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6 results

  • IgG4-Related Disease of the Central Nervous System: A Case Series

  • Ghada Abbas, Jason Karamchandani, Anthony Ciarallo, Liam Durcan
  • Journal:
    Canadian Journal of Neurological Sciences / Volume 50 / Issue 6 / November 2023
    Published online by Cambridge University Press:
    11 November 2022, pp. 907-913
    • Article
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  • IgG4-related disease (IgG4-RD) is a rare and often misdiagnosed disorder with limited literature that highlights the different neurological presentations of this treatable disease. The diagnosis of IgG4-RD could be challenging, while imaging is fundamental for the diagnosis, biopsy is considered the gold standard. Most cases respond well to steroids and immunosuppressive therapy. This is a case series study that illustrates the varied neurological presentations of IgG4-RD through three different patients that were followed at the Montreal Neurological Institute. This paper takes you through the diagnostic strategy that we followed to accurately diagnose and treat those patients.

  • Chapter 18 - CNS Syndromes at the Frontier of Autoimmune Encephalitis

  • from Section 3 - Specific Syndromes and Diseases
  • Josep Dalmau, Universitat de Barcelona, Francesc Graus, Universitat de Barcelona
  • Book:
    Autoimmune Encephalitis and Related Disorders of the Nervous System
    Published online:
    27 January 2022
    Print publication:
    17 February 2022, pp 476-502

  • Summary

    In this chapter we review several CNS disorders of probable autoimmune origin or of unclear aetiology that sometimes are considered in the differential diagnosis of autoimmune encephalitis. These syndromes include the CNS complications of systemic autoimmune disorders: IgG4-related disease, Behçet disease, systemic lupus erythematosus (SLE), and sarcoidosis. In each of them, neurological symptoms may precede the onset of systemic symptoms. Other ‘frontier disorders’ include several diseases associated with primary involvement of the vascular endothelium or blood vessels: cerebral amyloid angiopathy-related inflammation, Susac’s syndrome, and primary angiitis of the CNS, which can all present with isolated neurological manifestations. These syndromes are immune-mediated, do not present specific or pathogenic neuronal antibodies, and their diagnosis is based on well-established clinical criteria that sometimes include neuroimaging and histopathological features. The clinical presentation of these syndromes may mimic that of several autoimmune encephalitis: SLE can present with psychosis (thus, it may need the differential diagnosis with anti-NMDAR encephalitis); IgG4-related disease can present with meningoencephalitis; and Behçet disease can present with brainstem dysfunction and neuroimaging findings resembling those of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). The clinical presentation of primary angiitis of the CNS and Susac syndrome can be indistinguishable from that of autoimmune encephalitis.

  • Chapter 2 - Gastrointestinal Involvement by Systemic Disease

  • Edited by Roger M. Feakins
  • Book:
    Non-Neoplastic Pathology of the Gastrointestinal Tract
    Published online:
    06 June 2020
    Print publication:
    09 July 2020, pp 13-29

  • Summary

    Many diseases that have a systemic distribution may involve the gastrointestinal (GI) tract and liver. Furthermore, diseases that usually manifest within one extra-gastrointestinal organ or system may also involve the GI tract, either directly or as a result of treatment for the extra-GI disease. This chapter focuses initially on five systemic diseases that can have GI manifestations: sarcoidosis, amyloidosis, mast cell diseases, IgG4-related disease, and Behçet’s disease, and then discusses diseases with cutaneous manifestations and their effect(s) on the GI tract. Systemic diseases may become manifest within the GI tract in the context of a known condition. When this occurs, the cause of the GI disease may be obvious. Alternatively, the GI tract features may represent the presenting phase of the disease, which could already be active but subclinical at other sites. The differential diagnosis of certain histopathological features associated with GI tract manifestations of systemic disease, e.g. granulomas, may be wide. Therefore, careful clinicopathological correlation is essential. Finally, treatments for some extra-GI conditions may cause GI-related side effects, e.g. methotrexate therapy for psoriasis.

