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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共 32 条
  • [1] Long-Term Infectious Complications of Kidney Transplantation
    Agrawal, Akansha
    Ison, Michael G.
    Danziger-Isakov, Lara
    [J]. CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2022, 17 (02): : 286 - 295
  • [2] Characteristics and prognosis of ANCA-positive retroperitoneal fibrosis: A systematic literature review
    Akiyama, Mitsuhiro
    Kaneko, Yuko
    Takeuchi, Tsutomu
    [J]. AUTOIMMUNITY REVIEWS, 2020, 19 (10)
  • [3] Diagnosis and management of ANCA associated vasculitis
    Berden, Annelies
    Goceroglu, Arda
    Jayne, David
    Luqmani, Raashid
    Rasmussen, Niels
    Bruijn, Jan Anthonie
    Bajema, Ingeborg
    [J]. BMJ-BRITISH MEDICAL JOURNAL, 2012, 344
  • [4] Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort
    Comarmond, Cloe
    Pagnoux, Christian
    Khellaf, Mehdi
    Cordier, Jean-Francois
    Hamidou, Mohamed
    Viallard, Jean-Francois
    Maurier, Francois
    Jouneau, Stephane
    Bienvenu, Boris
    Puechal, Xavier
    Aumaitre, Olivier
    Le Guenno, Guillaume
    Le Quellec, Alain
    Cevallos, Ramiro
    Fain, Olivier
    Godeau, Bertrand
    Seror, Raphaele
    Dunogue, Bertrand
    Mahr, Alfred
    Guilpain, Philippe
    Cohen, Pascal
    Aouba, Achille
    Mouthon, Luc
    Guillevin, Loic
    [J]. ARTHRITIS AND RHEUMATISM, 2013, 65 (01): : 270 - 281
  • [5] Mediastinal IgG4-Related Disease Manifesting as Superior Vena Cava Syndrome
    DeLuzio, Matthew R.
    Rassaei, Negar
    Taylor, Matthew D.
    Reed, Michael F.
    [J]. ANNALS OF THORACIC SURGERY, 2021, 112 (01) : E49 - E51
  • [6] Tumors of the mediastinum
    Duwe, BV
    Sterman, DH
    Musani, AI
    [J]. CHEST, 2005, 128 (04) : 2893 - 2909
  • [7] Wegener's granulomatosis of the orbit: A clinicopathological study of 15 patients
    Fechner, FP
    Faquin, WC
    Pilch, BZ
    [J]. LARYNGOSCOPE, 2002, 112 (11) : 1945 - 1950
  • [8] Antineutrophil cytoplasm antibody-associated vasculitis: recent developments
    Furuta, Shunsuke
    Jayne, David R. W.
    [J]. KIDNEY INTERNATIONAL, 2013, 84 (02) : 244 - 249
  • [9] Mediastinal mass and hilar adenopathy - Rare thoracic manifestations of Wegener's granulomatosis
    George, TM
    Cash, JM
    Farver, C
    Sneller, M
    vanDyke, CW
    Derus, CL
    Hoffman, GS
    [J]. ARTHRITIS AND RHEUMATISM, 1997, 40 (11): : 1992 - 1997
  • [10] Clinicopathologic Findings in Mass Forming ANCA-Associated Vasculitis
    Gilani, Sarwat I.
    Alexander, Mariam P.
    Nasr, Samih H.
    Fidler, Mary E.
    Takahashi, Naoki
    Cornell, Lynn D.
    [J]. KIDNEY INTERNATIONAL REPORTS, 2022, 7 (12): : 2709 - 2713