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IgG4-related disease: a complex under-diagnosed clinical entity

Rheumatology International volume 38, pages 169–177 (2018)Cite this article

Abstract

IgG4-related disease (IgG4-RD) encompasses a spectrum of complex fibro-inflammatory disorders which are often under diagnosed due to unfamiliarity by clinicians. A challenging multitude of clinical manifestations makes the diagnosis cumbersome. The primary clinical feature in IgG4-RD entails a tumor-like presentation coupled with tissue-destructive lesions. Histopathological findings include lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. These findings, in combination with elevated serum immunoglobulin G4 levels, are diagnostic in the setting of single- or multi-organ involvement. A closer understanding of the role of T cells and B cells in the increased production of IgG4 has led to a notion that IgG4 can act as a pathogen, anti-inflammatory agent, or rheumatoid factor. Glucocorticoids are the primary treatment modality; however, relapse is common with prolonged therapy. Alternatively, immunomodulatory agents are being increasingly used as therapy. The aim of this article is to raise awareness of IgG4-RD and review the diagnostic algorithm, as IgG4-RD often mimics a wide array of clinical conditions. In addition, we summarize the pathogenesis and current treatment guidelines of IgG4-RD for clinicians. Awareness and accurate diagnosis are crucial in preventing progression to chronic diseases, thereby diminishing disease-related morbidity and mortality.

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Author information

Affiliations

  1. Department of Internal Medicine, Albert Einstein Medical Center, Philadelphia, PA, USA

    Sujani Yadlapati

  2. Department of Internal Medicine, St. Luke’s-Roosevelt Hospital Center, New York, NY, USA

    Elijah Verheyen

  3. Division of Rheumatology, New York-Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY, USA

    Petros Efthimiou

  4. Weill Cornell Medical College, New York, NY, USA

    Petros Efthimiou

Authors
  1. Sujani Yadlapati
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  2. Elijah Verheyen
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  3. Petros Efthimiou
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Correspondence to Petros Efthimiou.

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Yadlapati, S., Verheyen, E. & Efthimiou, P. IgG4-related disease: a complex under-diagnosed clinical entity. Rheumatol Int 38, 169–177 (2018). https://doi.org/10.1007/s00296-017-3765-7

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  • DOI: https://doi.org/10.1007/s00296-017-3765-7

Keywords

  • IgG4
  • Fibro-inflammatory disease
  • AIP
  • IgG4-RD
  • Review