Case Report
Inflammatory Abdominal Aortic Aneurysm: A Case Report and Review of Literature
Vascular and Endovascular Surgery 2014, Vol 48(1) 65-69 ª The Author(s) 2013 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/1538574413510616 ves.sagepub.com
Siva S. Ketha, MD1, Kenneth J. Warrington, MD2, and Ian R. McPhail, MD1
Abstract We report a case of an abdominal aortic aneurysm (AAA) that underwent inflammatory transformation which we treated medically with corticosteroids. Medical therapy resulted in resolution of presenting symptoms and observed inflammatory changes. We review the clinical features, associated pathology, diagnostic, and therapeutic options in the management of inflammatory AAA. Keywords inflammatory abdominal aortic aneurysm, IgG4-related disease
Case Report A 60-year-old man was referred to the vascular clinic for evaluation of an abdominal aortic aneurysm (AAA) that was incidentally noted on imaging performed for evaluation of worsening back pain. He had a long-standing (several year) history of low back pain, generally controlled with as needed use of ibuprofen. However, approximately 2 months prior to this presentation, the back pain worsened significantly. He was evaluated at another institution and treated with opioid analgesics, muscle relaxants, and an epidural steroid injection with limited relief. His local care provider also treated him with short courses of tapering steroids on 2 occasions with dramatic improvement in his symptoms. However, his pain recurred during prednisone reduction and intensified further after cessation of therapy. He was therefore referred to our institution for evaluation of his back pain by neurosurgery. A noncontrast computed tomography (CT) scan of the lumbar spine was performed, and a diagnosis of L5-S1 spondylolisthesis was made. This study incidentally revealed an AAA, and he was therefore referred to the vascular clinic. Of note, a CT urogram done at another institution (Figure 1), 6 months prior to this presentation for a self-limited episode of painless hematuria showed a 4-cm infrarenal AAA with no visible inflammatory changes. In addition to the worsening back pain, he also reported intermittent, drenching night sweats, and a 24-pound weight loss during the past 2 months but was otherwise asymptomatic. His medical history was significant for hyperlipidemia, controlled with simvastatin, and a 30 pack-year smoking history. Family history was significant for an AAA in his mother. Physical examination was unremarkable. He subsequently underwent CT angiography of the abdomen and pelvis
(Figure 2A and B), which revealed an infrarenal AAA measuring approximately 4.2 3.9 cm2 with a soft tissue inflammatory rind measuring 1.4 cm in thickness. Laboratory evaluation revealed a normal complete blood count, creatinine, urinalysis, and erythrocyte sedimentation rate. C-reactive protein (CRP) was elevated at 17.7 mg/L (reference range 40% and >50 IgG4 plasma cells per high power field.25,26 Recent studies have shown an increase in the
Ketha et al
67
fraction of IgG4-producing plasma cells among the inflammatory constituents of IAAA, similar to autoimmune pancreatitis.27,28 Therefore, it is possible that our patient may in fact have IgG4-RD despite normal serum IgG-4 levels.
