Answer for BIR CoW 13 April 2025
TYPE V TAKAYASU ARTERITIS
Findings
Evidence of diffuse circumferential wall thickening noted from ascending aorta, arch of aorta, and its branches, extending to descending thoracic aorta, abdominal aorta. Diffuse circumferential wall thickening noted from the origin of bilateral common carotid artery upto carotid bulb. Circumferential wall thickening noted in left subclavian artery causing significant luminal narrowing(>70%) from the mid-subclavian approximately 3.1cm from the origin. The wall thickening extending into left axillary and visualized segment of left brachial artery. Evidence of fusiform aneurysmal dilatation of abdominal aorta with surrounding calcific plaques for a length of 10cm from L1 vertebral body to L3 vertebral body level, with multiple penetrating ulcers. Evidence of short segment stenosis noted in ostio proximal segment of celiac artery and kinking noted in the proximal common hepatic artery. Right kidney appears reduced in size. Left kidney appears normal in size. Bilateral renal artery seen arising from the aneurysmal segment of abdominal aorta Right renal artery appears severely narrowed from its origin Left renal artery shows diffuse wall thickening at its origin and causing in the ostio proximal segment.
Discussion
Takayasu's arteritis (TA) is a necrotizing, obliterative and segmental primary large vessel panarteritis of unknown etiology that affects the aorta and its major branches including the pulmonary artery. It is most common in young Asian women and occurs about 10 times more commonly in women than in men. Etiology: Previous tubercular infection, genetic factors and autoimmune mechanisms. Pathology: TA is a T-cell-mediated pan arterities which begins in adventitial vasa vasorum with inward progression. Destructive perivascular cuffing of vasa vasorum is followed by fibrosis and calcifications and leads to aneurysmal dilatations followed by stenosis of aorta and its branches. Types: Type I : only the branches of the aortic arch involved. Type IIA involves the aorta only at its ascending portion with or without aortic arch. The branches of the aortic arch may be involved as well. Type IIB: affects the descending thoracic aorta with or without involvement of the ascending aorta or the aortic arch with its branches. Abdominal aorta is not involved. Type III : concomitant involvement of the descending thoracic aorta, the abdominal aorta, and/or the renal arteries. The ascending aorta and the aortic arch and its branches are not involved. Type IV : involves only the abdominal aorta and/or the renal arteries. Type V : Generalized type, with combined features of the other types. Note: Involvement of the coronary and pulmonary arteries should be indicated as C(+) or P(+), respectively. The commonly involved arteries are left subclavian artery (50%), left common carotid (20%), brachiocephalic, renal arteries, celiac trunk, SMA and pulmonary arteries (50%). Imaging Findings : USG, CT, MRI and/or PET CT/MRI are the main imaging modalities. USG: long, smooth, homogeneous and moderately echogenic circumferential thickening on transverse section, this is termed the "macaroni sign". vascular occlusion due to intimal thickening and/or secondary thrombus formation aneurysms , loss of pulsatility of the vessel CT/MRI: Early/acute stage: The arterial wall thickening seen as soft tissue thickening encircling the aorta (swollen intima) with circumferential peripheral contrast enhancement (inflamed media and adventitia) On T2-weighted sequences mural edema is depicted as high signal. Nuclear fusion imaging (PET CT / MRI ) is now emerging as the initial diagnosis and assessment of disease activity with high sensitivity and specificity. Chronic stage: Aneurysm and long diffuse or short segmental irregular stenosis. Occlusion of the major branches of the aorta near its origin . Skip lesions, abundant collateralization, saccular or fusiform aortic aneurysm arterial wall thickening, mural calcium deposition and full thickness calcification . Management: Active Stage: Steroids or immunosuppressants before revascularization as revascularization during the active phase can increase the risk of procedure-related complications, including restenosis and dissection. Chronic stage: Surgery or balloon angioplasty. Balloon angioplasty is a relatively simple and cost-effective method for relief of stenotic lesions in patients with TA .The problem with balloon angioplasty is recurrence of the stenosis, which is however, easily tackled by a secondary reintervention.
