Author + information
- Nilay Kumar, MD∗ ( and )
- Neetika Garg, MD
- ↵∗Department of Medicine, University of Alabama at Birmingham, 619 19th Street South, Birmingham, Alabama 35233
We read with keen interest the paper by Posligua et al. (1) in which the authors describe a young woman with left main and right coronary artery ostial stenosis due to an infiltrative fibrous mass involving the aortic root and arch, ascending aorta, and pulmonary artery, and extrinsically compressing the coronary arteries. The authors conclude that the cause of the mass remained idiopathic despite extensive workup. It is worth mentioning that immunoglobulin G4 (IgG4)-related disease was not considered in the differential diagnoses. We would like to know whether serum IgG4 levels were checked and whether tissue staining for IgG4-positive plasma cells was performed.
First described in association with autoimmune pancreatitis, IgG4-related disease is now understood to be a systemic fibroinflammatory condition characterized by tumefactive lesions, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and storiform fibrosis in a matted or whorled pattern with or without elevated serum IgG4 levels. Although the authors describe the histological findings, they do not clarify whether fibrosis was present in a pattern that may be suggestive of IgG4-related disease. Coronary obstruction because of mediastinal fibrosis has been described in the past (2). Recent evidence indicates that several previously recognized fibrosing conditions such as mediastinal fibrosis, Reidel's thyroiditis, inflammatory pseudotumor, and inflammatory aortitis are part of the spectrum of IgG4-related disease (3). Physicians should remain cognizant of IgG4-related disease when evaluating localized or systemic fibrosclerosing conditions before labeling them “idiopathic.” This has therapeutic and prognostic implications because glucocorticoids and the anti-CD20 antibody rituximab are considered effective treatment strategies (3).
- 2013 American College of Cardiology Foundation