A 70-year-old man develops sudden severe chest/back pain. CT angiography shows an infrarenal aortic aneurysm (7 cm) with surrounding inflammatory infiltrate and fibrosis. Biopsy shows lymphoplasmacytic infiltrate with giant cells and IgG4+ plasma cells. This 'inflammatory aortic aneurysm' is now classified within:
- A Giant cell arteritis (GCA) affecting the aorta in elderly men
- B Takayasu arteritis extending to the abdominal aorta
- C IgG4-related aortitis/periaortitis — a manifestation of IgG4-related disease ✓
- D Tertiary syphilitic aortitis with obliterative endarteritis
Explanation
Inflammatory aortic aneurysm with dense lymphoplasmacytic infiltrate, periaortic fibrosis, and IgG4+ plasma cells (>50 IgG4+ cells/HPF, IgG4/IgG ratio >40%) is now recognized as IgG4-related aortitis/periaortitis, part of the IgG4-related disease (IgG4-RD) spectrum. These patients often have elevated serum IgG4, respond to corticosteroids, and may have other IgG4-RD manifestations. GCA affects medium-large vessels with granulomatous inflammation in elderly women (temporal artery predilection). Syphilitic aortitis shows obliterative endarteritis of vasa vasorum with tree-bark intimal wrinkling and affects the ascending aorta.
Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.