IgG4-Related Aortitis: Multimodality Imaging Approach

IgG4-related aortitis mimicking intramural hematoma

Acute aortic syndromes (AAS) are life-threatening conditions, and despite advances in imaging techniques, their diagnosis and treatment remain challenging. While intramural hematoma is much less common than acute aortic dissection (AD), which is the most common form of AAS, they have similar mortality rates (1). On the other hand, aortitis may mimic intramural hematoma (IMH) due to radiologic s...

IgG4-related disease manifesting as pachymeningitis and aortitis.

IgG4 Aortitis: A Case Report.

IgG4 aortitis is one of the entities seen in the spectrum of IgG4-related disease (IgG4-RD). It is characterized by serologic (elevated serum IgG4) and histologic features including a lymphoplasmacytic infiltrate with increased numbers of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Some studies have described a correlation between infections and IgG4 aortitis. We ...

IgG4-Related Autoimmune Pancreatitis Mimic Pancreatic Tumor with Aortitis — A Case Report

IgG4-related diseases (IgG4-RD) are a spectrum of diseases involving many organs. Type 1 autoimmune pancreatitis is the most common disease of IgG4-RD. It often presents with obstructive jaundice and swelling of the pancreas. We report a case of a 56-year-old male with initial presentation of epigastric pain. Endoscopic abdominal sonogram, CT scan and MRI revealed a tumor over pancreatic tail. ...

Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study

INTRODUCTION Immunoglobulin G4 (IgG4)-related aortitis/periaortitis and periarteritis are vascular manifestations of IgG4-related disease. In this disease, the affected aneurysmal lesion has been suspected to be at risk of rupture. In this study, we aimed to clarify the clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis. METHODS We retrospecti...