Lessons of the month: ANCA-associated vasculitis – granulomatosis with polyangiitis: ‘the great mimic’

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...

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Bibliographic Details
Published in:Clinical medicine (London, England) England), 2021-03, Vol.21 (2), p.e231-e233
Main Authors: Hesford, James, Medford, Andrew RL, Gunawardena, Harsha
Format: Article
Language:English
Subjects:
Aneurysms
anti-neutrophil cytoplasmic antibodies
aortitis
Back pain
Biopsy
Blood tests
Cancer
Disease
Dyspnea
granulomatosis with polyangiitis
Inflammation
Inflammatory diseases
Lessons of the Month
Literature reviews
Lung cancer
Lungs
Neutrophils
Otolaryngology
pachymeningitis
Patients
Pneumonia
Remission (Medicine)
Rheumatology
Spinal cord
Steroids
vasculitis
Vein & artery diseases
Veins & arteries
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Description
Summary: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 anti-neutrophil 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.
ISSN:1470-2118
1473-4893
DOI:10.7861/clinmed.2020-1037