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A heterogenous presentation, symptom variability, and a lack of established diagnostic criteria can complicate and delay diagnosis1,2

ACR/EULAR criteria for clinical trials are the only validated criteria for classifying IgG4-RD3

Organ

Select Criteria

Pancreas and biliary tract

  • Diffuse enlargement of more than 2/3 of the pancreas
  • Sclerosing cholangitis in the biliary tract
  • Smooth thickening of bile duct walls

Retroperitoneum

  • Fibrosis on the anterolateral sides of the aorta

Head to neck

  • Tomographical evidence in lacrimal and/or major salivary glands

Kidney

  • Hypocomplementemia (low levels of C3, C4, or both)

Chest

  • Peribronchovascular and septal thickening in the lungs

Exclusion criteria involve multiple clinical, serologic, radiologic, and pathologic findings, and identification of specific known diseases.3 Explore the full list of potential IgG4-RD manifestations here.

Patients with IgG4-RD will often suffer subclinical signs or symptoms for months or years before seeking help or diagnosis. Patients with IgG4-RD present with an average of 3 affected organs.4,5

While awareness is increasing, misdiagnosis of IgG4-RD remains common2

  • The characteristic fibrotic masses of IgG4-RD may be mistaken for cancerous tumors, and the disease can mimic other autoimmune conditions1,4

Lack of awareness and recognition can delay an IgG4-RD diagnosis for up to 2+ years1,6

igg4-related-disease-diagnostic-criteria-flowchart
EXPLORE MORE MANIFESTATIONS OF IgG4-RD

ACR, American College of Rheumatology; EULAR, European League Against Rheumatism; IgG4-RD, immunoglobulin G4–related disease.

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  • References:

    1. Wallace ZS, Zhang Y, Perugino CA, et al. Ann Rheum Dis. 2019;78(3):406-412. doi:10.1136/annrheumdis-2018-214603 2. Lanzillotta M, Mancuso G, Della-Torre E. BMJ. 2020;369:m1067. doi:10.1136/bmj.m1067 3. Wallace ZS, Naden RP, Chari S, et al. Ann Rheum Dis. 2019;79:77–87. doi:10.1136/annrheumdis-2019-216561 4. Goodchild G, Peters RJR, Cargill TN, et al. Clin Med. 2020;20(3):e32-e39. doi:10.7861/clinmed.2019-0457 5. Yamada K, Yamamoto M, Saeki T, et al. Arthritis Res Ther. 2017;19(1):262. doi:10.1186/s13075-017-1467-x 6. Alliance for Patient Access. 2021. https://admin.allianceforpatientaccess.org/wp-content/uploads/2021/12/AfPA_In-Their-Own-Voices_IgG4-RD-Report_Dec-2021.pdf