Ann Thorac Surg 2005;80:1914-1916
© 2005 The Society of Thoracic Surgeons
Case report
Successful Surgical Treatment of Takayasu's Arteritis Associated With Pyoderma Gangrenosum
Shinji Kanemitsu, MD, PhD
*
,
Takatsugu Shimono, MD, PhD,
Hitoshi Kusagawa, MD, PhD,
Koji Onoda, MD, PhD,
Isao Yada, MD, PhD
Department of Thoracic and Cardiovascular Surgery, Mie University School of Medicine, Mie, Japan
Accepted for publication June 4, 2004.
* Address correspondence to Dr Kanemitsu, Department of Thoracic and Cardiovascular Surgery, Mie University School of Medicine, 2-174, Edobashi, Tsu, Mie, 514–8507 Japan (Email: kanemitsu{at}kosei.anjo.aichi.jp).
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Abstract
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Pyoderma gangrenosum is rarely associated with Takayasu's arteritis. We report the successful surgical treatment of a 34-year-old woman who was diagnosed with pyoderma gangrenosum associated with thoracic aortic aneurysm and dissection due to Takayasu's arteritis. She underwent graft replacement of the thoracic aortic aneurysm under cardiopulmonary bypass, with perioperative management using prednisolone and cyclosporine A. She has had no evidence of the development of skin lesions or the progression of Takayasu's arteritis.
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Introduction
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Takayasu's arteritis consists of inflammation characterized by large artery occlusion and aneurysmal formation, and is most commonly found in young Asian females. Depending on race, cutaneous manifestations have been estimated to affect between 2.8% and 28% of patients [1–3]. Cutaneous manifestations include erythema nodosum in Europe and North America, and pyoderma gangrenosum in Japan. Most cases of pyoderma gangrenosum associated with Takayasu's arteritis have been observed in Japan, with reports appearing only rarely in the Western literature [1–3].
A 34-year-old woman presented at our hospital in 1990 with a skin eruption. Based on clinical and histopathologic findings, she was diagnosed to have pyoderma gangrenosum. The eruption was controlled with prednisolone and cyclosporine A. Chest radiography and computed tomography showed a thoracic aortic aneurysm, 70 mm in maximum diameter. Examination at presentation revealed numerous vesiculopustular lesions and cicatrisation lesions on the neck, the chest, and the upper limbs (Fig 1). Transthoracic echocardiography revealed moderate aortic regurgitation, dilated sinotubular junction and Valsalva sinus (44.4 mm), and well-preserved systolic function. Digital subtraction aortography showed the aneurysm from the ascending aorta to the aortic arch, but no obstruction or stenosis of the major vessels of the aortic arch or coronary artery. Systemic inflammation was controlled with prednisolone and cyclosporine A. Serum C-reactive protein (CRP) and white blood cell count were gradually reduced. Preoperative laboratory data included a white blood cell count of 11,430/mL and CRP less than 0.2. Human leukocyte antigen (HLA) typing analysis showed the presence of A9 and BW52 antigens.
The patient was diagnosed as having a thoracic aortic aneurysm and dissection of the descending aorta associated with pyoderma gangrenosum. After checking that a culture of her skin eruption was negative, we conducted ascending and arch replacement. The ascending aorta and the aortic arch were white colored owing to arteritis under the pericardial adhesion. Cardiopulmonary bypass was initiated with the right axillary and right femoral arteries as arterial inflow, the right atrium as a venous return, and a left ventricle vent through the right upper pulmonary vein. The distal ascending aorta was cross-clamped, and retrograde cardioplegia was conducted to obtain immediate cardiac arrest. The proximal anastomosis was performed at the sinotubular junction. After lowering the patient's core temperature to 20°C, we induced circulatory arrest, and selective cerebral perfusion was established. The aortic arch including the ascending aorta was replaced using a woven Dacron (C. R. Bard, Haverhill, PA) four-branched graft of 26 mm diameter (Hemashield; Boston Scientific Corporation, Boston, MA). The pathology findings of the resected aneurismal wall showed tears of the elastic fibers in the media containing inflammatory cells and thickened intima; the findings did not contradict as the typical findings of chronic stage of Takayasu's arteritis (Fig 2).
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Fig 2. Histologic findings of the aortic aneurysm wall. (A, B) The intima shows thickening, and the media has inflammatory cells (hematoxylin & eosin stain: A, x20; B, x40). (C) Immunohistochemical staining for elastica van Gieson shows tears of the elastic fibers in the media and the thickened intima.
