Proc. 12th Conf. Int. Soc. Geograph. Path., Zurich, September 1975 Path. Microbiol. 43: 157-162 (73-78) (1975)
Pathological Features of Thromboangiitis obliterans and Arteritis temporalis and Some Unusual Manifestations E. G. J. O lsen Department of Morbid Anatomy and Histopathology. National Heart Hospital, and Cardiothoracic Institute, University of London, London
Key Words. Arteritis • Granulomatous arteritis • Temporal arteritis • Thrombo angiitis obliterans • Buerger’s disease
Thromboangiitis obliterans (Buerger’s disease) Since the original description of this disease of vessels by B u e r g e r in 1908 (and re-issued in 1973 [4]), its existence had from time to time been doubted in the intervening years [3, 9, 23]. Subsequently it has become clear that thromboangiitis obliterans exists [2, 20, 24], which is also the belief of this author. To substantiate this claim, material from 52 cases obtained from the Department of Morbid Anatomy, Royal Postgraduate Medical School, and the National Heart Hospital, London, from 1935 to 1975 was available. 45 males (mean age 41 years) and 7 females (mean age 36 years), mean age for both sexes 37 years, were analysed. The arteries were involved as follows: Lower limb 35 cases, small vessels in toes 9 cases, upper limb vessels 8 cases.
Macroscopy The vessels are segmentally involved and may present as thick fibrous cords. Occasionally thrombus can be discerned with the naked eye. Arteries and veins are not necessarily involved in the same cross section.
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Pathology
158
Ol.SUN
(74)
Fig. I . Photomicrograph of a posterior tibial artery from a man aged 31 suffer ing from thromboangiitis obliterans. The lumen of the vessel is occluded by an organising thrombus. The internal elastic lamina is prominent and intact. There is no medial destruction or atherosclerosis. Weigert's elastic van Gieson. x 50.
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Histology In the acute stages recent thrombus occludes the lumen, and micro abscesses, as well as multinucleated giant cells, are found. Atheroma at the site of the occluding thrombus is never present. A moderate in flammatory cell infiltrate involving media and adventitia is present. As organisation proceeds, endothelial proliferation and recanalisation of the thrombus takes place, usually without retraction. Vessels of recanalisation characteristically have a very thick muscle media. The internal elastic lamina remains intact and is often thick (fig. 1). though at times, small interruptions may occur at sites of organisation. Fragmentation of the internal elastic lamina previously reported [15] has not been found in the cases analysed here. In the healed phase, aneurysm formation, wall destruction, eccentric lumina, atheroma, or calcification are not found.
(75)
Pathology of Thromboangiitis obliterans and Arteritis temporalis
159
A rteritis temporalis Good reviews on the subject have previously been reported [11, 13]. The disease affects mainly elderly patients. Material from the same source from 49 patients, 21 males (mean age 68 years) and 28 females (mean age 73 years), mean age for both sexes 71 years, was available for study (1945-1975). The temporal arteries were involved in 48 cases (bilateral in over half), the small vessels of the skeletal muscle in 3, the subclavian artery in 2 cases, and the aorta in 1 case.
Pathology
Macroscopy The artery is thick, white and may contain thrombus.
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Histology The ultima is often severely thickened, consisting often of two distinct layers. The inner intimal layer is mucinous and contains only scanty, loosely arranged spindle cells. The outer intimal layer is denser and contains numerous fibroblasts. It is in this region extending to the inner medial layer where most severe inflammatory changes occur, consisting mainly of chronic inflammatory cells, but eosinophils may occasionally be found. Multinucleated giant cells are frequently, but not invariably present (fig. 2). The internal elastic lamina is often fragmented to a very severe degree. The inflammatory process extends to the adventitia. Fibrinoid necrosis may occur. Electron-microscopically, fibrillar material, possibly altered elastic tissue or possibly fibrin, has been demonstrated [21]. As healing pro ceeds, the two intimal layers often remain recognisable. The condition is usually easily distinguishable from polyarteritis nodosa, but very rarely distinction may be difficult [13]. Identical changes were encountered in the vessels of the skeletal muscle in three patients suffering from polymyalgia rhcumatica. In these cases the temporal arteries had also been involved. The subject of poly myalgia rheumatica and arteritis temporalis has been extensively in vestigated [7|. Table I summarises the salient histological features of thromboangiitis obliterans and arteritis temporalis.
