Relapsing ANCA-associated vasculitis presenting as a mediastinal mass

被引:0
作者
Pieri, Christopher [1 ]
Challoner, Benjamin [2 ]
Giaslakiotis, Konstantinos [2 ]
Hawthorne, Mark [2 ]
Sheaff, Michael [2 ]
Rajakariar, Ravindra [1 ]
机构
[1] Barts Hlth NHS Trust, Dept Nephrol, London, England
[2] Barts Hlth NHS Trust, Dept Cellular Pathol, London, England
关键词
Vasculitis; Chronic renal failure; Proteinurea; Pathology; ANTIBODY-ASSOCIATED VASCULITIS; WEGENERS-GRANULOMATOSIS; MANAGEMENT; DISEASE;
D O I
10.1136/bcr-2024-262481
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Anti-neutrophil cytoplasmic antibody-associated vasculitides (AAV) represent a heterogeneous multisystem group of disorders typified by necrotising inflammation of smaller blood vessels, classically yielding a pauci-immune, crescentic glomerulonephritis. Without prompt treatment, there is a significant risk of irreversible damage and ensuing renal impairment. Diagnosis is often challenging, exacerbated by the disorder's often vague and insidious presentation. Even with known AAV, relapse can mimic other similarly serious conditions including infection or malignancy, with significant ramifications on respective downstream management options. Here, we present the case of a young man with pre-existing myeloperoxidase-positive vasculitis, describing a subacute cluster of symptoms and subsequently found to have an anterior mediastinal mass. Urgent tissue sampling excluded other aetiologies and ultimately delineated this as a surprising complication of vasculitis, allowing for prompt immunosuppression escalation. This case highlights the diagnostic challenge these patients represent, and the utility timely access to multimodality imaging holds alongside a multidisciplinary approach to expedite correct management decisions.
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页数:5
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