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Large renal artery aneurysm in Takayasu arteritis
Large renal artery aneurysm in Takayasu arteritis Kentaro Matsubara, MD,a Kenji Matsumoto, MD, PhD,a Kaori Kameyama, MD, PhD,b Hideaki Obara, MD, PhD,a and Masaki Kitajima, MD, PhD,a Tokyo, Japan Takayasu arteritis (TA), a primary arteritis of unknown cause, commonly affects the aorta and its main branches. Most patients with TA have stenotic or occlusive changes; aneurysmal degeneration is relatively rare, and renal artery aneurysms are extremely rare. We observed a fusiform, 7-cm renal artery aneurysm in a 57-year-old man with TA who had been followed up for 17 years. The patient underwent nephrectomy and resection of the aneurysm. Histologic examinations of resected specimens of the lesion showed characteristics typical of TA. ( J Vasc Surg 2006;44:1107-9.)
Takayasu arteritis (TA), which was first described in 1908 by a Japanese ophthalmologist,1 is an uncommon inflammatory arteritis that leads to occlusion or ectatic changes in the aorta and its main branches. Most patients are Asian, and the reported incidence in Japan (on the basis of autopsy studies) is 1 case per 3000 people.2 The presence of TA may be suggested by clinical manifestations of aortitis such as neck pain or a systemic inflammation of unknown origin indicated by an increased C-reactive protein concentration or erythrocyte sedimentation rate. The diagnosis is usually based on the results of a magnetic resonance angiography, computed tomography, or digital subtraction angiography examination. Several sets of criteria for the definitive diagnosis of TA are used, including those of Ishikawa,3 the American College of Rheumatology,4 and Sharma et al.5 Most patients with TA have stenotic or occlusive arterial changes. Aneurysmal degeneration is relatively rare, occurring in perhaps 10% to 30% of cases6-8; the actual frequency is unknown. When aneurysmal changes do develop, the aorta is the vessel most often affected, although degeneration of the arch branches of the aorta and the iliac and visceral vessels has been reported.9 We here report a large (7-cm) fusiform renal artery aneurysm (RAA) in a patient with TA. CASE REPORT The patient initially presented with malaise when he was 33 years old. Because leukocytosis and elevation of C-reactive protein and erythrocyte sedimentation rate values were observed, periodic follow-up was performed. When the patient was 40 years old, hypertension and headache developed, and digital subtraction angiography showed irregular, dilated bilateral subclavian arteries and thoracoabdominal aorta, a dilated left renal artery, and partial infarction of the left kidney. A diagnosis of TA was made, and treatment with prednisolone (30 mg/d) was begun immediately. IntermitFrom the Department of Surgerya and the Division of Diagnostic Pathology,b Keio University School of Medicine. Competition of interest: none. Reprint requests: Kenji Matsumoto, MD, PhD, Department of Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan (e-mail: [email protected]). 0741-5214/$32.00 Copyright © 2006 by The Society for Vascular Surgery. doi:10.1016/j.jvs.2006.07.016
tent claudication of the left leg appeared when the patient was 45 years old, and digital subtraction angiography showed aneurysmal dilatation of the left external iliac artery. Conservative follow-up continued, and 2 years later, the patient’s clinical symptoms had disappeared and complete thrombotic occlusion of the aneurysm at the left external iliac artery and development of collateral vessels was observed. The dose of prednisolone was gradually tapered in accordance with improvements in C-reactive protein and erythrocyte sedimentation rate values. The patient stopped taking prednisolone at the age of 51 years, and neither changes in laboratory values nor symptom developments occurred. Subsequently, at the age of 57 years, the patient underwent an abdominal ultrasonographic examination because of an increase in serum alkaline phosphatase. The assessment incidentally revealed a 7-cm fusiform aneurysm in the left renal artery. Abdominal computed tomography imaging (Fig 1) showed that the lesion extended from the origin of the left renal artery to the hilum of the left kidney. It also revealed a 4.2-cm dilatation of the suprarenal abdominal aorta. No remarkable changes in the patient’s laboratory values or symptoms were observed. The discovery of the large RAA was incidental because until the increase in alkaline phosphatase occurred, the results of the patient’s blood tests had been within normal limits and he had no symptoms; thus, a radiologic evaluation had not been considered necessary. The patient underwent transabdominal exploratory laparotomy through a median incision, a procedure customarily used at our institution. The large aneurysm was found to have pushed the left kidney toward the lower part of the pelvis. Nephrectomy was considered necessary because the left kidney had atrophic changes and the aneurysm affected the entire length of the left renal artery. Because the RAA was very large and at high risk of rupture with operative manipulation, resection of this lesion was given precedence over resection of the left kidney. Thus, the left renal vein was cut, and the RAA was resected. The left kidney was then removed. Histologic studies of the wall of the resected RAA (Fig 2) showed severe fibrotic changes and marked infiltration of lymphocytes into the outer part of the media. Degeneration had resulted in an almost complete absence of the media, and the vasa vasorum showed intimal prolif1107