160
(76)
O lsk n
Fig. 2. Photomicrograph of part of a temporal artery from a man aged 72. showing inflammatory cells and giant cells, particularly in the outer layer of the thickened intima of the inner layer of the media. Fragmentation of the elastic lamina can also be recognised (arrowed). The lower right hand area in the photograph is occupied by the inner layer of the thickened intima. HE. x 350. Table /. Comparison of thromboangiitis obliterans (Buerger’s disease) and arteritis temporalis
Internal elastic lamina Intimal thickening Lumen Giant cells Healing
Media
Arteritis temporalis
mean 37 years male dominance 6:1 medium-sized and small arteries and veins intact endothelial proliferation occluded by thrombus usually absent may be present organised thrombus (no retraction)
mean 71 years slight female dominance mainly medium-sized but also large and small arteries fragmented severe often occluded by thrombus often present
panarteritis
intima in two layers thrombus retraction and incorporation into wall panarteritis Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 2/28/2020 9:30:55 PM
Age Sex Type of vessel
Thromboangiitis obliterans (Buerger's disease)
(77)
Pathology of Thromboangiitis obliterans and Arteritis temporalis
161
Some Unusual Manifestations Thromboangiitis obliterans As far as this disease is concerned, cerebral vessels [16] may be in volved, as well as systemic vessels, such as mesenteric arteries [25].
Arteritis temporalis Cerebral vessel involvement presenting as senile or presenile dementia has been reported |18J. Skin involvement, bullae and necrotic ulcers of the scalp [14] and butterfly rash [19] may occur. Miosis, trismus and dysphagia occasionally occur [6], ‘Tongue angina' [12] and peripheral neuropathy [22J have been reported. The subclavian arteries, producing the subclavian steal syndrome 117], and a variety of other arteries may be involved, such as coronary arteries [5], renal arteries [1], skeletal muscle arteries (polymyalgia rheumatica) 17], limb arteries [8], mesenteric arteries [19], aortitis without temporal arterial involvement [5], and dissecting aneurysm of the aorta [10],
1 Balmforth, G .V .: Temporal arteritis and renal failure. Archs intern. Med. 113! 230 234(1964). 2 Bloodwell, R. D.: S helley, W. M.; O ttesen, O.; G oodman, R. M., and M c K usick, V.: Buerger’s disease: a distinct clinical and pathologic entity. Abstract. Circulation 26: 689 (1962). 3 Leader: Buerger's disease. Br. med. J. ii: 1214 (I960). 4 Buerger, W . : Thrombo-angiitis obliterans: a study of vascular lesions leading to presenile spontaneous gangrene. Am. J. med. Sei. 266 : 278 291 (1973). 5 C ardell, B. S. and H anley, T. J.: A fatal case of giant cell or temporal arteritis. J. Path. Bact. 63: 587 597 (1951). 6 D esser, E. J.: Miosis, trismus and dysphagia; an unusual presentation of temporal arteritis. Ann. intern. Med. 71: 961 962 (1969). 7 F auchald, F.; R ygvold, O., and O vstese, B.: Temporal arteritis and polymyalgia rheumatica. Ann. intern. Med. 77: 845 852 (1972). 8 F inlayson , R. and R obinson , J. O.: Giant-cell arteritis o f the legs. Br. med. J. ii: 1595 1597 (1955). 9 G ore, I. and Burrows, S .: A reconsideration of the pathogenesis of Buerger’s disease. Am. J. clin. Path. 29: 319-330 (1958). 10 H arris, M.: Dissecting aneurysm of the aortta due to giant cell arteritis. Br. Heart J. 30: 840 844 (1968).