Diagnosis Computed tomography scan with contrast enhancement is a highly reliable imaging modality for the diagnosis of IAAA.29 Magnetic resonance angiography is also useful for diagnosis and follow-up. Once a diagnosis of IAAA is made, it is important to obtain imaging of the entire aorta with an appropriate modality like CT or magnetic resonance angiography to rule out inflammatory changes in the thoracic aorta. Positron emission tomography scanning has recently emerged as an excellent modality for targeted imaging of vascular inflammation, especially in CP.30
Medical Management Aneurysms less than 5.5.cm in diameter do not generally require surgical intervention as survival is not improved by elective repair of such AAAs, even when operative mortality is low.31 Conservative management with periodic surveillance is indicated until the aneurysm size reaches the size threshold to warrant surgical intervention.32 Because the bulk of evidence points toward IAAA resulting from a local inflammatory reaction to atherosclerotic lipids, minimizing atherosclerotic risk factors with smoking cessation, optimal control of hypertension, and hyperlipidemia may play an important role in medical management.33 Since inflammation is a key component of an IAAA, corticosteroids or other anti-inflammatory, immunosuppressive therapies have been recommended in patients with symptomatic IAAA in whom the aortic diameter does not warrant surgical repair.34 Improvement in symptoms, signs, and resolution of inflammatory changes in imaging has been described with steroid therapy. Corticosteroid sparing agents, such as methotrexate, cyclophosphamide, and azathioprine, have also been used, although no consensus or guidelines for medical therapy exist currently.14 Identifying the IgG4 subset of patients with IAAA may have therapeutic implications because of the frequent good response of IgG4-related systemic disease to glucocorticoid treatment without additional therapy and because treatment of aortitis may prevent progression of disease to other organs.23 Occasionally these patients may require prolonged treatment with glucocorticoids and are unable to taper these medications. Immunosuppressive agents like azathioprine and methotrexate have been used as second-line agents, and there have been reports of successful treatment with rituximab.35
Surgical Management The aim of surgery in AAA is to prevent rupture, and repair is usually indicated when the aortic diameter exceeds 5.5
Figure 3. A, Computed tomography (CT) angiography of the abdomen and pelvis after 2 months of oral corticosteroid therapy showing significant reduction in the size of the inflammatory soft tissue component surrounding the abdominal aortic aneurysm. B, CT angiography of the abdomen and pelvis (sagittal view) showing reduction in the size of the inflammatory soft tissue component surrounding the abdominal aortic aneurysm.
cm.31,32 Open surgery with excision of the aneurysm and graft placement has been the traditional approach in the management of IAAA, although endovascular repair is being increasingly performed.36-38 Endovascular repair, although appropriate when the anatomical features are conducive, is associated with lower rates of resolution of the inflammatory process.36-38 Open surgery is technically challenging, because IAAAs are usually associated with dense adhesions that surround the aneurysm and frequently involve the duodenum (97%-100%), inferior vena cava (63%-70%), ureters (20%-44%), and left renal vein (48%51%).6 Despite initial technical difficulties and associated increase in morbidity and mortality when compared to open surgery for noninflammatory AAA, surgical results have greatly improved over time with little difference in mortality between non-IAAA and IAAA repair. Open surgery with grafting was once thought to resolve symptoms and inflammation, but studies have shown that periaortic inflammation may persist after surgery.39 Steroid therapy seems to be effective if used preoperatively causing a reduction in the size of the inflammatory rind of the AAA thus facilitating surgery. Steroids also have a role to play in preventing disease progression and relapse when used after surgery.34
Case Follow-Up At the 2-month follow-up, patient’s presenting symptoms resolved completely. Laboratory testing revealed normalization of CRP (3 mg/L). Repeat CT angiography of the abdomen and pelvis (Figure 3A and B) showed that the aneurysm itself was unchanged in size at 4.2 cm in anteroposterior diameter. The inflammatory soft tissue component surrounding the aneurysm decreased in size significantly, and slow taper of corticosteroid therapy was recommended.
