Heart Vessels (1992) Suppl. 7:44-47
Heart andVesse~ S © Springer-Verlag1992
Takayasu arteritis in Israel Talma Rosenthal,l B e n j a m i n a Morag, 2 and Yacov Itzchak, 2 Department of Medicine D, Chaim Sheba Medical Center, Tel Hashomer 52621, Israel 2Department of Diagnostic Imaging, Tel Aviv University Sackler School of Medicine, Tel Aviv, Israel
Introduction Chaim Sheba Medical Center is a national referral center and treats approximately 75% of the patients with Takayasu arteritis in Israel. A total of 50 cases have been seen to date, including the first series of 22 patients hospitalized at Sheba from 1965-1973 and reported by Deutsch [1] in 1974, and the 28 cases reported here. All cases were confirmed by arteriography, since clinical findings and plain film chest roentgenogram may indicate the presence of the disease but confirmation of the diagnosis and determination of the extent and type of lesion can be obtained only by angiographic studies. Vascular lesions may involve the aortic arch and brachiocephalic vessels as well as the abdominal aorta and its main branches. The angiographic features are narrowing and dilatations due to loss of elasticity of the vessel wall. Takayasu arteritis has been reported among Jewish ethnic groups almost exclusively in the Oriental and Sephardic communities. This is in contrast to Burger's disease which affects mainly Ashkenazic Jews [2]. A few words are required here about the Jewish communities of Israel, which divide roughly into 2 groups of differing origins. Sephardim are descendants of the Jews who were expelled from Spain in 1492 during the Inquisition and settled mainly in the then Turkish Empire; their descendants can be found in Greece, Turkey, Bulgaria, and other Mediterranean countries, as well as in North Africa. Ashkenazim are the descendants of the German Jews who populated Eastern Europe and form the majority of the present day Jewish communities of North and South America, England, and South Africa [1]. Of the 22 patients in Deutsch's series, 9 were Arabs or Bedouins, 8 were Oriental Jews, and 5 were
Address correspondence to: Y. Itzchak
Sephardic Jews. Our group of 28 patients numbers 4 Arabs, 2 Yemenite Jews, and the rest mainly Sephardic Jews, including a group from the Balkans. The four Arab patients in the earlier group and the 4 cases in the second group give a frequency of 16% for the Arab population - much higher than the rare occurrence reported among Arabs in the literture. Slatter et al. [3] listed two Palestinians, one Kuwaiti, and one Egyptian in his study, and stated that this entity had not been described previously in the Arab population. Lack of reporting might account for the apparent rarity of the disease in this population (Table
1). With regard to the Bedouins, genetic studies have confirmed the introduction of many African genes in the Sinai Bedouin due to the proximity of Africa and the Sinai Peninsula [2]. It is also noteworthy that all our patients who originated from India had aneurysmal changes in the thoracic aorta, and three of the five Beduin and most of the Arabs had stenosis or complete occlusion of the abdominal aorta. All the Yemenites showed involvement of the renal artery. The incidence of Takayasu arteritis is definitely higher in non-Caucasian racial groups, including the Japanese. It may, however, be more common among Caucasians than formerly realized, and there is also evidence that it is more common in North America than was previously thought [4]. The reported female : male ratio in Takayasu disease of 8 : 1 is strikingly different from our groups: 1.2 : 1 in the earlier group of 22 patients, and 2.5:1 in the present group of 28 patients. The 1.7 : 1 ratio for these 2 groups taken together also attests to a definite female predominance. The most common physical findings in out and others' series were multiple vascular bruits and/or absent or reduced pulses, leg and arm claudication, and hypertension, reflecting widespread vascular stenosis. Ecke et al.'s [5] recommendation to include Takayasu disease in the differential diagnosis of
T. Rosenthal et al.: Takayasu arteritis in Israel
45
b
Fig. l a - d . Angiographic study of the aortic arch and abdominal aorta in a 41-year-old female with hypertension and diminished pulses on both arms. a Arterial phase of arch angiography showing bilateral subclavian artery narrowings and dilatations (arrows). b Selective subclavian artery angiography showing long stenosis and colaterals (arrows). e Abdominal aorta with irregularities and right renal artery occlusion and left renal artery stenosis, el Selective left renal artery demonstrates severe concentric stenosis, 2 cm distal to its origin. Post-stenotic dilatation is present
hypertension in infancy and childhood gains support from our finding of the disease in 3 children, aged 6, 13, and 16 years old. Nonspecific symptoms of inflam-
mation were reported by m a n y of our patients prior to diagnosis, including fever, sweats, malaise, arthralgia, and myalgia. Hypertension, which can b e due to coarctation of aorta - rare in out series - or renovascular hypertension can also be quite severe in Takayasu disease and is often predominant [6]. Renovascular hypertension was present in 17 of the 28 patients (60%) presented here. This high per-
46
T. Rosenthal et al. : Takayasu arteritis in Israel
Table 1. Ethnic distribution of cases Case no.
