DIAGNOSTIC CRITERIA AND CHALLENGES

When IgG4-RD won’t respect boundaries, diagnosis poses a significant challenge^1,2

A step toward revealing the systemic nature of IgG4-RD is recognizing its potential individual manifestations.^1,2

A heterogenous presentation, symptom variability, and a lack of established diagnostic criteria can complicate and delay diagnosis^1,2

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 conditions^1,4

Lack of awareness and recognition can delay an IgG4-RD diagnosis for up to 2+ years^1,6

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EXPLORE MORE MANIFESTATIONS OF IgG4-RD

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

EXPLORE DIAGNOSTIC TESTING

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

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DIAGNOSTIC CRITERIA AND CHALLENGES

When IgG4-RD won’t respect boundaries, diagnosis poses a significant challenge1,2

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

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

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

When IgG4-RD won’t respect boundaries, diagnosis poses a significant challenge^1,2

A heterogenous presentation, symptom variability, and a lack of established diagnostic criteria can complicate and delay diagnosis^1,2

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

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

Lack of awareness and recognition can delay an IgG4-RD diagnosis for up to 2+ years^1,6