68 Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Pennell RC, Hollier LH, Lie JT, et al. Inflammatory abdominal aortic aneurysms: a thirty-year review. J Vasc Surg. 1985;2(6): 859-869. 2. Crawford JL, Stowe CL, Safi HJ, Hallman CH, Crawford ES. Inflammatory aneurysms of the aorta. J Vasc Surg. 1985;2(1): 113-124. 3. Sterpetti AV, Hunter WJ, Feldhaus RJ, et al. Inflammatory aneurysms of the abdominal aorta: incidence, pathologic, and etiologic considerations. J Vasc Surg. 1989;9(5):643-649. 4. Hill J, Charlesworth D. Inflammatory abdominal aortic aneurysms: a report of thirty-seven cases. Ann Vasc Surg. 1988;2(4): 352-327. 5. Boontje AH, van den Dungen JJ, Blanksma C. Inflammatory abdominal aortic aneurysms. J Cardiovasc Surg (Torino). 1990; 31(5):611-616. 6. Rasmussen TE, Hallett JW Jr. Inflammatory aortic aneurysms. a clinical review with new perspectives in pathogenesis. Ann Surg. 1997;225(2):155-164. 7. Walker DI, Bloor K, Williams G, Gillie I. Inflammatory aneurysms of the abdominal aorta. Br J Surg. 1972;59(8):609-614. 8. Nitecki SS, Hallett JW Jr., Stanson AW, et al. Inflammatory abdominal aortic aneurysms: a case–control study. J Vasc Surg. 1996;23(5):860-868; discussion 68-9. 9. Mitchinson MJ. Chronic periaortitis and periarteritis. Histopathology. 1984;8(4):589-600. 10. van Bommel EF. Retroperitoneal fibrosis. Neth J Med. 2002; 60(6):231-242. 11. Parums DV. The spectrum of chronic periaortitis. Histopathology. 1990;16(5):423-431. 12. Rasmussen TE, Hallett JW Jr. New insights into inflammatory abdominal aortic aneurysms. Eur J Vasc Endovasc Surg. 1997; 14(5):329-332. 13. Parums DV, Brown DL, Mitchinson MJ. Serum antibodies to oxidized low-density lipoprotein and ceroid in chronic periaortitis. Arch Pathol Lab Med. 1990;114(4):383-387. 14. Vaglio A, Buzio C. Chronic periaortitis: a spectrum of diseases. Curr Opin Rheumatol. 2005;17(1):34-40. 15. Haug ES, Skomsvoll JF, Jacobsen G, Halvorsen TB, Saether OD, Myhre HO. Inflammatory aortic aneurysm is associated with increased incidence of autoimmune disease. J Vasc Surg. 2003; 38(3):492-497. 16. Vaglio A, Pipitone N, Salvarani C. Chronic periaortitis: a largevessel vasculitis? Curr Opin Rheumatol. 2011;23(1):1-6. 17. Karlsson L, Gnarpe J, Naas J, et al. Detection of viable Chlamydia pneumoniae in abdominal aortic aneurysms. Eur J Vasc Endovasc Surg. 2000;19(6):630-635.
Vascular and Endovascular Surgery 48(1) 18. Tanaka S, Komori K, Okadome K, Sugimachi K, Mori R. Detection of active cytomegalovirus infection in inflammatory aortic aneurysms with RNA polymerase chain reaction. J Vasc Surg. 1994;20(2):235-243. 19. Miller DV, Maleszewski JJ. The pathology of large-vessel vasculitides. Clin Exp Rheumatol. 2011;29:S92-S98. 20. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539-551. 21. Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344(10):732-738. 22. Saeki T, Saito A, Hiura T, et al. Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4related systemic disease. Intern Med. 2006;45(3):163-167. 23. Stone JH, Khosroshahi A, Hilgenberg A, Spooner A, Isselbacher EM, Stone JR. IgG4-related systemic disease and lymphoplasmacytic aortitis. Arthritis Rheum. 2009;60(10):3139-3145. 24. Dahlgren M, Khosroshahi A, Nielsen GP, Deshpande V, Stone JH. Riedel’s thyroiditis and multifocal fibrosclerosis are part of the IgG4-related systemic disease spectrum. Arthritis Care Res (Hoboken). 2010;62(9):1312-1318. 25. Cheuk W, Yuen HK, Chu SY, Chiu EK, Lam LK, Chan JK. Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol. 2008;32(5):671-681. 26. Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010;17(5):303-332. 27. Kasashima S, Zen Y, Kawashima A, et al. Inflammatory abdominal aortic aneurysm: close relationship to IgG4-related periaortitis. Am J Surg Pathol. 2008;32(2):197-204. 28. Stone JR. Aortitis, periaortitis, and retroperitoneal fibrosis, as manifestations of IgG4-related systemic disease. Curr Opin Rheumatol. 2011;23(1):88-94. 29. Iino M, Kuribayashi S, Imakita S, et al. Sensitivity and specificity of CT in the diagnosis of inflammatory abdominal aortic aneurysms. J Comput Assist Tomogr. 2002;26(6):1006-1012. 30. Salvarani C, Pipitone N, Versari A, et al. Positron emission tomography (PET): evaluation of chronic periaortitis. Arthritis Rheum 2005;53(2):298-303. 31. Lederle FA, Wilson SE, Johnson GR, et al. Immediate repair compared with surveillance of small abdominal aortic aneurysms. N Engl J Med. 2002;346(19):1437-1444. 32. Mortality results for randomised controlled trial of early elective surgery or ultrasonographic surveillance for small abdominal aortic aneurysms. the UK small aneurysm trial participants. Lancet. 1998;352(9141):1649-1655. 33. Hellmann DB, Grand DJ, Freischlag JA. Inflammatory abdominal aortic aneurysm. JAMA. 2007;297(4):395-400. 34. Palmisano A, Vaglio A. Chronic periaortitis: a fibroinflammatory disorder. Best Pract Res Clin Rheumatol. 2009; 23(3):339-353. 35. Khosroshahi A, Carruthers MN, Deshpande V, Unizony S, Bloch DB, Stone JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore). 2012;91(1):57-66.