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References
162
Ol.SI-N
(78)
11 H arrison, C. V.: Giant cell or temporal arteritis: a review. J. clin. Path. I: 197 211 (1947/48). 12 H enderson, A. H.: Tongue pain with giant cell arteritis. Br. med. J. iv; 337 (1967). 13 H eptinstall, R. A.; Porter, K. A., and Barkley, H.: Giant-cell (temporal) arteritis. J. Path. Bact. 67: 507 519 (1954). 14 K inqont, p. o . C. and M c C allum, D. I.: Skin manifestations of giant cell arteritis. Br. J. Derm. 76: 299 308 (1964). 15 Leu , H. J. und Brunner, U .: Zur pathologisch-anatomischen Abgrenzung der Throm boangiitis obliterans von der Arteriosklerose. Dt. med. Wschr. 98: 158 161 (1973). 16 L ippmann , H. I.: Cerebrovascular thrombosis in patients with Buerger’s disease. Circulation 5: 680 692 (1952). 17 P ollock, M.; Blennerhassett, J. B., and C larke, A. M.: Giant cell arteritis and subclavian steal syndrome. Neurology, Minneap. 23: 653 657 (1973). 18 R eid, A. H. and M aloney, A. F. J.: Giant cell arteritis and arteriolitis associated with amyloid angiopathy in an elderly Mongol. Acta neuropath. 27: 131 137 (1974). 19 R ussel, R. W. R.: Giant-cell arteritis: a review of 35 cases. Q. II Med. 28: 471 489 (1959). 20 Schatz , I.J.; F ine, G.. and Eyler, W. R.: Thromboangiitis obliterans. Br. Heart J. 28: 84 91 (1966). 21 S mith, K. R„ jr.: Electron microscopy of giant cell (temporal) arteritis. J. Neurol. Neurosurg. Psychiat. 32: 348 353 (1969). 22 W arrell, D. A.; G odfrey, S„ and O lsen, E. G. J.: Giant-cell arteritis with peripheral neuropathy. Lancet ii: 1010 1013 (1968). 23 W essler, S.; M ing , S.-C ; G urewich ., and F reiman, D. G .: A critical evaluation of thromboangiitis obliterans. The case against Buerger's disease. New Engl. J. Med. 262: 1149-1160 (1968). 24 W illiams, G.: Recent views on Buerger's disease. J. clin. Path. 22: 573-577 (1969). 25 Wolf. E. A .; S umner, D. S.. and Strandness, D. E.: Disease o f the mesenteric circulation in patients with thromboangiitis obliterans. Vase. Surg. 6: 218-223
Dr. E. G.J. O lsen, Department of Morbid Anatomy and Histopathology, National Heart Hospital. Westmoreland Street, London W !M 8HA (England)
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 2/28/2020 9:30:55 PM
(1972).
Pathological Features of Thromboangiitis obliterans and Arteritis temporalis and Some Unusual Manifestations E. G. J. O lsen Department of Morbid Anatomy and Histopathology. National Heart Hospital, and Cardiothoracic Institute, University of London, London
Key Words. Arteritis • Granulomatous arteritis • Temporal arteritis • Thrombo angiitis obliterans • Buerger’s disease
Thromboangiitis obliterans (Buerger’s disease) Since the original description of this disease of vessels by B u e r g e r in 1908 (and re-issued in 1973 [4]), its existence had from time to time been doubted in the intervening years [3, 9, 23]. Subsequently it has become clear that thromboangiitis obliterans exists [2, 20, 24], which is also the belief of this author. To substantiate this claim, material from 52 cases obtained from the Department of Morbid Anatomy, Royal Postgraduate Medical School, and the National Heart Hospital, London, from 1935 to 1975 was available. 45 males (mean age 41 years) and 7 females (mean age 36 years), mean age for both sexes 37 years, were analysed. The arteries were involved as follows: Lower limb 35 cases, small vessels in toes 9 cases, upper limb vessels 8 cases.
Macroscopy The vessels are segmentally involved and may present as thick fibrous cords. Occasionally thrombus can be discerned with the naked eye. Arteries and veins are not necessarily involved in the same cross section.
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Pathology
158
Ol.SUN
(74)
Fig. I . Photomicrograph of a posterior tibial artery from a man aged 31 suffer ing from thromboangiitis obliterans. The lumen of the vessel is occluded by an organising thrombus. The internal elastic lamina is prominent and intact. There is no medial destruction or atherosclerosis. Weigert's elastic van Gieson. x 50.
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 2/28/2020 9:30:55 PM
Histology In the acute stages recent thrombus occludes the lumen, and micro abscesses, as well as multinucleated giant cells, are found. Atheroma at the site of the occluding thrombus is never present. A moderate in flammatory cell infiltrate involving media and adventitia is present. As organisation proceeds, endothelial proliferation and recanalisation of the thrombus takes place, usually without retraction. Vessels of recanalisation characteristically have a very thick muscle media. The internal elastic lamina remains intact and is often thick (fig. 1). though at times, small interruptions may occur at sites of organisation. Fragmentation of the internal elastic lamina previously reported [15] has not been found in the cases analysed here. In the healed phase, aneurysm formation, wall destruction, eccentric lumina, atheroma, or calcification are not found.