REFERENCES:
1.Wolfgang Dähnert. Radiology Review Manual. (2011) ISBN: 9781609139438 - Google Books
2. Sueyoshi E, Sakamoto I, Uetani M. MRI of Takayasu's arteritis: typical appearances and complications. AJR Am J Roentgenol. 2006;187 (6): W569-75. doi:10.2214/AJR.05.1093 - Pubmed citation
3.AIIMS-MAMC-PGI's Comprehensive Textbook of Diagnostic Radiology
Findings
Evidence of diffuse circumferential wall thickening noted from ascending aorta, arch of aorta, and its branches, extending to descending thoracic aorta, abdominal aorta. Diffuse circumferential wall thickening noted from the origin of bilateral common carotid artery upto carotid bulb. Circumferential wall thickening noted in left subclavian artery causing significant luminal narrowing(>70%) from the mid-subclavian approximately 3.1cm from the origin. The wall thickening extending into left axillary and visualized segment of left brachial artery. Evidence of fusiform aneurysmal dilatation of abdominal aorta with surrounding calcific plaques for a length of 10cm from L1 vertebral body to L3 vertebral body level, with multiple penetrating ulcers. Evidence of short segment stenosis noted in ostio proximal segment of celiac artery and kinking noted in the proximal common hepatic artery. Right kidney appears reduced in size. Left kidney appears normal in size. Bilateral renal artery seen arising from the aneurysmal segment of abdominal aorta Right renal artery appears severely narrowed from its origin Left renal artery shows diffuse wall thickening at its origin and causing in the ostio proximal segment.
Discussion
Takayasu's arteritis (TA) is a necrotizing, obliterative and segmental primary large vessel panarteritis of unknown etiology that affects the aorta and its major branches including the pulmonary artery. It is most common in young Asian women and occurs about 10 times more commonly in women than in men. Etiology: Previous tubercular infection, genetic factors and autoimmune mechanisms. Pathology: TA is a T-cell-mediated pan arterities which begins in adventitial vasa vasorum with inward progression. Destructive perivascular cuffing of vasa vasorum is followed by fibrosis and calcifications and leads to aneurysmal dilatations followed by stenosis of aorta and its branches. Types: Type I : only the branches of the aortic arch involved. Type IIA involves the aorta only at its ascending portion with or without aortic arch. The branches of the aortic arch may be involved as well. Type IIB: affects the descending thoracic aorta with or without involvement of the ascending aorta or the aortic arch with its branches. Abdominal aorta is not involved. Type III : concomitant involvement of the descending thoracic aorta, the abdominal aorta, and/or the renal arteries. The ascending aorta and the aortic arch and its branches are not involved. Type IV : involves only the abdominal aorta and/or the renal arteries. Type V : Generalized type, with combined features of the other types. Note: Involvement of the coronary and pulmonary arteries should be indicated as C(+) or P(+), respectively. The commonly involved arteries are left subclavian artery (50%), left common carotid (20%), brachiocephalic, renal arteries, celiac trunk, SMA and pulmonary arteries (50%). Imaging Findings : USG, CT, MRI and/or PET CT/MRI are the main imaging modalities. USG: long, smooth, homogeneous and moderately echogenic circumferential thickening on transverse section, this is termed the "macaroni sign". vascular occlusion due to intimal thickening and/or secondary thrombus formation aneurysms , loss of pulsatility of the vessel CT/MRI: Early/acute stage: The arterial wall thickening seen as soft tissue thickening encircling the aorta (swollen intima) with circumferential peripheral contrast enhancement (inflamed media and adventitia) On T2-weighted sequences mural edema is depicted as high signal. Nuclear fusion imaging (PET CT / MRI ) is now emerging as the initial diagnosis and assessment of disease activity with high sensitivity and specificity. Chronic stage: Aneurysm and long diffuse or short segmental irregular stenosis. Occlusion of the major branches of the aorta near its origin . Skip lesions, abundant collateralization, saccular or fusiform aortic aneurysm arterial wall thickening, mural calcium deposition and full thickness calcification . Management: Active Stage: Steroids or immunosuppressants before revascularization as revascularization during the active phase can increase the risk of procedure-related complications, including restenosis and dissection. Chronic stage: Surgery or balloon angioplasty. Balloon angioplasty is a relatively simple and cost-effective method for relief of stenotic lesions in patients with TA .The problem with balloon angioplasty is recurrence of the stenosis, which is however, easily tackled by a secondary reintervention.
REFERENCES:
1.Wolfgang Dähnert. Radiology Review Manual. (2011) ISBN: 9781609139438 - Google Books
2. Sueyoshi E, Sakamoto I, Uetani M. MRI of Takayasu's arteritis: typical appearances and complications. AJR Am J Roentgenol. 2006;187 (6): W569-75. doi:10.2214/AJR.05.1093 - Pubmed citation
3.AIIMS-MAMC-PGI's Comprehensive Textbook of Diagnostic Radiology
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!