Symptoms & Flares

IgG4-RD respects no boundaries and can appear in unexpected organs with potentially serious manifestations^1,2

IgG4-RD can occur in nearly any organ system and most commonly affects tear ducts, salivary glands, and gastrointestinal and abdominal organs.^1,2

Some manifestations of IgG4-RD put patients at high risk for serious complications, including pancreatic enlargement, retroperitoneal fibrosis, and aortitis^1,2

Explore this interactive tool to see how IgG4-RD can appear throughout the body and have potentially serious manifestations.^1,3,4

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VIEW A FACT SHEET TO LEARN MORE ABOUT IgG4-RD

Illustration of human body affected by IgG4-related disease symptoms, identifying IgG4-RD manifestations in meninges, orbits, heart and aorta, biliary tract and liver, kidneys, retroperitoneum, pancreas, lungs, salivary and lacrimal glands, mediastinum, and thyroid and pituitary glands

Select a manifestation to learn more

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When a patient with IgG4-RD begins to experience symptoms, they are specific to the affected organ(s).^8,9

Organ(s)	Frequency	Systemic Manifestations	Potential Symptoms
Pachymeninges	Infrequent	Infundibulo-hypophysitis Hypertrophic pachymeningitis Inflammatory orbital pseudotumor Perineural infiltration	Cranial nerve palsies
Orbits	Occasional	Idiopathic orbital inflammatory pseudotumor	Proptosis, vision loss, diplopia
Lacrimal glands	Frequent	Mikulicz disease Chronic dacryoadenitis	Sicca
Skull bones, sinuses, ears	Occasional	Sinonasal masses	Chronic sinusitis, midline destructive lesions, anosmia, hearing loss, bone destruction
Salivary glands	Frequent	Mikulicz disease Chronic sialadenitis	Sicca
Heart, aorta	Infrequent	Aortic wall thickening	Coronary artery disease, inflammatory thoracic or abdominal aortic aneurisms
Thyroid	Infrequent	Hashimoto's thyroiditis Riedel's thyroiditis	Hypothyroidism
Mediastinum	Infrequent	Mediastinal adenopathy	Compression of local structures
Lungs	Occasional	Multiple lung nodules Ground-glass opacity Reticular interstitial thickening	Pulmonary fibrosis, interstitial lung disease, pleural effusion and thickening
Pancreas	Frequent	Autoimmune pancreatitis	Diabetes mellitus & malabsorption due to exocrine insufficiency
Bile ducts, gallbladder, liver	Occasional	Primary sclerosing cholangitis IgG4-related hepatopathy	Biliary stenting, infectious cholangitis, hepatic failure
Kidneys	Occasional	Renal parenchymal nodules, focal or diffuse	Renal failure due to interstitial nephritis or glomerulonephritis
Retroperitoneum	Frequent	Retroperitoneal fibrosis Periaortitis Sclerosing mesenteritis	Renal atrophy or injury due to hydronephrosis

Organ(s)

Pachymeninges

Frequency

Infrequent

Systemic Manifestations

Infundibulo-hypophysitis
Hypertrophic pachymeningitis
Inflammatory orbital pseudotumor
Perineural infiltration

Potential Symptoms

Cranial nerve palsies

Organ(s)

Orbits

Frequency

Occasional

Systemic Manifestations

Idiopathic orbital inflammatory pseudotumor

Potential Symptoms

Proptosis, vision loss, diplopia

Organ(s)

Lacrimal glands

Frequency

Frequent

Systemic Manifestations

Mikulicz disease
Chronic dacryoadenitis

Potential Symptoms

Sicca

Organ(s)

Skull bones, sinuses, ears

Frequency

Occasional

Systemic Manifestations

Sinonasal masses

Potential Symptoms

Chronic sinusitis, midline destructive lesions, anosmia, hearing loss, bone destruction

Organ(s)