Age (years)
Table 2. Vascular findings on angiography in 50 patients Sex
Origin a
Earlier series, 1965-1973 [1] 1. 18 2. 22 3. 12 4. 22 5. 40 6. 40 7. 23 8. 27 9. 40 10. 40 11. 24 12. 36 13. 27 14. 42 15. 30 16. 26 17. 45 18. 36 19. 24 20. 26 21. 21 22. 38
F F F M M M F F M M F M M F F M M F M F F F
India India India India Beduin Beduin Beduin Beduin Beduin Arab Arab Arab Arab Bulgaria Iran Ethiopia Greece Iraq Yemen Syria Tunisia Turkey
Present series, 1973-1991 23. 35 24. 6 25. 13 26. 39 27. 3 28. 24 29. 28 30. 36 31. 40 32. 39 33. 26 34. 28 35. 16 36. 41 37. 26 38. 32 39. 20 40. 29 41. 38 42. 40 43. 28 44. 46 45. 31 46. 47 47. 55 48. 30 49. 17 50. 55
F M F F F F F M M F F F F F F F F M M M M F M F F F F F
Egypt Egypt Egypt Yemen Yemen Arab Arab Arab Arab Iraq Iraq Turkey Turkey Turkey Syria Tunisia Tunisia India India Bulgaria Greece Greece Israel Russia Russia Bucharia Rumania Hungary
aPatients 5-13 and 28-31 are Israeli Arabs
centage of renal artery stenosis contrasts with the rarity of this finding in a Japanese series reported by M o r o o k a et al. [7]. These authors [8] later noted that the stenosed renal artery in all seven of their patients was less than 1 . 5 m m at the m a x i m u m area of stenosis, and that even when high-grade renal stenosis or obstruction is present, renal atrophy is not always
Aortic arch + abdominal aorta Present series Deutsch's group [1] Total
Aortic arch
11 7
11 10
18 (36%)
21 (42%)
Abdominal aorta 6 5 11 (22%)
frequent or marked. The frequency of renal artery involvement in our series was 60% higher than the 34% reported by the Japanese, but lower than the 85% incidence reported for Singapore [9]. Systemic hypertension secondary to renal artery stenosis is a common major complication of Takayasu disease [10, 11]. It is suggested that the differential diagnosis of renovascular hypertension takes account of aortoarteritis, particularly in young females in whom it greatly affects the course of the disease. The vascular findings in the 50 patients are presented in Table 2. Altogether, 36% had combined involvement of the aortic arch (Fig. 1), 42% had involvement of the aortic arch only, and the remaining 29% had disease in the abdominal aorta and its branches only. Our survey points to a n u m b e r of interesting findings. Firstly, it indicates that Takayasu arteritis affects males and not mainly females, as previously thought. Furthermore, relative to their numbers in Israel, a high percentage of Arabs are affected by Takayasu arteritis, and recognition of this fact by clinicians might lead to earlier detection of more cases in the early stages. This same awareness is also important for Bedouins who have a disproportionately high incidence of Takayasu arteritis for their numbers in the population.