Ketha et al 36. Lange C, Hobo R, Leurs LJ, Daenens K, Buth J, Myhre HO. Results of endovascular repair of inflammatory abdominal aortic aneurysms. A report from the EUROSTAR database. Eur J Vasc Endovasc Surg. 2005;29(4):363-370. 37. Coppi G, Rametta F, Aiello S, Saitta G, Gennai S, Silingardi R. Inflammatory abdominal aortic aneurysm endovascular repair
69 into the long-term follow-up. Ann Vasc Surg. 2010;24(8): 1053-1059. 38. Stone WM, Fankhauser GT. Inflammatory aneurysms treated with EVAR. Semin Vasc Surg. 2012;25(4):227-231. 39. Tang T, Boyle JR, Dixon AK, Varty K. Inflammatory abdominal aortic aneurysms. Eur J Vasc Endovasc Surg. 2005;29(4):353-362.
Inflammatory Abdominal Aortic Aneurysm: A Case Report and Review of Literature
Vascular and Endovascular Surgery 2014, Vol 48(1) 65-69 ª The Author(s) 2013 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/1538574413510616 ves.sagepub.com
Siva S. Ketha, MD1, Kenneth J. Warrington, MD2, and Ian R. McPhail, MD1
Abstract We report a case of an abdominal aortic aneurysm (AAA) that underwent inflammatory transformation which we treated medically with corticosteroids. Medical therapy resulted in resolution of presenting symptoms and observed inflammatory changes. We review the clinical features, associated pathology, diagnostic, and therapeutic options in the management of inflammatory AAA. Keywords inflammatory abdominal aortic aneurysm, IgG4-related disease
Case Report A 60-year-old man was referred to the vascular clinic for evaluation of an abdominal aortic aneurysm (AAA) that was incidentally noted on imaging performed for evaluation of worsening back pain. He had a long-standing (several year) history of low back pain, generally controlled with as needed use of ibuprofen. However, approximately 2 months prior to this presentation, the back pain worsened significantly. He was evaluated at another institution and treated with opioid analgesics, muscle relaxants, and an epidural steroid injection with limited relief. His local care provider also treated him with short courses of tapering steroids on 2 occasions with dramatic improvement in his symptoms. However, his pain recurred during prednisone reduction and intensified further after cessation of therapy. He was therefore referred to our institution for evaluation of his back pain by neurosurgery. A noncontrast computed tomography (CT) scan of the lumbar spine was performed, and a diagnosis of L5-S1 spondylolisthesis was made. This study incidentally revealed an AAA, and he was therefore referred to the vascular clinic. Of note, a CT urogram done at another institution (Figure 1), 6 months prior to this presentation for a self-limited episode of painless hematuria showed a 4-cm infrarenal AAA with no visible inflammatory changes. In addition to the worsening back pain, he also reported intermittent, drenching night sweats, and a 24-pound weight loss during the past 2 months but was otherwise asymptomatic. His medical history was significant for hyperlipidemia, controlled with simvastatin, and a 30 pack-year smoking history. Family history was significant for an AAA in his mother. Physical examination was unremarkable. He subsequently underwent CT angiography of the abdomen and pelvis
(Figure 2A and B), which revealed an infrarenal AAA measuring approximately 4.2 3.9 cm2 with a soft tissue inflammatory rind measuring 1.4 cm in thickness. Laboratory evaluation revealed a normal complete blood count, creatinine, urinalysis, and erythrocyte sedimentation rate. C-reactive protein (CRP) was elevated at 17.7 mg/L (reference range 40% and >50 IgG4 plasma cells per high power field.25,26 Recent studies have shown an increase in the
Ketha et al
67
fraction of IgG4-producing plasma cells among the inflammatory constituents of IAAA, similar to autoimmune pancreatitis.27,28 Therefore, it is possible that our patient may in fact have IgG4-RD despite normal serum IgG-4 levels.
