Images in Cardiovascular Medicine

Abdominal Aortic Aneurysm and Sarcoidosis: Rare Presentation of an Uncommon Disease

Sandeep S. Patel, MD Madjid Mirzai-Tehrane, MD David A. Ott, MD Ali Mortazavi, MD Haroonur Rashid, MD Benjamin Y.C. Cheong, MD, FACC

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71-year-old woman presented for follow-up evaluation of an infrarenal abdominal aortic aneurysm (AAA). The aneurysm had been diagnosed at another hospital 2 years earlier and was reportedly about 4 cm in diameter. The patient now reported having had intermittent abdominal pain for the past 4 to 5 months. She had previously undergone investigation for hilar adenopathy and lung nodules; results of a lung biopsy were inconclusive. Computed tomographic angiograms now showed a 6.4 × 6.3-cm complex infrarenal AAA with an irregular lumen (Fig. 1A) and with patchy enhancement in the aortic wall after contrast administration (Fig. 1B). Coronal, sagittal, and volume-rendered images revealed no dissection or rupture (Fig. 2). The patient underwent abdominal aortoiliac repair and Hemashield® (Boston Scientific Corporation; Natick, Mass) graft replacement of the right renal artery. During surgery, the aortic wall was friable and contained thick lymphoid tissue. Surgical histopathologic examination revealed severe, nonnecrotizing granulomatous inflammation consistent with sarcoidosis (Fig. 3). The patient was started on systemic steroid therapy for sarcoidosis before her discharge from the hospital.

Comment

Section Editor: Raymond F. Stainback, MD, Department of Adult Cardiology, Texas Heart Institute, 6624 Fannin St., Suite 2480, Houston, TX 77030

Sarcoidosis, a systemic disorder of unknown cause that is characterized by nonnecrotizing granuloma, typically affects the skin, the respiratory, cardiac, lymphatic, renal, and nervous systems, and the eyes.1 This case is unusual because the primary presenting symptom was abdominal pain caused by AAA. Only a few cases have been reported regarding sarcoid involvement of the aorta.2,3

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From: Departments of Cardiology (Drs. Cheong, Mirzai-Tehrane, Mortazavi, Patel, and Rashid), Cardiovascular Surgery (Dr. Ott), and Radiology (Dr. Cheong), Texas Heart Institute, Houston, Texas 77030 Address for reprints: Benjamin Y.C. Cheong, MD, FACC, Department of Diagnostic and Interventional Radiology, MC 2-270, CHI St. Luke’s Health–Baylor St. Luke’s Medical Center, 6720 Bertner Ave., Houston, TX 77030 E-mail: [email protected] © 2015 by the Texas Heart ® Institute, Houston

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Fig. 1 Computed tomographic angiograms (axial views) show the aneurysm with intraluminal thrombus. A) Before contrast administration, a region of interest was seen over the aortic wall (arrow, 38 Hounsfield units). B) After administration, enhancement was seen in this region (arrow, 128 Hounsfield units).

http://dx.doi.org/10.14503/THIJ-13-3691

Volume 42, Number 1, 2015

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Fig. 2 Computed tomograms. A) Coronal and B) sagittal images (multiplanar reconstruction) reveal the complex nature of the infrarenal aneurysm; however, no dissection or rupture was seen. C) Volume-rendered image shows involvement of the iliac arteries.

symptoms of infection, an inflammatory cause should be considered during differential diagnosis.

Acknowledgments We thank Cristina Montalvo, MD, of the Department of Pathology, CHI St. Luke’s Health–Baylor St. Luke’s Medical Center, who provided the photomicrograph. Stephen N. Palmer, PhD, ELS, contributed to the editing of the manuscript.

References

Fig. 3 Photomicrograph of the surgical specimen shows severe noncaseating granuloma and multinucleated giant cells (arrows) that suggest sarcoidosis (H & E, orig. ×400).

Atherosclerosis is the most frequent cause of AAA.4 Other risk factors include older age, active smoking, a family history of aneurysm, and current cardiovascular disease. Our case is somewhat unusual in that the lumen of the aneurysm was diffusely irregular, with patchy areas of contrast enhancement. In the absence of Texas Heart Institute Journal

1. Newman LS, Rose CS, Maier LA. Sarcoidosis [published erratum appears in N Engl J Med 1997;337(2):139]. N Engl J Med 1997;336(17):1224-34. 2. Numata S, Kanda K, Hatta T, Tanda S, Inoue T, Doi K, et al. Sarcoidosis with double saccular abdominal aortic aneurysms. J Vasc Surg 2005;41(6):1065. 3. Weiler V, Redtenbacher S, Bancher C, Fischer MB, Smolen JS. Concurrence of sarcoidosis and aortitis: case report and review of the literature. Ann Rheum Dis 2000;59(11):850-3. 4. Kent KC, Zwolak RM, Egorova NN, Riles TS, Manganaro A, Moskowitz AJ, et al. Analysis of risk factors for abdominal aortic aneurysm in a cohort of more than 3 million individuals. J Vasc Surg 2010;52(3):539-48.

Abdominal Aortic Aneurysm and Sarcoidosis

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Abdominal aortic aneurysm and sarcoidosis: rare presentation of an uncommon disease.

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