References 1. Deutsch V, Wexler L, Deutsch H (1974) Takayasu's arteritis. An angiographic study with remarks on ethnic distribution in Israel. Am J Roentgenol Rad Therapy 122:13-27 2. Goodman RM (1979) Genetic disorders among the Jewish people. Johns Hopkins University Press, Baltimore, pp 364-367 3. Slatter MA, White AG, Eklof B, Fenech FF (1985) Takayasu's disease in Arabs. Postgrad Med J 61: 387-390 4. Hall S, Barr W, Lie JT, Stamson AW, Kazmier FJ, Hunder GG (1985) Takayasu arteritis. A study of 32 North American patients. Medicine 64:89-99 5. Ecke FU, Balle JW, Hardy BE (1984) Three patients with arteritis. Arch Dis Child 59:877-883 6. Gordon RD, Feeney GFK, Jeavons SJ, Naughton L, Roberts HJV (1987) A young woman with malignant hypertension. Aust NZ J Med 17:351-358
T. Rosenthal et al.: Takayasu arteritis in Israel 7. Morooka S, Saito Y, Nonaka Y, Gyotoku Y, Sugimoto T (1984) Clinical features and course of aortitis syndrome in Japanese women older than 40 years. Am J Cardiol 53:859-861 8. Morooka S, Kimura Y, Sumino S, Takabetake Y, Sugimoto T (1985) The size of kidney with renovascular hypertension in patients with aortitis syndrome. Angiology 36:105-109 9. Teoh PC, Tan LKA, Chia BL, Chao TC, Tambyah JA, Feng PH (1978) Non-specific aorto-arteritis in Singapore
47 with special reference to hypertension. Am Heart J 95:683-690 10. Cook PG, Wells IP, Marshall AJ (1986) Case report: Renovascular hypertension in Takayasu's disease treated by percutaneous angioplasty. Clin Radiol 37: 583-584 11. Cuocolo A, McCarthy KE, Sandrock D, Miller DL, Neumann RD (1989) Radionuclide renography predicts functional changes in patients with renal artery involvement by Takayasu's arteritis. Urol Radiol 11:69-76
Heart andVesse~ S © Springer-Verlag1992
Takayasu arteritis in Israel Talma Rosenthal,l B e n j a m i n a Morag, 2 and Yacov Itzchak, 2 Department of Medicine D, Chaim Sheba Medical Center, Tel Hashomer 52621, Israel 2Department of Diagnostic Imaging, Tel Aviv University Sackler School of Medicine, Tel Aviv, Israel
Introduction Chaim Sheba Medical Center is a national referral center and treats approximately 75% of the patients with Takayasu arteritis in Israel. A total of 50 cases have been seen to date, including the first series of 22 patients hospitalized at Sheba from 1965-1973 and reported by Deutsch [1] in 1974, and the 28 cases reported here. All cases were confirmed by arteriography, since clinical findings and plain film chest roentgenogram may indicate the presence of the disease but confirmation of the diagnosis and determination of the extent and type of lesion can be obtained only by angiographic studies. Vascular lesions may involve the aortic arch and brachiocephalic vessels as well as the abdominal aorta and its main branches. The angiographic features are narrowing and dilatations due to loss of elasticity of the vessel wall. Takayasu arteritis has been reported among Jewish ethnic groups almost exclusively in the Oriental and Sephardic communities. This is in contrast to Burger's disease which affects mainly Ashkenazic Jews [2]. A few words are required here about the Jewish communities of Israel, which divide roughly into 2 groups of differing origins. Sephardim are descendants of the Jews who were expelled from Spain in 1492 during the Inquisition and settled mainly in the then Turkish Empire; their descendants can be found in Greece, Turkey, Bulgaria, and other Mediterranean countries, as well as in North Africa. Ashkenazim are the descendants of the German Jews who populated Eastern Europe and form the majority of the present day Jewish communities of North and South America, England, and South Africa [1]. Of the 22 patients in Deutsch's series, 9 were Arabs or Bedouins, 8 were Oriental Jews, and 5 were