Diagnosis Computed tomography scan with contrast enhancement is a highly reliable imaging modality for the diagnosis of IAAA.29 Magnetic resonance angiography is also useful for diagnosis and follow-up. Once a diagnosis of IAAA is made, it is important to obtain imaging of the entire aorta with an appropriate modality like CT or magnetic resonance angiography to rule out inflammatory changes in the thoracic aorta. Positron emission tomography scanning has recently emerged as an excellent modality for targeted imaging of vascular inflammation, especially in CP.30
Medical Management Aneurysms less than 5.5.cm in diameter do not generally require surgical intervention as survival is not improved by elective repair of such AAAs, even when operative mortality is low.31 Conservative management with periodic surveillance is indicated until the aneurysm size reaches the size threshold to warrant surgical intervention.32 Because the bulk of evidence points toward IAAA resulting from a local inflammatory reaction to atherosclerotic lipids, minimizing atherosclerotic risk factors with smoking cessation, optimal control of hypertension, and hyperlipidemia may play an important role in medical management.33 Since inflammation is a key component of an IAAA, corticosteroids or other anti-inflammatory, immunosuppressive therapies have been recommended in patients with symptomatic IAAA in whom the aortic diameter does not warrant surgical repair.34 Improvement in symptoms, signs, and resolution of inflammatory changes in imaging has been described with steroid therapy. Corticosteroid sparing agents, such as methotrexate, cyclophosphamide, and azathioprine, have also been used, although no consensus or guidelines for medical therapy exist currently.14 Identifying the IgG4 subset of patients with IAAA may have therapeutic implications because of the frequent good response of IgG4-related systemic disease to glucocorticoid treatment without additional therapy and because treatment of aortitis may prevent progression of disease to other organs.23 Occasionally these patients may require prolonged treatment with glucocorticoids and are unable to taper these medications. Immunosuppressive agents like azathioprine and methotrexate have been used as second-line agents, and there have been reports of successful treatment with rituximab.35
Surgical Management The aim of surgery in AAA is to prevent rupture, and repair is usually indicated when the aortic diameter exceeds 5.5
Figure 3. A, Computed tomography (CT) angiography of the abdomen and pelvis after 2 months of oral corticosteroid therapy showing significant reduction in the size of the inflammatory soft tissue component surrounding the abdominal aortic aneurysm. B, CT angiography of the abdomen and pelvis (sagittal view) showing reduction in the size of the inflammatory soft tissue component surrounding the abdominal aortic aneurysm.
