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Small-vessel vasculitis with prominent IgG4 positive plasma cell infiltrates as potential part of the spectrum of IgG4-related disease: a case report

1, 2, 3, 4, 5, 6, 7, 8

  1. Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi Sunyer (IDIBAPS), Barcelona, Spain.
  2. Department of Internal Medicine, Hospital Clínic, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi Sunyer (IDIBAPS), Barcelona, Spain.
  3. Department of Internal Medicine, Hospital Clínic, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi Sunyer (IDIBAPS), Barcelona, Spain.
  4. Department of Anatomic Pathology, Hospital Clínic, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi Sunyer (IDIBAPS), Barcelona, Spain.
  5. Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi Sunyer (IDIBAPS), Barcelona, Spain.
  6. Department of Internal Medicine, Hospital Clínic, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi Sunyer (IDIBAPS), Barcelona, Spain.
  7. Department of Anatomic Pathology, Hospital Clínic, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi Sunyer (IDIBAPS), Barcelona, Spain.
  8. Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi Sunyer (IDIBAPS), Barcelona, Spain.

Vasculitis Research Unit, Departments of Systemic Autoimmune Diseases, Hospital Clínic, Barcelona, Spain.

CER7832 Submission on line
2015 Vol.33, N°89 ,Suppl.89 - PI 0138, PF 0141
Case Reports

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Rheumatology Article

 

Abstract

IgG4-related disease (IgG4-RD) is a systemic entity characterised by multiorgan inflammatory lesions with abundant IgG4+ plasma cells, obliterative phlebitis, and storiform fibrosis. Involvement of several organs such as the pancreas, gastrointestinal tract, salivary glands, periorbital tissue and lymph nodes has been described. Up to now, vascular involvement by IgG4-RD has been thought to be essentially confined to large vessels. We present a patient with small-vessel systemic vasculitis involving muscle, peripheral nerve and kidney (glomerulonephritis) in the context of IgG4-RD diagnosed on the basis of elevated serum IgG4+ concentrations and histologically consistent signs in all biopsied tissues. Thoracic and abdominal aortic aneurysms in addition to aortitis, suggestive of large-vessel involvement, were also present. This observation expands the spectrum of vascular involvement in the context of IgG4-RD and supports the inclusion of IgG4-RD in the category of vasculitis associated with systemic disorder.

PMID: 26016765 [PubMed]

Received: 08/08/2014 - Accepted : 03/10/2014 - In Press: 26/05/2015 - Published: 26/05/2015