Address correspondence to: Y. Itzchak
Sephardic Jews. Our group of 28 patients numbers 4 Arabs, 2 Yemenite Jews, and the rest mainly Sephardic Jews, including a group from the Balkans. The four Arab patients in the earlier group and the 4 cases in the second group give a frequency of 16% for the Arab population - much higher than the rare occurrence reported among Arabs in the literture. Slatter et al. [3] listed two Palestinians, one Kuwaiti, and one Egyptian in his study, and stated that this entity had not been described previously in the Arab population. Lack of reporting might account for the apparent rarity of the disease in this population (Table
1). With regard to the Bedouins, genetic studies have confirmed the introduction of many African genes in the Sinai Bedouin due to the proximity of Africa and the Sinai Peninsula [2]. It is also noteworthy that all our patients who originated from India had aneurysmal changes in the thoracic aorta, and three of the five Beduin and most of the Arabs had stenosis or complete occlusion of the abdominal aorta. All the Yemenites showed involvement of the renal artery. The incidence of Takayasu arteritis is definitely higher in non-Caucasian racial groups, including the Japanese. It may, however, be more common among Caucasians than formerly realized, and there is also evidence that it is more common in North America than was previously thought [4]. The reported female : male ratio in Takayasu disease of 8 : 1 is strikingly different from our groups: 1.2 : 1 in the earlier group of 22 patients, and 2.5:1 in the present group of 28 patients. The 1.7 : 1 ratio for these 2 groups taken together also attests to a definite female predominance. The most common physical findings in out and others' series were multiple vascular bruits and/or absent or reduced pulses, leg and arm claudication, and hypertension, reflecting widespread vascular stenosis. Ecke et al.'s [5] recommendation to include Takayasu disease in the differential diagnosis of
T. Rosenthal et al.: Takayasu arteritis in Israel
45
b
Fig. l a - d . Angiographic study of the aortic arch and abdominal aorta in a 41-year-old female with hypertension and diminished pulses on both arms. a Arterial phase of arch angiography showing bilateral subclavian artery narrowings and dilatations (arrows). b Selective subclavian artery angiography showing long stenosis and colaterals (arrows). e Abdominal aorta with irregularities and right renal artery occlusion and left renal artery stenosis, el Selective left renal artery demonstrates severe concentric stenosis, 2 cm distal to its origin. Post-stenotic dilatation is present
hypertension in infancy and childhood gains support from our finding of the disease in 3 children, aged 6, 13, and 16 years old. Nonspecific symptoms of inflam-
mation were reported by m a n y of our patients prior to diagnosis, including fever, sweats, malaise, arthralgia, and myalgia. Hypertension, which can b e due to coarctation of aorta - rare in out series - or renovascular hypertension can also be quite severe in Takayasu disease and is often predominant [6]. Renovascular hypertension was present in 17 of the 28 patients (60%) presented here. This high per-
46
T. Rosenthal et al. : Takayasu arteritis in Israel
Table 1. Ethnic distribution of cases Case no.