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DISCUSSION
Fig 1. Abdominal and three-dimensional computerized tomography (CT) images. A, An abdominal CT scan shows a 7.0-cm fusiform aneurysm in the left renal artery. B, A three-dimensional CT scan shows that the aneurysm extends from the origin of the left renal artery to the hilum of the left kidney. The suprarenal abdominal aorta has a 4.2-cm dilatation.
eration and marked luminal narrowing. These findings were compatible with the scar stage of TA. The patient recovered uneventfully and was discharged on the 13th postoperative day in good condition. The patient’s long hospitalization was due to lack of a home caregiver. A magnetic resonance angiography image obtained before discharge showed a normal postoperative appearance. Eight months after surgery, another magnetic resonance angiography study showed no signs of recurrence of the aneurysm (Fig 3), and a clinical evaluation revealed no hypertension or other symptoms.
RAAs are uncommon, occurring in only approximately 0.09% of the general population.10 Most cases are due to degeneration of the renal artery caused by atherosclerosis, fibromuscular dysplasia, inflammation with arteritis, a congenital defect, trauma, or iatrogenic complications after kidney puncture.11 Intraparenchymal aneurysms of the kidney are frequently caused by arteritis disorders such as periarteritis nodosa.11 We found one previous report of an RAA associated with TA.6 However, our case is apparently the first in which a large RAA was found during follow-up in a patient with TA, and definitive histologic characteristics of TA were found in resected specimens of the aneurysm wall. Most RAAs are 1.7 to 2.6 cm when initially observed and managed; lesions larger than 5.0 cm on initial observation are extremely rare.12,13 Surgical treatment is generally performed only for RAAs larger than 2 cm. However, this practice may not be optimal, because rupture of RAAs smaller than 2.0 cm has been reported.13 Thus, the indications for surgical treatment remain controversial. TA has two recognized stages. The first is characterized by fever, myalgia, and anorexia in approximately two thirds of patients. In the second stage, these symptoms may be followed by multiple arterial occlusive symptoms, with the specific manifestations dependent on disease location.14 In patients given a definitive diagnosis of TA, prompt initiation of steroid therapy should be considered to suppress inflammatory development. Aneurysmal changes are uncommon in TA. In reviewing 16 reported series that included a total of 726 patients with TA, Sharma et al8 found that aneurysmal formation had occurred in 67 patients, an overall incidence of 9.2%. Most patients with TA have a favorable prognosis because the disease progresses slowly and because disease processes can be inactivated if adequate treatment is provided. Patients with TA in whom aneurysms are discovered, however, should be considered to have a serious, life-threatening disorder.15 Moreover, long-term steroid therapy for TA may result in weakening of arterial walls and an increase in the risk of aneurysm rupture. Hall et al16 reported the rupture of a thoracic aortic aneurysm in a patient with TA that occurred 3 weeks after steroid therapy was begun. Our patient did undergo long-term steroid therapy, but we do not think that this was responsible for his RAA because histologic studies of the wall of the resected lesion showed typical TA characteristics. Even after surgical treatment, patients with TA require periodic, long-term observation. For example, our patient must be closely observed for formation of an aneurysm at the stump of the left renal artery. A serious, unavoidable complication that commonly develops over the long term in patients with TA who undergo prosthetic graft interposition is anastomotic aneurysm at the interface between the native vessel and prosthetic graft.17 Because TA weakens the arterial wall, patients with the disease who have undergone aneurysm resection should always be considered at
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Fig 2. Histologic sections of the renal artery aneurysm show severe fibrotic changes and marked infiltration of lymphocytes into the outer part of the media. A, Stain, hematoxylin-eosin; original magnification, ⫻15. B, Stain, Elastica van Gieson; original magnification, ⫻15.
