IgG4 related disease: Difference between revisions
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==Background== | ==Background== | ||
*IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory illness of unknown etiology | *IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory illness of unknown etiology | ||
*It's a multi organ disease that most commonly affects the pancreas, kidneys, retroperitoneum, salivary glands and orbits | |||
==Clinical Features== | |||
*No specific symptoms as it is dependent on which organs are affected | |||
*Autoimmune pancreatitis is the most common presentation of IgG4-RD and can be mistaken for malignancy | |||
*Patients do not typically experience generalized symptoms such as fevers or night sweats | |||
==Differential Diagnosis== | |||
{{Pancreatic cancer}} | |||
{{Sjogren's syndrome}} | |||
{{Primary sclerosing cholangitis}} | |||
{{Retroperitoneal fibrosis}} | |||
{{Castleman disease}} | |||
==Evaluation== | |||
*Tissue biopsy with IgG stain is required for diagnosis | |||
*Serum IgG4 levels are usually elevated and helpful for diagnosis and also helpful for serial monitoring | |||
*C3 and C4 complement levels may be decreased | |||
*Total serum IgG levels may also be elevated | |||
==Management== | |||
*Prednisone at higher doses is the current standard treatment | |||
*Further immunosuppression may be warranted based on response to prednisone | |||
*Consultation with a rheumatologist is usually necessary | |||
==References== | |||
<references/> | |||
[[Category:Rheumatology]] | |||
Revision as of 06:53, 18 August 2022
Background
- IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory illness of unknown etiology
- It's a multi organ disease that most commonly affects the pancreas, kidneys, retroperitoneum, salivary glands and orbits
Clinical Features
- No specific symptoms as it is dependent on which organs are affected
- Autoimmune pancreatitis is the most common presentation of IgG4-RD and can be mistaken for malignancy
- Patients do not typically experience generalized symptoms such as fevers or night sweats
Differential Diagnosis
Template:Pancreatic cancer Template:Sjogren's syndrome Template:Primary sclerosing cholangitis Template:Retroperitoneal fibrosis Template:Castleman disease
Evaluation
- Tissue biopsy with IgG stain is required for diagnosis
- Serum IgG4 levels are usually elevated and helpful for diagnosis and also helpful for serial monitoring
- C3 and C4 complement levels may be decreased
- Total serum IgG levels may also be elevated
Management
- Prednisone at higher doses is the current standard treatment
- Further immunosuppression may be warranted based on response to prednisone
- Consultation with a rheumatologist is usually necessary