IgG4-related systemic disease and lymphoplasmacytic aortitis

Area of Science:

• Cardiovascular Medicine
• Rheumatology
• Immunology

Background:

• Idiopathic aortitis is a group of inflammatory conditions affecting the aorta of unknown cause.
• Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect multiple organs.

Observation:

• A patient presented with ascending aortic dissection in the context of IgG4-RD.
• Aortic surgery revealed a transmural lymphoplasmacytic infiltrate with IgG4-positive plasma cells in the aortic media.
• Elevated serum IgG4 levels and prior mediastinal lymph node biopsy consistent with IgG4-RD were noted.

Findings:

• The aortic infiltrate demonstrated polytypic kappa and lambda light chains, indicating a polyclonal plasma cell response.
• The patient's IgG4 levels were significantly elevated (nearly 10-fold).
• Glucocorticoid therapy led to a prompt clinical response, suggesting efficacy in managing IgG4-RD-associated aortitis.

Implications:

• IgG4-RD should be considered in the differential diagnosis of noninfectious aortitis, including ascending aortic involvement.
• Effective management of IgG4-RD with glucocorticoids may offer a therapeutic strategy for associated aortitis, potentially preventing organ damage.
• This finding necessitates a re-evaluation of idiopathic aortitis classification and management strategies.