Fig 3. Magnetic resonance angiogram at 8 months after resection of the left renal artery aneurysm and left kidney shows a normal postoperative appearance.
risk of new aneurysm development and rupture and are thus in need of careful observation and, perhaps, early additional surgery. REFERENCES 1. Ito I. Aortitis syndrome (Takayasu’s arteritis). A historical perspective. Jpn Heart J 1995;36:273-81. 2. Nasu T. Takayasu’s truncoarteritis in Japan. A statistical observation of 76 autopsy cases. Pathol Microbiol (Basel) 1975;43:140-6. 3. Ishikawa K. Diagnostic approach and proposed criteria for the clinical diagnosis of Takayasu’s arteriopathy. J Am Coll Cardiol 1988;12:964-72.
4. Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990;33:1129-34. 5. Sharma BK, Siveski-Iliskovic N, Singal PK. Takayasu arteritis may be underdiagnosed in North America. Can J Cardiol 1995;11:311-6. 6. Matsumura K, Hirano T, Takeda K, Matsuda A, Nakagawa T, Yamaguchi N, et al. Incidence of aneurysms in Takayasu’s arteritis. Angiology 1991; 42:308-15. 7. Yamato M, Lecky JW, Hiramatsu K, Kohda E. Takayasu arteritis: radiographic and angiographic findings in 59 patients. Radiology 1986; 161:329-34. 8. Sharma S, Rajani M, Kamalakar T, Kumar A, Talwar KK. The association between aneurysm formation and systemic hypertension in Takayasu’s arteritis. Clin Radiol 1990;42:182-7. 9. Rigberg DA, Quinones-Baldrich W. Takayasu’s disease: nonspecific aortoarteritis. In: Rutherford RB, editor. Vascular surgery. 6th ed. Philadelphia: Saunders; 2005. p. 419-30. 10. Stanley JC, Rhodes EL, Gewertz BL, Chang CY, Walter JF, Fry WJ. Renal artery aneurysms. Significance of macroaneurysms exclusive of dissections and fibrodysplastic mural dilations. Arch Surg 1975;110: 1327-33. 11. Calligaro KD, Dougherty MJ. Renal artery aneurysms and arteriovenous fistulae. In: Rutherford RB, editor. Vascular surgery. 6th ed. Philadelphia: Saunders; 2005. p. 1861-70. 12. Dzsinich C, Gloviczki P, McKusick MA, Pairolero PC, Bower TC, Hallett JW Jr, et al. Surgical management of renal artery aneurysm. Cardiovasc Surg 1993;1:243-7. 13. English WP, Pearce JD, Craven TE, Wilson DB, Edwards MS, Ayerdi J, et al. Surgical management of renal artery aneurysms. J Vasc Surg 2004;40:53-60. 14. Landry GJ, Edwards JM. Nonatherosclerotic vascular disease. In: Moore WS, editor. Vascular and endovascular surgery: a comprehensive review. 7th ed. Philadelphia: Saunders; 2006. p. 107-40. 15. Hachiya J. Current concept of Takayasu’s arteritis. Semin Roentgenol 1970;5:245-59. 16. Hall S, Barr W, Lie JT, Stanson AW, Kazmier FJ, Hunder GG. Takayasu arteritis. A study of 32 North American patients. Medicine (Baltimore) 1985;64:89-99. 17. Miyata T, Sato O, Koyama H, Shigematsu H, Tada Y. Long-term survival after surgical treatment of patients with Takayasu’s arteritis. Circulation 2003;108:1474-80.