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Perioperative management was with prednisolone and cyclosporine A. Preoperatively, oral prednisolone and cyclosporine A had been administered at 60 mg and 280 mg, respectively, during the operation, and for a few days after the operation they were given intravenously. The trough level of cyclosporine A had been maintained at 200 to 250 ng/mL. Postoperative aortography showed a reduction of aortic regurgitation. Echocardiography, performed 1 week after surgery, revealed that the diameter of the Valsalva sinus was reduced (to 38.9 mm), and that there was trivial aortic regurgitation. Her clinical course and pathology findings are consistent with a diagnosis of Takayasu's arteritis associated with pyoderma gangrenosum, which was successfully treated with prednisolone and cyclosporine A. The postoperative course was uneventful. Twenty months after surgery, the patient remained well, and there was no evidence of the development of skin lesions or of the progression of Takayasu's arteritis.
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Comment
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Pyoderma gangrenosum is characterized by relatively indolent ulcers with extensive necrosis around the edges of the lesions. While pyoderma gangrenosum may be an isolated finding, it is most often associated with ulcerative colitis, Crohn's disease, or rarely Takayasu's arteritis. Skin manifestations of Takayasu's arteritis can appear at any time; however, pyoderma gangrenosum tends to occur more frequently at the occlusive stage of the disease [1, 4]. Healing tends to parallel the activity of the underlying inflammatory bowel disease, and scarring is relatively common. There is also a relationship between the skin lesion and the area of arteritis. Several authors have hypothesized that both skin and arterial lesions are caused by autoantibodies to elastin [4]. In our case, the patient had a skin lesion on her chest and a thoracic aneurysm.
Corticosteroids are the mainstay of treatments for patients with Takayasu's arteritis, and are also the treatment of choice for pyoderma gangrenosum. Corticosteroid resistance in some patients is well recognized [5], however; in these patients, many other systemic agents including minocycline and methotrexate have been tried with varying success. An intravenous dosage of cyclosporin A is determined as about half the oral dose [6]; when our patient was switched from oral to intravenous administration, frequent measurements of the blood concentration were made to minimize toxicity. Mitruka and colleagues [7] have also reported using prednisolone and cyclosporine A in the cardiac operations in solid organ transplant recipients. Cyclosporine A levels reduced with the initiation of cardiopulmonary bypass [8]. Therefore, we think that monitoring serial cyclosporin A levels is important during the perioperative period. We did not have a specific maker to monitor the immune response in patients with Takayasu's arteritis. However, we evaluated the postoperative systemic inflammation from this patient's physical examination, leukocyte counts, and CRP values. In this case, the leukocyte counts and CRP values after surgery were within the range of the usual surgery with cardiopulmonary bypass without immunosuppressive drugs.
Although the results of surgery for Takayasu's arteritis presenting with an associated aortic aneurysm are generally improving, no previous report of successful surgical treatment using cardiopulmonary bypass for Takayasu's arteritis associated with pyoderma gangrenosum has been documented in the literature. This patient illustrates that pyoderma gangrenosum needs to be considered within the differential diagnosis of nonhealing wounds, and that treatment with cyclosporin should be contemplated in severe cases. It is also important to remember the rare possibility of underlying Takayasu's arteritis in any patient with pyoderma gangrenosum.
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References
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- Frances C, Boisnic S, Bletry O, et al. Cutaneous manifestations of Takayasu arteritisA retrospective study of 80 cases. Dermatologica 1990;181:266-272.[Medline]
- Perniciaro CV, Winkelmann RK, Hunder GG. Cutaneous manifestations of Takayasu's arteritisA clinicopathologic correlation. J Am Acad Dermatol 1987;17:998-1005.[Medline]
- Nakao K, Ikeda M, Kimata S, Niitani H, Niyahara M. Takayasu's arteritisClinical reports of eighty-four cases and immunological studies of seven cases. Circulation 1967;35:1141-1155.[Abstract/Free Full Text]
- Taieb A, Dufillot D, Pellegrin-Carloz B, et al. Postgranulomatous anetoderma associated with Takayasu's arteritis in a child Arch Dermatol 1987;123:796-800.[Abstract/Free Full Text]
- Shelhamer JH, Volkman DJ, Parrillo JE, Lawley TJ, Johnston MR, Fauci AS. Takayasu's arteritis and its therapy Ann Intern Med 1985;103:121-126.
- Parquet N, Reigneau O, Humbert H, et al. New oral formulation of cyclosporin A (Neoral) pharmacokinetics in allogeneic bone marrow transplant recipients Bone Marrow Transpl 2000;25:965-968.[Medline]
- Mitruka SN, Griffith BP, Kormos RL, et al. Cardiac operations in solid-organ transplant recipients Ann Thorac Surg 1997;64:1270-1278.[Abstract/Free Full Text]
- Eide TR, Beflenker S. Effect of cardiopulmonary bypass on plasma cyclosporin A levels in a renal transplant patient Anesth Analg 1992;74:288-290.[Medline]
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Abstract
Introduction