(75)
Pathology of Thromboangiitis obliterans and Arteritis temporalis
159
A rteritis temporalis Good reviews on the subject have previously been reported [11, 13]. The disease affects mainly elderly patients. Material from the same source from 49 patients, 21 males (mean age 68 years) and 28 females (mean age 73 years), mean age for both sexes 71 years, was available for study (1945-1975). The temporal arteries were involved in 48 cases (bilateral in over half), the small vessels of the skeletal muscle in 3, the subclavian artery in 2 cases, and the aorta in 1 case.
Pathology
Macroscopy The artery is thick, white and may contain thrombus.
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 2/28/2020 9:30:55 PM
Histology The ultima is often severely thickened, consisting often of two distinct layers. The inner intimal layer is mucinous and contains only scanty, loosely arranged spindle cells. The outer intimal layer is denser and contains numerous fibroblasts. It is in this region extending to the inner medial layer where most severe inflammatory changes occur, consisting mainly of chronic inflammatory cells, but eosinophils may occasionally be found. Multinucleated giant cells are frequently, but not invariably present (fig. 2). The internal elastic lamina is often fragmented to a very severe degree. The inflammatory process extends to the adventitia. Fibrinoid necrosis may occur. Electron-microscopically, fibrillar material, possibly altered elastic tissue or possibly fibrin, has been demonstrated [21]. As healing pro ceeds, the two intimal layers often remain recognisable. The condition is usually easily distinguishable from polyarteritis nodosa, but very rarely distinction may be difficult [13]. Identical changes were encountered in the vessels of the skeletal muscle in three patients suffering from polymyalgia rhcumatica. In these cases the temporal arteries had also been involved. The subject of poly myalgia rheumatica and arteritis temporalis has been extensively in vestigated [7|. Table I summarises the salient histological features of thromboangiitis obliterans and arteritis temporalis.
160
(76)
O lsk n
Fig. 2. Photomicrograph of part of a temporal artery from a man aged 72. showing inflammatory cells and giant cells, particularly in the outer layer of the thickened intima of the inner layer of the media. Fragmentation of the elastic lamina can also be recognised (arrowed). The lower right hand area in the photograph is occupied by the inner layer of the thickened intima. HE. x 350. Table /. Comparison of thromboangiitis obliterans (Buerger’s disease) and arteritis temporalis
Internal elastic lamina Intimal thickening Lumen Giant cells Healing
Media
Arteritis temporalis
mean 37 years male dominance 6:1 medium-sized and small arteries and veins intact endothelial proliferation occluded by thrombus usually absent may be present organised thrombus (no retraction)
mean 71 years slight female dominance mainly medium-sized but also large and small arteries fragmented severe often occluded by thrombus often present
panarteritis
intima in two layers thrombus retraction and incorporation into wall panarteritis Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 2/28/2020 9:30:55 PM
Age Sex Type of vessel
Thromboangiitis obliterans (Buerger's disease)
(77)
Pathology of Thromboangiitis obliterans and Arteritis temporalis
161
Some Unusual Manifestations Thromboangiitis obliterans As far as this disease is concerned, cerebral vessels [16] may be in volved, as well as systemic vessels, such as mesenteric arteries [25].
