Author + information
- Received September 5, 2017
- Revision received October 9, 2017
- Accepted October 12, 2017
- Published online February 5, 2018.
- Marat Fudim, MDa,∗ (, )
- Matthew P. Thorpe, MD, PhDb,
- Leslie L. Chang, BSc,
- E. William St. Clair, MDd,
- Lynne M. Hurwitz Koweek, MDb and
- Andrew Wang, MDa
- aDepartment of Medicine, Division of Cardiology, Duke University, Durham, North Carolina
- bDepartment of Radiology, Division of Radiology, Duke University, Durham, North Carolina
- cDuke School of Medicine, Durham, North Carolina
- dDepartment of Medicine, Division of Rheumatology and Immunology, Duke University Medical Center, Durham, North Carolina
- ↵∗Address for correspondence:
Dr. Marat Fudim, Duke University Medical Center, Duke Clinical Research Institute, 2301 Erwin Road, Durham, North Carolina 27710.
Fibroinflammatory disorders are rare disorders characterized by chronic inflammation in combination with fibrosis that affect multiple organs. Among the fibroinflammatory disorders, 2 present with specific cardiovascular involvement, the immunoglobulin G4–related disease, which presents with perivascular infiltration in approximately 5% of patients (1), and Erdheim-Chester disease with periaortic, pericardial, myocardial, or less often pericoronary fibrosis in 30% of cases (2). Whereas tissue biopsy with immunohistochemistry is the current gold standard for diagnosis for immunoglobulin G4–related disease and Erdheim-Chester disease, imaging with 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) can guide the diagnosis. Wall thickening of the aorta and coronary arteries on cross-sectional imaging can be due to an intramural hematoma, lymphoma, vasculitis (e.g., giant cell arteritis, Kawasaki disease, sarcoid, histiocytosis, relapsing polychondritis, rheumatoid arthritis) or fibroinflammatory disorders. A multisystem involvement would favor the latter 2 etiologies, and positive laboratory findings could help diagnose vasculitis. In this paper, we present 2 cases of fibroinflammatory diseases involving the cardiovascular system to illustrate the role of 18F-FDG-PET/CT imaging in the diagnosis of fibroinflammatory disease (Figures 1, 2, 3, 4, 5, and 6⇓⇓⇓⇓⇓⇓). Furthermore, our cases demonstrate the utility of 18F-FDG-PET/CT imaging to detect degree and site of disease. This information can be useful in guiding the optimal site for biopsy (as in this report), the timing of surgical intervention (if needed), the timing of medical intervention (corticosteroids or other immunosuppressives), and response to medical intervention.
Dr. Fudim is supported by American Heart Association grant 17MCPRP33460225 and National Institutes of Health T32 grant 5T32HL007101. Dr. St. Clair has received consulting fees and a research grant form Bristol-Myers Squibb; an in-kind and study drug support for a National Institutes of Health–funded clinical trial of baminercept in primary Sjøgren syndrome; and royalties from UpToDate. Dr. Wang has received research support from MyoKardia, Abbott Vascular, and Gilead Science. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received September 5, 2017.
- Revision received October 9, 2017.
- Accepted October 12, 2017.
- 2018 American College of Cardiology Foundation