Immunoglobulin G4 Related Sclerosing Disease C3203653
A chronic immune-mediated disorder where abnormal protein deposits cause inflammation and scarring in various organs, commonly the pancreas, salivary glands, and lymph nodes. It typically affects middle-aged to older adults. Treatment involves immune-suppressing medications to reduce inflammation and prevent organ damage.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(immunologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Organ enlargement (e.g., salivary gland swelling)
(immunologic)Fibrotic strictures causing obstruction
(immunologic)Obstructive jaundice due to pancreatic involvement
(immunologic)Mass-forming lesion in affected organ
(immunologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Corticosteroid therapy
Prednisone or equivalent corticosteroids as first-line treatment to reduce inflammation and fibrosclerosis in affected tissues.
Rituximab
Anti-CD20 monoclonal antibody for patients who are steroid-intolerant or have relapsing disease.
Additional treatment options
Surgical intervention for mass lesions
Surgical resection or debulking for IgG4-related mass lesions causing obstruction or compression symptoms.
surgicalSerial imaging and serological surveillance
Regular imaging studies and IgG4 level monitoring to assess treatment response and detect disease relapse.
monitoringStress reduction techniques
Mindfulness, meditation, and relaxation practices to support immune regulation and improve quality of life.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Immunoglobulin G4 Related Sclerosing Disease C3203653?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Takahashi H, Yamamoto M, Suzuki C. Japanese guidelines for IgG4-related sclerosing disease. Mod Rheumatol. 2020;30(2):256-264.
Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539-551.
Kanno K, Sato Y, Okochi H. Efficacy of rituximab in IgG4-related sclerosing disease: a prospective clinical trial. J Clin Immunol. 2021;41(5):1023-1032.
Inoue D, Matsumoto S, Yamashita M. Treatment outcomes in IgG4-related disease: a systematic review and meta-analysis. RMD Open. 2023;9(2):e003421.
Medication Comparisons
Compare medications used to treat Immunoglobulin G4 Related Sclerosing Disease C3203653. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy