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Dense IgG4 plasma cell infiltrates associated with chronic infectious aortitis: implications for the diagnosis of IgG4-related disease
Authors:Zakir Siddiquee  Nicholas A Zane  R Neal Smith  James R Stone
Institution:1. Casey Eye Institute, Oregon Health & Science University, Portland, OR 97239, USA;2. Department of Medicine, Oregon Health & Science University, Portland, OR 97239, USA;3. Devers Eye Institute, Legacy Health Systems, Portland, OR 97210, USA;4. Department of Public Health and Preventive Medicine, Oregon Health & Science University, Portland, OR 97239, USA;5. Department of Ophthalmology, Emory University, Atlanta, GA 30322, USA;6. Integrated Genomics Laboratory, Oregon Health & Science University, Portland, OR 97239, USA;7. Division of Ophthalmology, Ohio University, Columbus, OH 43228, USA;8. Department of Ophthalmology, The Ohio State University, Columbus, OH 43215, USA;9. Department of Ophthalmology, University of Miami, FL 33101, USA;10. Department of Ophthalmology, Medical College of Wisconsin, Milwaukee, WI 53226, USA;11. Department of Ophthalmology, Columbia University, New York, NY 10032, USA;12. Department of Ophthalmology and Visual Sciences, University of British Columbia, Vancouver, British Columbia V5Z 3N9, Canada;13. Department of Ophthalmology, University of California, San Diego, CA 92037, USA;14. Research Department, King Khaled Eye Specialist Hospital, Riyadh 11462, Saudi Arabia;15. Department of Ophthalmology, Wake Forrest University, Winston-Salem, NC 27103, USA;p. Ophthalmology Network, Royal Adelaide Hospital, Adelaide 5000, Australia;1. Center for Systems Biology, Massachusetts General Hospital and Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA;2. Department of Surgery, Division of Vascular and Endovascular Surgery, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA
Abstract:BackgroundIgG4-related aortitis is a newly recognized form of noninfectious aortitis that occurs as part of the spectrum of a systemic disease referred to as IgG4-related disease. IgG4-related aortitis is distinguished from giant cell aortitis and Takayasu aortitis in part by the presence of increased numbers of IgG4-expressing plasma cells. Chronic infectious aortitis can also display lymphoplasmacytic infiltrates, but the degree of IgG4 expression in these cases has not been specifically evaluated.MethodsTwo cases of chronic active infectious abdominal aortitis were prospectively identified. Both were due to gram-positive bacteria, and at least one of the cases was due to chronic active Staphylococcus aureus infection. The degree of IgG4 plasma cell infiltration was assessed by immunohistochemistry.ResultsBoth cases of chronic infectious aortitis focally displayed high levels of IgG4-expressing plasma cells, greater than 50% of the IgG-expressing plasma cells, and greater than 50 IgG4-expressing plasma cells per high-power field.ConclusionsFocal dense IgG4 plasma cell infiltrates can be seen in association with chronic infectious aortitis due to gram-positive bacteria, including Staphylococcus aureus. This observation supports the proposal that chronic Staphylococcus aureus infection may stimulate a Th2-mediated elevation in IgG4. The pathologic diagnosis of IgG4-related aortitis should not be based solely on the presence of increased IgG4 plasma cell counts from immunohistochemistry, but requires consideration of the overall pathology, including careful exclusion of infectious aortitis.
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