Submitted Apr 24, 2006; accepted Jul 6, 2006.
Takayasu arteritis (TA), which was first described in 1908 by a Japanese ophthalmologist,1 is an uncommon inflammatory arteritis that leads to occlusion or ectatic changes in the aorta and its main branches. Most patients are Asian, and the reported incidence in Japan (on the basis of autopsy studies) is 1 case per 3000 people.2 The presence of TA may be suggested by clinical manifestations of aortitis such as neck pain or a systemic inflammation of unknown origin indicated by an increased C-reactive protein concentration or erythrocyte sedimentation rate. The diagnosis is usually based on the results of a magnetic resonance angiography, computed tomography, or digital subtraction angiography examination. Several sets of criteria for the definitive diagnosis of TA are used, including those of Ishikawa,3 the American College of Rheumatology,4 and Sharma et al.5 Most patients with TA have stenotic or occlusive arterial changes. Aneurysmal degeneration is relatively rare, occurring in perhaps 10% to 30% of cases6-8; the actual frequency is unknown. When aneurysmal changes do develop, the aorta is the vessel most often affected, although degeneration of the arch branches of the aorta and the iliac and visceral vessels has been reported.9 We here report a large (7-cm) fusiform renal artery aneurysm (RAA) in a patient with TA. CASE REPORT The patient initially presented with malaise when he was 33 years old. Because leukocytosis and elevation of C-reactive protein and erythrocyte sedimentation rate values were observed, periodic follow-up was performed. When the patient was 40 years old, hypertension and headache developed, and digital subtraction angiography showed irregular, dilated bilateral subclavian arteries and thoracoabdominal aorta, a dilated left renal artery, and partial infarction of the left kidney. A diagnosis of TA was made, and treatment with prednisolone (30 mg/d) was begun immediately. IntermitFrom the Department of Surgerya and the Division of Diagnostic Pathology,b Keio University School of Medicine. Competition of interest: none. Reprint requests: Kenji Matsumoto, MD, PhD, Department of Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan (e-mail: [email protected]). 0741-5214/$32.00 Copyright © 2006 by The Society for Vascular Surgery. doi:10.1016/j.jvs.2006.07.016
tent claudication of the left leg appeared when the patient was 45 years old, and digital subtraction angiography showed aneurysmal dilatation of the left external iliac artery. Conservative follow-up continued, and 2 years later, the patient’s clinical symptoms had disappeared and complete thrombotic occlusion of the aneurysm at the left external iliac artery and development of collateral vessels was observed. The dose of prednisolone was gradually tapered in accordance with improvements in C-reactive protein and erythrocyte sedimentation rate values. The patient stopped taking prednisolone at the age of 51 years, and neither changes in laboratory values nor symptom developments occurred. Subsequently, at the age of 57 years, the patient underwent an abdominal ultrasonographic examination because of an increase in serum alkaline phosphatase. The assessment incidentally revealed a 7-cm fusiform aneurysm in the left renal artery. Abdominal computed tomography imaging (Fig 1) showed that the lesion extended from the origin of the left renal artery to the hilum of the left kidney. It also revealed a 4.2-cm dilatation of the suprarenal abdominal aorta. No remarkable changes in the patient’s laboratory values or symptoms were observed. The discovery of the large RAA was incidental because until the increase in alkaline phosphatase occurred, the results of the patient’s blood tests had been within normal limits and he had no symptoms; thus, a radiologic evaluation had not been considered necessary. The patient underwent transabdominal exploratory laparotomy through a median incision, a procedure customarily used at our institution. The large aneurysm was found to have pushed the left kidney toward the lower part of the pelvis. Nephrectomy was considered necessary because the left kidney had atrophic changes and the aneurysm affected the entire length of the left renal artery. Because the RAA was very large and at high risk of rupture with operative manipulation, resection of this lesion was given precedence over resection of the left kidney. Thus, the left renal vein was cut, and the RAA was resected. The left kidney was then removed. Histologic studies of the wall of the resected RAA (Fig 2) showed severe fibrotic changes and marked infiltration of lymphocytes into the outer part of the media. Degeneration had resulted in an almost complete absence of the media, and the vasa vasorum showed intimal prolif1107
JOURNAL OF VASCULAR SURGERY November 2006
1108 Matsubara et al
DISCUSSION
Fig 1. Abdominal and three-dimensional computerized tomography (CT) images. A, An abdominal CT scan shows a 7.0-cm fusiform aneurysm in the left renal artery. B, A three-dimensional CT scan shows that the aneurysm extends from the origin of the left renal artery to the hilum of the left kidney. The suprarenal abdominal aorta has a 4.2-cm dilatation.