Arteritis temporalis Cerebral vessel involvement presenting as senile or presenile dementia has been reported |18J. Skin involvement, bullae and necrotic ulcers of the scalp [14] and butterfly rash [19] may occur. Miosis, trismus and dysphagia occasionally occur [6], ‘Tongue angina' [12] and peripheral neuropathy [22J have been reported. The subclavian arteries, producing the subclavian steal syndrome 117], and a variety of other arteries may be involved, such as coronary arteries [5], renal arteries [1], skeletal muscle arteries (polymyalgia rheumatica) 17], limb arteries [8], mesenteric arteries [19], aortitis without temporal arterial involvement [5], and dissecting aneurysm of the aorta [10],
1 Balmforth, G .V .: Temporal arteritis and renal failure. Archs intern. Med. 113! 230 234(1964). 2 Bloodwell, R. D.: S helley, W. M.; O ttesen, O.; G oodman, R. M., and M c K usick, V.: Buerger’s disease: a distinct clinical and pathologic entity. Abstract. Circulation 26: 689 (1962). 3 Leader: Buerger's disease. Br. med. J. ii: 1214 (I960). 4 Buerger, W . : Thrombo-angiitis obliterans: a study of vascular lesions leading to presenile spontaneous gangrene. Am. J. med. Sei. 266 : 278 291 (1973). 5 C ardell, B. S. and H anley, T. J.: A fatal case of giant cell or temporal arteritis. J. Path. Bact. 63: 587 597 (1951). 6 D esser, E. J.: Miosis, trismus and dysphagia; an unusual presentation of temporal arteritis. Ann. intern. Med. 71: 961 962 (1969). 7 F auchald, F.; R ygvold, O., and O vstese, B.: Temporal arteritis and polymyalgia rheumatica. Ann. intern. Med. 77: 845 852 (1972). 8 F inlayson , R. and R obinson , J. O.: Giant-cell arteritis o f the legs. Br. med. J. ii: 1595 1597 (1955). 9 G ore, I. and Burrows, S .: A reconsideration of the pathogenesis of Buerger’s disease. Am. J. clin. Path. 29: 319-330 (1958). 10 H arris, M.: Dissecting aneurysm of the aortta due to giant cell arteritis. Br. Heart J. 30: 840 844 (1968).
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 2/28/2020 9:30:55 PM
References
162
Ol.SI-N
(78)
11 H arrison, C. V.: Giant cell or temporal arteritis: a review. J. clin. Path. I: 197 211 (1947/48). 12 H enderson, A. H.: Tongue pain with giant cell arteritis. Br. med. J. iv; 337 (1967). 13 H eptinstall, R. A.; Porter, K. A., and Barkley, H.: Giant-cell (temporal) arteritis. J. Path. Bact. 67: 507 519 (1954). 14 K inqont, p. o . C. and M c C allum, D. I.: Skin manifestations of giant cell arteritis. Br. J. Derm. 76: 299 308 (1964). 15 Leu , H. J. und Brunner, U .: Zur pathologisch-anatomischen Abgrenzung der Throm boangiitis obliterans von der Arteriosklerose. Dt. med. Wschr. 98: 158 161 (1973). 16 L ippmann , H. I.: Cerebrovascular thrombosis in patients with Buerger’s disease. Circulation 5: 680 692 (1952). 17 P ollock, M.; Blennerhassett, J. B., and C larke, A. M.: Giant cell arteritis and subclavian steal syndrome. Neurology, Minneap. 23: 653 657 (1973). 18 R eid, A. H. and M aloney, A. F. J.: Giant cell arteritis and arteriolitis associated with amyloid angiopathy in an elderly Mongol. Acta neuropath. 27: 131 137 (1974). 19 R ussel, R. W. R.: Giant-cell arteritis: a review of 35 cases. Q. II Med. 28: 471 489 (1959). 20 Schatz , I.J.; F ine, G.. and Eyler, W. R.: Thromboangiitis obliterans. Br. Heart J. 28: 84 91 (1966). 21 S mith, K. R„ jr.: Electron microscopy of giant cell (temporal) arteritis. J. Neurol. Neurosurg. Psychiat. 32: 348 353 (1969). 22 W arrell, D. A.; G odfrey, S„ and O lsen, E. G. J.: Giant-cell arteritis with peripheral neuropathy. Lancet ii: 1010 1013 (1968). 23 W essler, S.; M ing , S.-C ; G urewich ., and F reiman, D. G .: A critical evaluation of thromboangiitis obliterans. The case against Buerger's disease. New Engl. J. Med. 262: 1149-1160 (1968). 24 W illiams, G.: Recent views on Buerger's disease. J. clin. Path. 22: 573-577 (1969). 25 Wolf. E. A .; S umner, D. S.. and Strandness, D. E.: Disease o f the mesenteric circulation in patients with thromboangiitis obliterans. Vase. Surg. 6: 218-223
Dr. E. G.J. O lsen, Department of Morbid Anatomy and Histopathology, National Heart Hospital. Westmoreland Street, London W !M 8HA (England)
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 2/28/2020 9:30:55 PM
(1972).