Salivary glands

Frequency

Frequent

Systemic Manifestations

Mikulicz disease
Chronic sialadenitis

Potential Symptoms

Sicca

Organ(s)

Heart, aorta

Frequency

Infrequent

Systemic Manifestations

Aortic wall thickening

Potential Symptoms

Coronary artery disease, inflammatory thoracic or abdominal aortic aneurisms

Organ(s)

Thyroid

Frequency

Infrequent

Systemic Manifestations

Hashimoto's thyroiditis
Riedel's thyroiditis

Potential Symptoms

Hypothyroidism

Organ(s)

Mediastinum

Frequency

Infrequent

Systemic Manifestations

Mediastinal adenopathy

Potential Symptoms

Compression of local structures

Organ(s)

Lungs

Frequency

Occasional

Systemic Manifestations

Multiple lung nodules
Ground-glass opacity
Reticular interstitial thickening

Potential Symptoms

Pulmonary fibrosis, interstitial lung disease, pleural effusion and thickening

Organ(s)

Pancreas

Frequency

Frequent

Systemic Manifestations

Autoimmune pancreatitis

Potential Symptoms

Diabetes mellitus & malabsorption due to exocrine insufficiency

Organ(s)

Bile ducts, gallbladder, liver

Frequency

Occasional

Systemic Manifestations

Primary sclerosing cholangitis
IgG4-related hepatopathy

Potential Symptoms

Biliary stenting, infectious cholangitis, hepatic failure

Organ(s)

Kidneys

Frequency

Occasional

Systemic Manifestations

Renal parenchymal nodules, focal or diffuse

Potential Symptoms

Renal failure due to interstitial nephritis or glomerulonephritis

Organ(s)

Retroperitoneum

Frequency

Frequent

Systemic Manifestations

Retroperitoneal fibrosis
Periaortitis
Sclerosing mesenteritis

Potential Symptoms

Renal atrophy or injury due to hydronephrosis

GI, gastrointestinal; IgG4-RD, immunoglobulin G4-related disease.

EXPLORE IgG4-RD PROGRESSION

References:

1. Perugino CA, Stone JH. Nature. 2020;16:702-714. doi:10.1038/s41584-020-0500-7 2. Khosroshahi A, Wallace ZS, Crowe JL, et al. Arthritis Rheumatol. 2015;67(7):1688-1699. doi:10.1002/art.39132 3. Lanzillotta M, Mancuso G, Della-Torre E. BMJ. 2020;369:m1067. doi:10.1136/bmj.m1067 4. Prabhu SM, Yadav V, Irodi A, et al. Indian J Radiol Imaging. 2014;24(2):117-120. doi:10.4103/0971-3026.134384 5. Stone JH, Zen Y, Deshpande V. N Engl J Med. 2012;366:539-551. 6. Data on File. Amgen; December 2023. 7. Wallace ZS, Naden RP, Chari S, et al. Arthritis Rheumatol. 2020;72(1):7-19. 8. Legatowicz-Koprowska M. Cent Eur J Immunol. 2018;43(2):204-208. doi:10.5114/ceji.2018.77391 9. Wu S, Wang H. Front Immunol. 2023;14:1278332. doi:10.3389/fimmu.2023.1278332

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Symptoms & Flares

IgG4-RD respects no boundaries and can appear in unexpected organs with potentially serious manifestations1,2

Some manifestations of IgG4-RD put patients at high risk for serious complications, including pancreatic enlargement, retroperitoneal fibrosis, and aortitis1,2

When a patient with IgG4-RD begins to experience symptoms, they are specific to the affected organ(s).8,9

Thank you

IgG4-RD respects no boundaries and can appear in unexpected organs with potentially serious manifestations^1,2

Some manifestations of IgG4-RD put patients at high risk for serious complications, including pancreatic enlargement, retroperitoneal fibrosis, and aortitis^1,2

When a patient with IgG4-RD begins to experience symptoms, they are specific to the affected organ(s).^8,9