessel vasculitis associated with diffuse alveolar hemorrhage. The chest imaging manifestations of pulmonary capillaritis in children have not previously characterized. To facilitate early diagnosis and institution of appropriate therapy, we present the salient chest imaging findings of the largest known series of pediatric pulmonary capillaritis.

All patients with histologically-confirmed pulmonary capillaritis over an eleven year period (1999-2010) at a tertiary children’s hospital were identified by search of a pathology database. A retrospective review of the clinical charts and chest imaging studies was conducted.

Pulmonary capillaritis was diagnosed in 14 pediatric patients (age of presentation 11 months - 18 years). A majority presented with respiratory symptoms or signs such as cough, dyspnea, and hypoxia, but only 4 (29%) had hemoptysis. All 10 patients with available hemoglobin levels were markedly anemic and 7 patients exhibited elevated ANCA levels. The predominant radiographic pattern seen in 11 (92%) of the 12 patients with chest CT scans available for review was bilateral patchy or geographic ground glass pulmonary opacities and ill-defined centrilobular pulmonary nodules, reflecting angiocentric inflammation and hemorrhage. Other findings included consolidation in 6 patients (50%), mediastinal and hilar lymphadenopathy in 6 patients (50%), crazy paving in 2 patients (17%), tiny parenchymal or juxtafissural cysts in 2 patients (17%) and septal thickening in 2 patients (17%). All patients were treated with intravenous corticosteroids resulting in clinical remission, corroborated by improvement in the CT findings in all patients with follow-up imaging.

Pulmonary capillaritis typically manifests on chest CT as bilateral ground glass pulmonary opacities and ill-defined pulmonary nodules. Consolidation and lymphadenopathy are also common. Anemia and elevated ANCA levels are frequently associated, while hemoptysis is less common. Recognition of these findings in the appropriate clinical setting expedites performance of confirmatory lung biopsy and institution of corticosteroid therapy.

Pulmonary capillaritis is a small vessel vasculitis that typically presents with anemia and CT findings of bilateral ground glass pulmonary opacities and ill-defined pulmonary nodules.

Metwalli, Z, Guillerman, R, Bayindir, P, Langston, C, Imaging Features of Pediatric Pulmonary Capillaritis.  Radiological Society of North America 2011 Scientific Assembly and Annual Meeting, November 26 - December 2, 2011 ,Chicago IL. http://archive.rsna.org/2011/11003058.html