eration and marked luminal narrowing. These findings were compatible with the scar stage of TA. The patient recovered uneventfully and was discharged on the 13th postoperative day in good condition. The patient’s long hospitalization was due to lack of a home caregiver. A magnetic resonance angiography image obtained before discharge showed a normal postoperative appearance. Eight months after surgery, another magnetic resonance angiography study showed no signs of recurrence of the aneurysm (Fig 3), and a clinical evaluation revealed no hypertension or other symptoms.
RAAs are uncommon, occurring in only approximately 0.09% of the general population.10 Most cases are due to degeneration of the renal artery caused by atherosclerosis, fibromuscular dysplasia, inflammation with arteritis, a congenital defect, trauma, or iatrogenic complications after kidney puncture.11 Intraparenchymal aneurysms of the kidney are frequently caused by arteritis disorders such as periarteritis nodosa.11 We found one previous report of an RAA associated with TA.6 However, our case is apparently the first in which a large RAA was found during follow-up in a patient with TA, and definitive histologic characteristics of TA were found in resected specimens of the aneurysm wall. Most RAAs are 1.7 to 2.6 cm when initially observed and managed; lesions larger than 5.0 cm on initial observation are extremely rare.12,13 Surgical treatment is generally performed only for RAAs larger than 2 cm. However, this practice may not be optimal, because rupture of RAAs smaller than 2.0 cm has been reported.13 Thus, the indications for surgical treatment remain controversial. TA has two recognized stages. The first is characterized by fever, myalgia, and anorexia in approximately two thirds of patients. In the second stage, these symptoms may be followed by multiple arterial occlusive symptoms, with the specific manifestations dependent on disease location.14 In patients given a definitive diagnosis of TA, prompt initiation of steroid therapy should be considered to suppress inflammatory development. Aneurysmal changes are uncommon in TA. In reviewing 16 reported series that included a total of 726 patients with TA, Sharma et al8 found that aneurysmal formation had occurred in 67 patients, an overall incidence of 9.2%. Most patients with TA have a favorable prognosis because the disease progresses slowly and because disease processes can be inactivated if adequate treatment is provided. Patients with TA in whom aneurysms are discovered, however, should be considered to have a serious, life-threatening disorder.15 Moreover, long-term steroid therapy for TA may result in weakening of arterial walls and an increase in the risk of aneurysm rupture. Hall et al16 reported the rupture of a thoracic aortic aneurysm in a patient with TA that occurred 3 weeks after steroid therapy was begun. Our patient did undergo long-term steroid therapy, but we do not think that this was responsible for his RAA because histologic studies of the wall of the resected lesion showed typical TA characteristics. Even after surgical treatment, patients with TA require periodic, long-term observation. For example, our patient must be closely observed for formation of an aneurysm at the stump of the left renal artery. A serious, unavoidable complication that commonly develops over the long term in patients with TA who undergo prosthetic graft interposition is anastomotic aneurysm at the interface between the native vessel and prosthetic graft.17 Because TA weakens the arterial wall, patients with the disease who have undergone aneurysm resection should always be considered at
JOURNAL OF VASCULAR SURGERY Volume 44, Number 5
Matsubara et al 1109
Fig 2. Histologic sections of the renal artery aneurysm show severe fibrotic changes and marked infiltration of lymphocytes into the outer part of the media. A, Stain, hematoxylin-eosin; original magnification, ⫻15. B, Stain, Elastica van Gieson; original magnification, ⫻15.