cm.31,32 Open surgery with excision of the aneurysm and graft placement has been the traditional approach in the management of IAAA, although endovascular repair is being increasingly performed.36-38 Endovascular repair, although appropriate when the anatomical features are conducive, is associated with lower rates of resolution of the inflammatory process.36-38 Open surgery is technically challenging, because IAAAs are usually associated with dense adhesions that surround the aneurysm and frequently involve the duodenum (97%-100%), inferior vena cava (63%-70%), ureters (20%-44%), and left renal vein (48%51%).6 Despite initial technical difficulties and associated increase in morbidity and mortality when compared to open surgery for noninflammatory AAA, surgical results have greatly improved over time with little difference in mortality between non-IAAA and IAAA repair. Open surgery with grafting was once thought to resolve symptoms and inflammation, but studies have shown that periaortic inflammation may persist after surgery.39 Steroid therapy seems to be effective if used preoperatively causing a reduction in the size of the inflammatory rind of the AAA thus facilitating surgery. Steroids also have a role to play in preventing disease progression and relapse when used after surgery.34
Case Follow-Up At the 2-month follow-up, patient’s presenting symptoms resolved completely. Laboratory testing revealed normalization of CRP (3 mg/L). Repeat CT angiography of the abdomen and pelvis (Figure 3A and B) showed that the aneurysm itself was unchanged in size at 4.2 cm in anteroposterior diameter. The inflammatory soft tissue component surrounding the aneurysm decreased in size significantly, and slow taper of corticosteroid therapy was recommended.
68 Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Pennell RC, Hollier LH, Lie JT, et al. Inflammatory abdominal aortic aneurysms: a thirty-year review. J Vasc Surg. 1985;2(6): 859-869. 2. Crawford JL, Stowe CL, Safi HJ, Hallman CH, Crawford ES. Inflammatory aneurysms of the aorta. J Vasc Surg. 1985;2(1): 113-124. 3. Sterpetti AV, Hunter WJ, Feldhaus RJ, et al. Inflammatory aneurysms of the abdominal aorta: incidence, pathologic, and etiologic considerations. J Vasc Surg. 1989;9(5):643-649. 4. Hill J, Charlesworth D. Inflammatory abdominal aortic aneurysms: a report of thirty-seven cases. Ann Vasc Surg. 1988;2(4): 352-327. 5. Boontje AH, van den Dungen JJ, Blanksma C. Inflammatory abdominal aortic aneurysms. J Cardiovasc Surg (Torino). 1990; 31(5):611-616. 6. Rasmussen TE, Hallett JW Jr. Inflammatory aortic aneurysms. a clinical review with new perspectives in pathogenesis. Ann Surg. 1997;225(2):155-164. 7. Walker DI, Bloor K, Williams G, Gillie I. Inflammatory aneurysms of the abdominal aorta. Br J Surg. 1972;59(8):609-614. 8. Nitecki SS, Hallett JW Jr., Stanson AW, et al. Inflammatory abdominal aortic aneurysms: a case–control study. J Vasc Surg. 1996;23(5):860-868; discussion 68-9. 9. Mitchinson MJ. Chronic periaortitis and periarteritis. Histopathology. 1984;8(4):589-600. 10. van Bommel EF. Retroperitoneal fibrosis. Neth J Med. 2002; 60(6):231-242. 11. Parums DV. The spectrum of chronic periaortitis. Histopathology. 1990;16(5):423-431. 12. Rasmussen TE, Hallett JW Jr. New insights into inflammatory abdominal aortic aneurysms. Eur J Vasc Endovasc Surg. 1997; 14(5):329-332. 13. Parums DV, Brown DL, Mitchinson MJ. Serum antibodies to oxidized low-density lipoprotein and ceroid in chronic periaortitis. Arch Pathol Lab Med. 1990;114(4):383-387. 14. Vaglio A, Buzio C. Chronic periaortitis: a spectrum of diseases. Curr Opin Rheumatol. 2005;17(1):34-40. 15. Haug ES, Skomsvoll JF, Jacobsen G, Halvorsen TB, Saether OD, Myhre HO. Inflammatory aortic aneurysm is associated with increased incidence of autoimmune disease. J Vasc Surg. 2003; 38(3):492-497. 16. Vaglio A, Pipitone N, Salvarani C. Chronic periaortitis: a largevessel vasculitis? Curr Opin Rheumatol. 2011;23(1):1-6. 17. Karlsson L, Gnarpe J, Naas J, et al. Detection of viable Chlamydia pneumoniae in abdominal aortic aneurysms. Eur J Vasc Endovasc Surg. 2000;19(6):630-635.