Age (years)
Table 2. Vascular findings on angiography in 50 patients Sex
Origin a
Earlier series, 1965-1973 [1] 1. 18 2. 22 3. 12 4. 22 5. 40 6. 40 7. 23 8. 27 9. 40 10. 40 11. 24 12. 36 13. 27 14. 42 15. 30 16. 26 17. 45 18. 36 19. 24 20. 26 21. 21 22. 38
F F F M M M F F M M F M M F F M M F M F F F
India India India India Beduin Beduin Beduin Beduin Beduin Arab Arab Arab Arab Bulgaria Iran Ethiopia Greece Iraq Yemen Syria Tunisia Turkey
Present series, 1973-1991 23. 35 24. 6 25. 13 26. 39 27. 3 28. 24 29. 28 30. 36 31. 40 32. 39 33. 26 34. 28 35. 16 36. 41 37. 26 38. 32 39. 20 40. 29 41. 38 42. 40 43. 28 44. 46 45. 31 46. 47 47. 55 48. 30 49. 17 50. 55
F M F F F F F M M F F F F F F F F M M M M F M F F F F F
Egypt Egypt Egypt Yemen Yemen Arab Arab Arab Arab Iraq Iraq Turkey Turkey Turkey Syria Tunisia Tunisia India India Bulgaria Greece Greece Israel Russia Russia Bucharia Rumania Hungary
aPatients 5-13 and 28-31 are Israeli Arabs
centage of renal artery stenosis contrasts with the rarity of this finding in a Japanese series reported by M o r o o k a et al. [7]. These authors [8] later noted that the stenosed renal artery in all seven of their patients was less than 1 . 5 m m at the m a x i m u m area of stenosis, and that even when high-grade renal stenosis or obstruction is present, renal atrophy is not always
Aortic arch + abdominal aorta Present series Deutsch's group [1] Total
Aortic arch
11 7
11 10
18 (36%)
21 (42%)
Abdominal aorta 6 5 11 (22%)
frequent or marked. The frequency of renal artery involvement in our series was 60% higher than the 34% reported by the Japanese, but lower than the 85% incidence reported for Singapore [9]. Systemic hypertension secondary to renal artery stenosis is a common major complication of Takayasu disease [10, 11]. It is suggested that the differential diagnosis of renovascular hypertension takes account of aortoarteritis, particularly in young females in whom it greatly affects the course of the disease. The vascular findings in the 50 patients are presented in Table 2. Altogether, 36% had combined involvement of the aortic arch (Fig. 1), 42% had involvement of the aortic arch only, and the remaining 29% had disease in the abdominal aorta and its branches only. Our survey points to a n u m b e r of interesting findings. Firstly, it indicates that Takayasu arteritis affects males and not mainly females, as previously thought. Furthermore, relative to their numbers in Israel, a high percentage of Arabs are affected by Takayasu arteritis, and recognition of this fact by clinicians might lead to earlier detection of more cases in the early stages. This same awareness is also important for Bedouins who have a disproportionately high incidence of Takayasu arteritis for their numbers in the population.
References 1. Deutsch V, Wexler L, Deutsch H (1974) Takayasu's arteritis. An angiographic study with remarks on ethnic distribution in Israel. Am J Roentgenol Rad Therapy 122:13-27 2. Goodman RM (1979) Genetic disorders among the Jewish people. Johns Hopkins University Press, Baltimore, pp 364-367 3. Slatter MA, White AG, Eklof B, Fenech FF (1985) Takayasu's disease in Arabs. Postgrad Med J 61: 387-390 4. Hall S, Barr W, Lie JT, Stamson AW, Kazmier FJ, Hunder GG (1985) Takayasu arteritis. A study of 32 North American patients. Medicine 64:89-99 5. Ecke FU, Balle JW, Hardy BE (1984) Three patients with arteritis. Arch Dis Child 59:877-883 6. Gordon RD, Feeney GFK, Jeavons SJ, Naughton L, Roberts HJV (1987) A young woman with malignant hypertension. Aust NZ J Med 17:351-358
T. Rosenthal et al.: Takayasu arteritis in Israel 7. Morooka S, Saito Y, Nonaka Y, Gyotoku Y, Sugimoto T (1984) Clinical features and course of aortitis syndrome in Japanese women older than 40 years. Am J Cardiol 53:859-861 8. Morooka S, Kimura Y, Sumino S, Takabetake Y, Sugimoto T (1985) The size of kidney with renovascular hypertension in patients with aortitis syndrome. Angiology 36:105-109 9. Teoh PC, Tan LKA, Chia BL, Chao TC, Tambyah JA, Feng PH (1978) Non-specific aorto-arteritis in Singapore
47 with special reference to hypertension. Am Heart J 95:683-690 10. Cook PG, Wells IP, Marshall AJ (1986) Case report: Renovascular hypertension in Takayasu's disease treated by percutaneous angioplasty. Clin Radiol 37: 583-584 11. Cuocolo A, McCarthy KE, Sandrock D, Miller DL, Neumann RD (1989) Radionuclide renography predicts functional changes in patients with renal artery involvement by Takayasu's arteritis. Urol Radiol 11:69-76
