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Digestive Disorders, Rare Autoimmune Diseases CE/CME accredited

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Addressing challenges in diagnosis and treatment of IgG4-related gastrointestinal disease

Learning Objectives

After watching this activity, participants should be better able to:

  • Appraise the challenges associated with diagnosing IgG4-related gastrointestinal disease
  • Differentiate type 1 autoimmune pancreatitis from type 2
  • Summarize treatments for IgG4-related gastrointestinal disease

In this activity, gastroenterology and hepatology specialists Prof. Matthias Löhr and Dr Shounak Majumder respond to questions from the clinical community on the diagnosis and management of the gastrointestinal and pancreaticobiliary manifestations of immunoglobulin G4-related disease (IgG4-RD).

This activity is jointly provided by USF Health and touchIME. read more

Target Audience

This activity has been designed to meet the educational needs of gastroenterologists, rheumatologists, hepatologists/pancreatologists and pathologists involved in the management of IgG4-related gastrointestinal disease.


USF Health adheres to the Standards for Integrity and Independence in Accredited Continuing Education. All individuals in a position to influence content have disclosed to USF Health any financial relationship with an ineligible organization. USF Health has reviewed and mitigated all relevant financial relationships related to the content of the activity. The relevant relationships are listed below. All individuals not listed have no relevant financial relationships.


Prof. Matthias Löhr discloses Consultancy fees from Molecular Health. Speaker’s bureau fees from Abbott, FrostPharma and Viatris.

Dr Shounak Majumder has no financial interests/relationships or affiliations in relation to this activity.

Content reviewer

Loutfi Succari, MD has no financial interests/relationships or affiliations in relation to this activity.

Touch Medical Contributors

Katrina Lester has no financial interests/relationships or affiliations in relation to this activity.

USF Health Office of Continuing Professional Development and touchIME staff have no financial interests/relationships or affiliations in relation to this activity.

Requirements for Successful Completion

In order to receive credit for this activity, participants must review the content and complete the post-test and evaluation form. Statements of credit are awarded upon successful completion of the post-test and evaluation form.If you have questions regarding credit please contact



This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through a joint providership of USF Health and touchIME. USF Health is accredited by the ACCME to provide continuing medical education for physicians.

USF Health designates this enduring material for a maximum of 0.75 AMA PRA Category 1 CreditTM.  Physicians should claim only the credit commensurate with the extent of their participation in the activity.

The European Union of Medical Specialists (UEMS) – European Accreditation Council for Continuing Medical Education (EACCME) has an agreement of mutual recognition of continuing medical education (CME) credit with the American Medical Association (AMA). European physicians interested in converting AMA PRA Category 1 CreditTM into European CME credit (ECMEC) should contact the UEMS (

Advanced Practice Providers

Physician Assistants may claim a maximum of 0.75 Category 1 credits for completing this activity. NCCPA accepts AMA PRA Category 1 CreditTM from organizations accredited by ACCME or a recognized state medical society.

The AANPCP accepts certificates of participation for educational activities approved for AMA PRA Category 1 CreditTM by ACCME-accredited providers. APRNs who participate will receive a certificate of completion commensurate with the extent of their participation.

Date of original release: 31 January 2024. Date credits expire: 31 January 2025. If you have any questions regarding credit please contact

This activity is CE/CME accredited

To obtain the CE/CME credit(s) from this activity, please complete this post-activity test.

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Addressing challenges in diagnosis and treatment of IgG4-related gastrointestinal disease
0.75 CE/CME credit

Question 1/5
When discussing with colleagues, how would you describe a common diagnostic challenge associated with IgG4-related GI disease?

GI, gastrointestinal; Ig, immunoglobulin.

The 2019 ACR/EULAR IgG4-RD classification criteria and the 2020 UEG and SGF evidence-based recommendations on IgG4-related digestive disease note a lack of reliable biomarkers as a key diagnostic challenge.1,2 While serum IgG4 levels have diagnostic value, IgG4 levels alone lack sensitivity and specificity. Therefore, a comprehensive workup, which includes histology, radiology, serology and response to glucocorticoids, is recommended to diagnose IgG4-related GI disease.1,2


ACR, American College of Rheumatology; EULAR, European League Against Rheumatism; GI, gastrointestinal; Ig, immunoglobulin; IgG4-RD, IgG4-related disease; SGF, Swedish Society of Gastroenterology; UEG, United European Gastroenterology.


  1. Wallace ZS, et al. Arthritis Rheumatol. 2020;72:7–19.
  2. Löhr J-M, et al. United European Gastroenterol J. 2020;8:637–66.
Question 2/5
Your 63-year-old male patient presents with worsening right upper quadrant abdominal pain, jaundice and weight loss. Blood results indicate elevated serum CA 19-9 levels, which may indicate AIP-1 or pancreatic adenocarcinoma. A CT scan showed a diffuse 'sausage-like' parenchymal enlargement with loss of normal multilobulated pattern. Which of the following would you consider next to best diagnose this patient?