Vascular and Endovascular Surgery 48(1) 18. Tanaka S, Komori K, Okadome K, Sugimachi K, Mori R. Detection of active cytomegalovirus infection in inflammatory aortic aneurysms with RNA polymerase chain reaction. J Vasc Surg. 1994;20(2):235-243. 19. Miller DV, Maleszewski JJ. The pathology of large-vessel vasculitides. Clin Exp Rheumatol. 2011;29:S92-S98. 20. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539-551. 21. Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344(10):732-738. 22. Saeki T, Saito A, Hiura T, et al. Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4related systemic disease. Intern Med. 2006;45(3):163-167. 23. Stone JH, Khosroshahi A, Hilgenberg A, Spooner A, Isselbacher EM, Stone JR. IgG4-related systemic disease and lymphoplasmacytic aortitis. Arthritis Rheum. 2009;60(10):3139-3145. 24. Dahlgren M, Khosroshahi A, Nielsen GP, Deshpande V, Stone JH. Riedel’s thyroiditis and multifocal fibrosclerosis are part of the IgG4-related systemic disease spectrum. Arthritis Care Res (Hoboken). 2010;62(9):1312-1318. 25. Cheuk W, Yuen HK, Chu SY, Chiu EK, Lam LK, Chan JK. Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol. 2008;32(5):671-681. 26. Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010;17(5):303-332. 27. Kasashima S, Zen Y, Kawashima A, et al. Inflammatory abdominal aortic aneurysm: close relationship to IgG4-related periaortitis. Am J Surg Pathol. 2008;32(2):197-204. 28. Stone JR. Aortitis, periaortitis, and retroperitoneal fibrosis, as manifestations of IgG4-related systemic disease. Curr Opin Rheumatol. 2011;23(1):88-94. 29. Iino M, Kuribayashi S, Imakita S, et al. Sensitivity and specificity of CT in the diagnosis of inflammatory abdominal aortic aneurysms. J Comput Assist Tomogr. 2002;26(6):1006-1012. 30. Salvarani C, Pipitone N, Versari A, et al. Positron emission tomography (PET): evaluation of chronic periaortitis. Arthritis Rheum 2005;53(2):298-303. 31. Lederle FA, Wilson SE, Johnson GR, et al. Immediate repair compared with surveillance of small abdominal aortic aneurysms. N Engl J Med. 2002;346(19):1437-1444. 32. Mortality results for randomised controlled trial of early elective surgery or ultrasonographic surveillance for small abdominal aortic aneurysms. the UK small aneurysm trial participants. Lancet. 1998;352(9141):1649-1655. 33. Hellmann DB, Grand DJ, Freischlag JA. Inflammatory abdominal aortic aneurysm. JAMA. 2007;297(4):395-400. 34. Palmisano A, Vaglio A. Chronic periaortitis: a fibroinflammatory disorder. Best Pract Res Clin Rheumatol. 2009; 23(3):339-353. 35. Khosroshahi A, Carruthers MN, Deshpande V, Unizony S, Bloch DB, Stone JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore). 2012;91(1):57-66.
Ketha et al 36. Lange C, Hobo R, Leurs LJ, Daenens K, Buth J, Myhre HO. Results of endovascular repair of inflammatory abdominal aortic aneurysms. A report from the EUROSTAR database. Eur J Vasc Endovasc Surg. 2005;29(4):363-370. 37. Coppi G, Rametta F, Aiello S, Saitta G, Gennai S, Silingardi R. Inflammatory abdominal aortic aneurysm endovascular repair
69 into the long-term follow-up. Ann Vasc Surg. 2010;24(8): 1053-1059. 38. Stone WM, Fankhauser GT. Inflammatory aneurysms treated with EVAR. Semin Vasc Surg. 2012;25(4):227-231. 39. Tang T, Boyle JR, Dixon AK, Varty K. Inflammatory abdominal aortic aneurysms. Eur J Vasc Endovasc Surg. 2005;29(4):353-362.
