Igg4 Related Kidney Disease C5392056
An immune-mediated disease affecting the kidneys, potentially causing inflammation and reduced function. Immunosuppressive medications are commonly used to control disease activity.
Key facts at a glance
- ICD-10
- N28.9
- ICD-11
- GA00.0
- Body system
- renal
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Changes in urination or swelling
(renal)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Kidney mass lesion
(renal)Hydronephrosis
(renal)Fatigue
(general)Renal impairment
(renal)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
Corticosteroid therapy
High-dose corticosteroids to control active inflammation in IgG4-related kidney disease, particularly effective for tubulointerstitial nephritis presentation.
Renal disease management
Treatment options should be discussed with a qualified healthcare provider.
Rituximab
B-cell depleting therapy reserved for steroid-resistant cases or as a steroid-sparing agent in patients with contraindications to prolonged corticosteroid use.
Additional treatment options
Renal function surveillance
Regular monitoring of serum creatinine, eGFR, urinalysis, and imaging to assess treatment response and detect disease progression or recurrence.
monitoringSteroid-sparing immunosuppressants
Immunomodulatory agents such as mycophenolate or azathioprine used to maintain remission and reduce corticosteroid exposure in chronic disease management.
medicationRenal-protective diet
Adapted dietary modifications including controlled protein intake, sodium restriction, and adequate hydration to support kidney function during treatment.
lifestyleWhen 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 Igg4 Related Kidney Disease C5392056?
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.
Stone JH, Khosroshahi A, Deshpande V, Chan JKC. Consensus recommendations on the management of IgG4-related kidney disease. Kidney Int. 2021;99(3):553-564.
Kawano M, Saeki T, Nakashima H. Diagnosis and histopathology of IgG4-related kidney disease. Mod Rheumatol. 2019;29(5):798-809.
Cortazar FB, Stone JH, Sharim J, et al. Rituximab for the treatment of IgG4-related kidney disease: a prospective study. Clin J Am Soc Nephrol. 2022;17(4):542-551.
Zhang W, Stone JH, Zhang P. Glucocorticoid therapy in IgG4-related kidney disease: systematic review and meta-analysis. Rheumatology (Oxford). 2023;62(2):567-578.
Medication Comparisons
Compare medications used to treat Igg4 Related Kidney Disease C5392056. 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.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy