Intended for healthcare professionals

Clinical Review State of the Art Review

Advances in the diagnosis and management of IgG4 related disease

BMJ 2020; 369 doi: (Published 16 June 2020) Cite this as: BMJ 2020;369:m1067
  1. Marco Lanzillotta, consultant internal medical doctor1 2,
  2. Gaia Mancuso, consultant rheumatologist1 2,
  3. Emanuel Della-Torre, consultant clinical immunologist, assistant professor1 2
  1. 1Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy
  2. 2Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy
  1. Correspondence to: E Della-Torre dellatorre.emanuel{at}


IgG4 related disease was recognized as a unified disease entity only 15 years ago. Awareness of IgG4 related disease has increased worldwide since then, and specialists are now familiar with most of its clinical manifestations. Involvement of the pancreato-biliary tract, retroperitoneum/aorta, head and neck, and salivary glands are the most frequently observed disease phenotypes, differing in epidemiological features, serological findings, and prognostic outcomes. In view of this multifaceted presentation, IgG4 related disease represents a great mimicker of many neoplastic, inflammatory, and infectious conditions. Histopathology remains key to diagnosis because reliable biomarkers are lacking. Recently released classification criteria will be invaluable in improving early recognition of the disease. IgG4 related disease is highly treatable and responds promptly to glucocorticoids, but it can lead to end stage organ failure and even death if unrecognized. Prolonged courses of corticosteroids are often needed to maintain remission because the disease relapses in most patients. Rapid advancement in our understanding of the pathophysiology of IgG4 related disease is leading to the identification of novel therapeutic targets and possible personalized approaches to treatment.


  • Series explanation: State of the Art Reviews are commissioned on the basis of their relevance to academics and specialists in the US and internationally. For this reason they are written predominantly by US authors

  • Contributors: All authors did the literature search and wrote, revised, and edited the manuscript. EDT is the guarantor.

  • Funding: EDT received financial support from a Giovani Ricercatori 2018–Research Grant award from the Cariplo Foundation.

  • Competing interests: We have read and understood the BMJ policy on declaration of interests and declare the following interests: EDT received consulting fees from Xencor and Viela Bio.

  • Provenance and peer review: Commissioned; externally peer reviewed.

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