Fig 3. Magnetic resonance angiogram at 8 months after resection of the left renal artery aneurysm and left kidney shows a normal postoperative appearance.
risk of new aneurysm development and rupture and are thus in need of careful observation and, perhaps, early additional surgery. REFERENCES 1. Ito I. Aortitis syndrome (Takayasu’s arteritis). A historical perspective. Jpn Heart J 1995;36:273-81. 2. Nasu T. Takayasu’s truncoarteritis in Japan. A statistical observation of 76 autopsy cases. Pathol Microbiol (Basel) 1975;43:140-6. 3. Ishikawa K. Diagnostic approach and proposed criteria for the clinical diagnosis of Takayasu’s arteriopathy. J Am Coll Cardiol 1988;12:964-72.
4. Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990;33:1129-34. 5. Sharma BK, Siveski-Iliskovic N, Singal PK. Takayasu arteritis may be underdiagnosed in North America. Can J Cardiol 1995;11:311-6. 6. Matsumura K, Hirano T, Takeda K, Matsuda A, Nakagawa T, Yamaguchi N, et al. Incidence of aneurysms in Takayasu’s arteritis. Angiology 1991; 42:308-15. 7. Yamato M, Lecky JW, Hiramatsu K, Kohda E. Takayasu arteritis: radiographic and angiographic findings in 59 patients. Radiology 1986; 161:329-34. 8. Sharma S, Rajani M, Kamalakar T, Kumar A, Talwar KK. The association between aneurysm formation and systemic hypertension in Takayasu’s arteritis. Clin Radiol 1990;42:182-7. 9. Rigberg DA, Quinones-Baldrich W. Takayasu’s disease: nonspecific aortoarteritis. In: Rutherford RB, editor. Vascular surgery. 6th ed. Philadelphia: Saunders; 2005. p. 419-30. 10. Stanley JC, Rhodes EL, Gewertz BL, Chang CY, Walter JF, Fry WJ. Renal artery aneurysms. Significance of macroaneurysms exclusive of dissections and fibrodysplastic mural dilations. Arch Surg 1975;110: 1327-33. 11. Calligaro KD, Dougherty MJ. Renal artery aneurysms and arteriovenous fistulae. In: Rutherford RB, editor. Vascular surgery. 6th ed. Philadelphia: Saunders; 2005. p. 1861-70. 12. Dzsinich C, Gloviczki P, McKusick MA, Pairolero PC, Bower TC, Hallett JW Jr, et al. Surgical management of renal artery aneurysm. Cardiovasc Surg 1993;1:243-7. 13. English WP, Pearce JD, Craven TE, Wilson DB, Edwards MS, Ayerdi J, et al. Surgical management of renal artery aneurysms. J Vasc Surg 2004;40:53-60. 14. Landry GJ, Edwards JM. Nonatherosclerotic vascular disease. In: Moore WS, editor. Vascular and endovascular surgery: a comprehensive review. 7th ed. Philadelphia: Saunders; 2006. p. 107-40. 15. Hachiya J. Current concept of Takayasu’s arteritis. Semin Roentgenol 1970;5:245-59. 16. Hall S, Barr W, Lie JT, Stanson AW, Kazmier FJ, Hunder GG. Takayasu arteritis. A study of 32 North American patients. Medicine (Baltimore) 1985;64:89-99. 17. Miyata T, Sato O, Koyama H, Shigematsu H, Tada Y. Long-term survival after surgical treatment of patients with Takayasu’s arteritis. Circulation 2003;108:1474-80.
Submitted Apr 24, 2006; accepted Jul 6, 2006.