Journal of Medical & Surgical Pathology
Open Access
ISSN: 2472-4971
ISSN: 2472-4971
Monica Pon and Sara Evora
NAFLD, China
Posters & Accepted Abstracts: J Med Surg Pathol
Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated fibro inflammatory condition, characterized by tumefied lesions that can affect multiple organs. Clinical manifestations depend on organ involvement and serum IgG4 levels can be elevated. Histopathology is gold standard which should be obtained whenever possible. Glucocorticoid remains the first-line agent for remission induction in all patients with active, untreated IgG4-RD unless contraindicated. Among the involved organs, large vessel involvement can be easily overlooked and not be timely diagnosed, mainly due to its nonspecific presentations and difficulty to obtain a pathological specimen. Some of the reported cases may have already led to irreversible damage or significant complications. Herein we had a case of IgG4-RD involving the aortic arch, ascending aorta, thoracic descending aorta, left carotid artery and right subclavian/axillary artery. The 28-year-old male patient presented with recurrent nocturnal low grade fever for more than one year accompanied with chills, night sweating, ill-defined back pain and upper abdominal pain. We reached the diagnosis in four months after he first visited our department. The patient achieved overall clinical improvement within one month after prednisolone and mycophenolate mofetil was started. Follow up serum IgG-4 concentration was decreased and PET/CT scan also showed significant therapy response with complete resolution of the vascular lesions. Without delaying diagnosis and treatment, no major complications occurred. In conclusion, IgG4-related aortitis can be one of the differential diagnoses in patients with fever of unknown origin, back pain and other unspecific symptoms. The goal is to make timely diagnosis and give appropriate treatment.