IgG4 related disease: Difference between revisions
(new page - plan to add/edit) |
|||
| (4 intermediate revisions by 2 users not shown) | |||
| Line 1: | Line 1: | ||
==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<ref>Nambiar S, Oliver TI. IgG4 Related Disease. [Updated 2022 May 10]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK499825/</ref> | |||
*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== | |||
*Typically, diagnosis not made in the ED | |||
===Outpatient Workup=== | |||
*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 | |||
==Disposition== | |||
==See Also== | |||
==External Links== | |||
==References== | |||
<references/> | |||
[[Category:Rheumatology]] | |||
Latest revision as of 18:07, 7 September 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[1]
- 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
- Typically, diagnosis not made in the ED
Outpatient Workup
- 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
Disposition
See Also
External Links
References
- ↑ Nambiar S, Oliver TI. IgG4 Related Disease. [Updated 2022 May 10]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK499825/