IgG4-related Aortitis

Authors

• Chun Wa Fong Department of Plastic Surgery, Centro Hospitalar Conde São Januário, Macau SAR
• Lin Ian Lio Department of Psychiatry, Centro Hospitalar Conde São Januário, Macau SAR
• Monica Pon Department of Internal Medicine, Centro Hospitalar Conde São Januário, Macau SAR
• Toi Meng Mok Department of Cardiology, Centro Hospitalar Conde São Januário, Macau SAR
• Hou Ng Department of Internal Medicine, Centro Hospitalar Conde São Januário, Macau

monicapon76@gmail.com

Keywords

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory condition characterized by tumefactive lesions that can affect multiple organs. Serum IgG4 levels may be elevated. Early recognition is sometimes difficult but is important to avoid irreversible organ damage. We describe the case of a 28-year-old male patient who presented with a 2-year history of recurrent low-grade fever, night sweats and non-specific manifestations. We eventually diagnosed IgG4-related aortitis by PET-CT scan. The patient was successfully treated with prednisolone and mycophenolate mofetil with complete clinical and radiological resolution.