AIP-1, autoimmune pancreatitis type 1; CA, carbohydrate antigen; CT, computed tomography; Ig, immunoglobulin; PET, positron emission tomography.

The Mayo Clinic HISORt criteria and the ICDC are the two most familiar diagnostic guidelines for IgG4-related GI disease,1 and include histological, serological and radiological diagnostic parameters.2,3

Elevated serum IgG4 levels that are more than twice the upper limit of normal is strongly suggestive of AIP-1 when patients present with a pancreatic mass or enlargement.3 Neither CA 19-9 nor IgG4 levels alone are accurate enough to confirm a diagnosis of AIP-1,1,4 but a combination of CA 19-9 <74 U/mL and IgG4 >1.0 g/L can help to distinguish AIP-1 from pancreatic carcinoma.1,5 The UEG and SGF guidelines recommend a biopsy and immunohistochemical staining for IgG4 for the histological diagnosis of AIP-1.4


AIP-1, autoimmune pancreatitis type 1; CA, carbohydrate antigen; HISORt, histology, imaging, serology, other organ involvement and response to therapy; ICDC, International Consensus Diagnostic Criteria; Ig, immunoglobulin; SGF, Swedish Society of Gastroenterology; UEG, United European Gastroenterology.


  1. On W, Huggett MT. Frontline Gastroenterol. 2022;13:171–4.
  2. Chari ST, et al. Clin Gastroenterol Hepatol. 2006;4:1010–6.
  3. Shimosegawa T, et al. Pancreas. 2011;40:352–8.
  4. Löhr J-M, et al. United European Gastroenterol J. 2020;8:637–66.
  5. Farrukh L, et al. Ann Rheum Diseas. 2018. Abstract AB1571.
Question 3/5
Which of the following statements best summarizes the overlap between AIP-1 and AIP-2?

AIP-1/2, autoimmune pancreatitis type 1/2; Ig, immunoglobulin.

AIP is divided into two subtypes: AIP-1 and AIP-2.1 AIP-1 is also termed IgG4-related pancreatitis.1,2 AIP-2, also known as idiopathic duct-centric pancreatitis, is not associated with IgG4-RD.1 The most typical feature of AIP is a global enlargement of the pancreatic gland, giving it a ‘sausage-like’ shape, with diffuse involvement more frequent in AIP-1 and focal involvement in AIP-2.3,4


AIP-1/2, autoimmune pancreatitis type 1/2; Ig, immunoglobulin; IgG4-RD, IgG4-related disease.


  1. Blaho M, et al. Adv Med Sci. 2020;65:403–8.
  2. Löhr J-M, et al. Nat Rev Gastroenterol Hepatol. 2022;19:185–97.
  3. Löhr J-M, et al. United European Gastroenterol J. 2020;8:637–66.
  4. Mack S, et al. World J Gastroenterol. 2022;28:6867–74.
Question 4/5
Your 69-year-old male patient was diagnosed with AIP-1 and started treatment with 40 mg/day prednisolone. At the 2-week consultation after treatment initiation, the patient showed a rapid clinical response according to the IgG4 Responder Index. Which of the following would you consider next in the management of this patient?

AIP-1; autoimmune pancreatitis type 1; Ig, immunoglobulin.

Glucocorticoid treatment (typically 40 mg/day prednisolone) is the mainstay of induction therapy to induce remission in IgG4-RD.1,2 According to European and US consensus recommendations, radiological or biochemical evaluation of treatment response should occur 2–4 weeks after initiating therapy.3 Dose tapering after 1 month should be undertaken if there is subjective and objective improvement; dose tapering is recommended at a rate of 5 mg every 1–2 weeks.1,2


IgG4-RD, immunoglobulin G4-related disease.


  1. On W, Huggett MT. Frontline Gastroenterol. 2022;13:171–4.
  2. Perugino CA, Stone J. Z Rheumatol. 2016;75:681–6.
  3. Löhr J-M, et al. Nat Rev Gastroenterol Hepatol. 2022;19:185–97.
Question 5/5
Which of the following monoclonal antibodies are currently in phase III development for IgG4-RD?

CD, cluster of differentiation; IgG4-RD, immunoglobulin G4-related disease.

Inebilizumab and obexelimab are anti-CD19 monoclonal antibodies that target CD19-positive B cells and mediate their rapid and durable depletion.1,2 Both treatments are in phase III development for IgG4-RD (NCT04540497 and NCT05662241, respectively).3,4 In addition to targeting CD19, obexelimab also targets the FcγRIIb on B cells.2


CD, cluster of differentiation; FcγRIIb, Fcγ receptor II B; IgG4-RD, immunoglobulin G4-related disease.


  1. Lanzillotta M, et al. Mod Rheumatol. 2023;33:258–65.
  2. Nakaymada S, Tanaka Y. Mod Rheumatol. 2023;33:266–70.
  3. NCT04540497. Available at: (accessed January 2024).
  4. NCT05662241. Available at: (accessed January 2024).
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