Vascular Survey in IgG4-Related Systemic Disease
Brittany J Holmes, Nikolay G Delev, Gary R Pasternack, Marc K Halushka. The Johns Hopkins Hospital, Baltimore, MD
Background: IgG4-related systemic disease (IgG4-SD) is a rare cause of non-necrotizing ascending aortitis. However, the extent of IgG4-related arteritis outside of the aorta has not been described. Although IgG4-SD is increasingly recognized as a cause of aortic aneurysms, coronary artery dissection and sudden cardiac death have not been reported.
Design: We describe the pattern of IgG4-related arteritis in a 61-year-old African-American female. This patient died unexpectedly at home one year following an episode of presumptive lupus cerebritis that resolved with immunosuppression. An extensive vascular survey was conducted at the time of post-mortem examination. Immunohistochemical staining using anti-IgG (dilution 1:20,000, Novacastra) and anti-IgG4 (dilution 1:2,000, Invitrogen) was performed on selected sections.
Results: Postmortem examination revealed a 3 cm dissection of the ascending aorta that had propagated in a retrograde fashion into the proximal left main coronary artery. This significant coronary artery dissection caused the patient's sudden cardiac death. Microscopic examination of the proximal aorta revealed a marked lymphoplasmacytic arteritis where the dissection occurred. The plasma cells demonstrated a cytoplasmic IgG4:IgG ratio of approximately 80%. Our expanded vascular survey identified the same plasma cell-rich infiltrate affecting the entire aorta (thoracic and abdominal) as well as the bilateral common iliac arteries, left renal artery, and bilateral internal carotid arteries. The coronary arteries, right renal artery and splenic artery were unaffected.
Conclusions: We report the first extensive vascular survey of IgG4-SD, demonstrating that IgG4-SD can cause large-vessel vasculitis beyond the aorta. While IgG4-SD is typically viewed as a chronic disease, this case is also notable as the first report of IgG4-SD-induced sudden death and illustrates the possibility of vascular catastrophe due to IgG4-related arteritis.
Wednesday, March 21, 2012 1:00 PM
Poster Session VI # 41, Wednesday Afternoon