IgG4 related disease: Difference between revisions
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==Differential Diagnosis== | ==Differential Diagnosis== | ||
*Pancreatic cancer | |||
*Sjogren's syndrome | |||
*Primary sclerosing cholangitis | |||
*Retroperitoneal fibrosis | |||
*Castleman disease | |||
==Evaluation== | ==Evaluation== | ||
Revision as of 06:54, 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
- 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