  • Immunoglobulin G4 related chronic sclerosing sialadenitis

  • E L Culver, A Hunt, E Crewe, K A Shah, P Martinez-Devesa
  • Journal:
    The Journal of Laryngology & Otology / Volume 129 / Issue 3 / March 2015
    Published online by Cambridge University Press:
    23 March 2015, pp. 226-231
    Print publication:
    March 2015
  • Background:

    ENT surgeons may be the first specialists to encounter and diagnose patients with salivary gland disease. A new entity involving the salivary glands has recently been described of which ENT surgeons need to be aware: immunoglobulin G4 related chronic sclerosing sialadenitis.

    Method:

    A literature search of Medline, Embase and Cochrane Library databases was performed, using the search terms ‘IgG4’, ‘hyperIgG4 syndrome’ and ‘IgG4 related chronic sclerosing sialadenitis’.

    Results:

    Knowledge concerning immunoglobulin G4 related chronic sclerosing sialadenitis is rapidly increasing. This new entity is part of a fibro-inflammatory corticosteroid-responsive systemic disease (immunoglobulin G4 related disease) and has been described in almost every organ. Biopsy of the submandibular gland can be diagnostic. However, the diagnosis can easily be overlooked if: clinical suspicion is not high, one is unaware of the classical morphology and/or immunoglobulin G4 staining is not performed. This paper presents a summary of the current understanding of the disease and its management.

    Conclusion:

    ENT surgeons should be aware of this new disease entity. Patients with systemic disease should be managed under a multidisciplinary team, with input from clinicians who have an interest in such diseases (such as gastroenterologists and rheumatologists), and input from histopathologists and radiologists.

  • Eosinophilic angiocentric fibrosis of the nose and sinuses

  • J Rimmer, P Andrews, V J Lund
  • Journal:
    The Journal of Laryngology & Otology / Volume 128 / Issue 12 / December 2014
    Published online by Cambridge University Press:
    17 November 2014, pp. 1071-1077
    Print publication:
    December 2014
  • Background:

    Eosinophilic angiocentric fibrosis is a rare benign disorder of the upper respiratory tract. It is slow growing and progressive, with characteristic histological appearances.

    Methods:

    We report the largest single-institution case series of sinonasal eosinophilic angiocentric fibrosis to date, comprising nine patients. The current literature is reviewed, showing emerging evidence that this condition may belong to the immunoglobulin G4-related disease spectrum.

    Results:

    The series comprised five female and four male patients, with a mean age at presentation of 53 years. All were treated surgically. Six patients had no signs of recurrent disease after an average of 8.5 years. One patient went on to develop granulomatosis with polyangiitis (Wegener's granulomatosis), which required immunosuppressive therapy.

    Conclusion:

    The first-line management of this rare condition is complete surgical excision. Chronic granulomatous conditions, including granulomatosis with polyangiitis, should be excluded before a diagnosis is made, and patients should be carefully followed.

  • Immunoglobulin G4 related disease isolated to the nasal cavity: a rare cause of nasal obstruction

  • C Morris, T Ng, P Kevin, N Singh
  • Journal:
    The Journal of Laryngology & Otology / Volume 129 / Issue S1 / January 2015
    Published online by Cambridge University Press:
    27 August 2014, pp. S57-S59
    Print publication:
    January 2015
  • Background:

    Immunoglobulin G4 related disease is a rare condition. Cases involving the sinonasal region are exceptionally uncommon. This paper describes a case of immunoglobulin G4 related disease isolated solely to the nasal cavity.

    Methods:

    Case report and literature review.

    Results:

    A 34-year-old man presented with painless, progressive bilateral nasal obstruction. Clinical examination and imaging findings demonstrated bilateral submucosal swelling of the anterior septum and right external nasal wall. Biopsy revealed immunoglobulin G4 related disease. The patient responded to oral corticosteroids initially, followed by long-term methotrexate.

    Conclusion:

    To the best of our knowledge, this case represents the first report in the literature of immunoglobulin G4 related disease isolated solely to the nasal cavity.