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IgG4-Related Aortitis
Chun Wa Fong1, Lin Ian Lio2, Monica Pon3
1Department of Plastic Surgery, Centro Hospitalar Conde São Januário, Macau SAR.
European Journal of Case Reports in Internal Medicine
|February 14, 2019
Summary
Immunoglobulin G4-related disease (IgG4-RD) can affect multiple organs. Early diagnosis of IgG4-related aortitis is crucial to prevent irreversible damage and ensure effective treatment with medications like prednisolone.
Area of Science:
- Immunology
- Rheumatology
- Radiology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic fibroinflammatory condition.
- It can manifest with tumefactive lesions affecting various organs, often with elevated serum IgG4 levels.
- Early diagnosis is challenging but vital to prevent organ damage.
Purpose of the Study:
- To report a case of IgG4-related aortitis.
- To highlight the diagnostic utility of PET-CT in such cases.
- To demonstrate successful treatment outcomes.
Main Methods:
- Case presentation of a 28-year-old male with a 2-year history of nonspecific symptoms.
- Diagnostic imaging using PET-CT scan.
- Treatment with prednisolone and mycophenolate mofetil.
Main Results:
- Diagnosis of IgG4-related aortitis confirmed via PET-CT.
- Complete clinical and radiological resolution achieved post-treatment.
- Successful management of a rare manifestation of IgG4-RD.
Conclusions:
- IgG4-related disease is a multisystemic immune-mediated disorder.
- Aortitis should be considered in the differential diagnosis of unexplained fever and nonspecific symptoms.
- Timely diagnosis and treatment are essential to avoid severe complications.
