Journal
CLINICAL MEDICINE
Volume 21, Issue 2, Pages E231-E233Publisher
ROY COLL PHYS LONDON EDITORIAL OFFICE
DOI: 10.7861/clinmed.2020-1037
Keywords
granulomatosis with polyangiitis; vasculitis; anti-neutrophil cytoplasmic antibodies; pachymeningitis; aortitis
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This case highlights the multisystem nature of granulomatosis with polyangiitis (GPA) with unusual dural and large vessel aortic and pulmonary trunk involvement.
We describe the case of a 61-year-old woman who presented with progressive respiratory symptoms and imaging demonstrating multiple opacities in the right lung with hilar and mediastinal lymphadenopathy suggestive of multifocal adenocarcinoma. Subsequent biopsies were consistent with focal changes of organising pneumonia (OP) and no evidence of malignancy. She was treated with steroids for cryptogenic OP with limited response. There was clinical and radiological progression with new lung nodules, mediastinal and thoracic spinal canal infiltration. There was ongoing concern that clinical findings represented disseminated malignancy. Following further investigation and multidisciplinary respiratory and rheumatology review, a diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) ? granulomatosis with polyangiitis (GPA) was confirmed. The case highlighted the multisystem nature of GPA with unusual dural and large vessel aortic and pulmonary trunk involvement.
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