Case Discussion 高安氏病（Takayasu's disease），(大動脈炎) < Case presentation > This 27-year-old woman, G3P2, had been in good health except that she ever received appendectomy at childhood. She noted dry cough, headache and intermittent chest pain for 7 months. These symptoms subsided spontaneously, but headache with upper back and occasionally dull chest pain developed. Sensation of the back and the fingers after early awakening was also noted. She also experienced itching sensation over bilateral forearms after sun exposure. On Nov 12, 2004, she went to a medical center due to severe cough with blood tinged sputum and generalized edema for three days. < Course and Treatment > On visiting, physical examination showed pale conjunctiva and discrepancy of bilateral brachial arterial blood pressure (right: 170/80 mmHg; left: 123/91 mmHg). Laboratory results showed microcytic anemia (Hb 8.2mg/dl, MCV 68.6%) and impaired renal function (Cr 5.4 mg/dL). Chest X-film revealed cardiomegaly, and bilateral lung field infiltration . Under the impression of pulmonary hemorrhage or pulmonary edema according to chest X-film, autoimmune profiles were checked and cardiac echogram was arranged. The results showed elevated ESR (106mm/1hr) and cardiac duplex revealed stenosis of the left proximal subclavian artery and bilateral carotid arteries. Takayasu's arteritis was impressed and hydrocortisone (Solucortef 100mg qd) was given. Her general Condition improved later after the treatment and she was then discharged uneventfully. < Laboratory and Image Study > 1. CBC/DC [ CBC+PLT ] 項目 WBC RBC HB HCT MCV MCH MCHC  PLT 日期  K/μL M/μL g/dL %  fL pg g/dL K/μL  931123 13.64 4.27 9.6 28.5 66.7 22.5 33.7 308.0 2. Biochemistry 項目 T-BIL AST ALT ALP GGT LDH BUN CR 日期 mg/dL U/L U/L U/L U/L U/L mg/dL mg/dL 931123 0.2 12.0 2.0 304.0 91.0 326.0 94.9 5.4 項目 Na K Cl Ca P  Mg 日期 mmol/L mmol/L mmol/L mmol/L mg/dL mmol/L 931127 141.7  4.01 89.0 2.63 6.79 0.91 3. Urine [ MULTISTIX ] 項目 Appearance Sp. Gr.(C) pH(C) Protein(C) Glu.(C) Ketones(C) O.B.(C) 日期 *  *  mg/dL g/dL *     931122   1.03 5.0 100.0 - 1+  - 項目  O.B.(C) Urobil.(C) Bil.(C) Nitrite(C) WBC(C) 日期 * EU/dL * * * 931122   0.1 1+  -  -    [SEDIMENT] 項目 Appearance RBC(S) WBC(S) EpithCell Cast(S) 日期  /HPF /HPF /HPF /LPF - 931122   1-2 5-10 10-15 -      4. ** 血清 (SI) ** 項 目 日 期 檢驗值 參考值 (單位) Anti-Nuclear Antibody 0931123 1:40 -   C-Reactive Protein 0931123 10.7 < 0.8 (mg/dl) C3 Quantitation 0931123 133 81.61 ~ 118.41 (mg/dl) C4 Quantitation 0931123 26.8 27.45 ± 10.72 (mg/dl) RA Factor (RAHA Titer) 0930711 1:40 - < 1: 40 (+) (1:N) S.T.S. (VDRL/RPR Test) 0930711 Non-Reactive Non-Reactive (1:N) APA  0931128  5.717 <5:(-);5~15:BL;>15:(+) (PL IU/mL) ACA 0931128  4.15 <16:(-);16~21:BL;21~60:Mod.(+); DRVVT 0931128 Positive (Ratio:1.25)   ANCA 0931227 4.3 u/ml <7: (-) ANCA PR3 0931227 <1.0 u/ml <7: (-) [ ESR ] 項 目 ESR 1Hr. ESR 2Hr. 日期 (時間) mm mm 0931218 (1402) >120 >120     檢體:SPINAL FLUID 項目 Appearance Pandy's None-Apelt Cell Count L/N Sugar 日期 * *   x11/9/μL * mg% 0931129 W;C - - 0 0/0 >50 項目 TP GLU Cytology 日期 mg/dL mg/dl   0931130 17.8mg/dl 69.0  Negative for malignant cells, A few lymphocytes and PMNs    < Renal echo > 0931126 renal parenchymal disease, bilateral (right 11.3 cm, left 11.9 cm) < Analysis > 高安氏病（Takayasu's disease），(大動脈炎)是指主動脈及其主要分支及肺動脈的慢性進行性非特異性炎性變病。以引起不同部位的狹窄或閉塞為主，少數病人因炎症破壞動脈壁的中層，而致動脈擴張或動脈瘤。因病變的部位不同，其臨床表現也不同。病變位於主動脈弓及其分支的病曾稱為“高安氏病（Takayasu's disease）或無脈病”； 在腎動脈可引起腎血管性高血壓；累及肺動脈可能產生肺動脈高壓；影響冠狀動脈可產生心絞痛或心肌梗塞。本病在亞洲裔的婦人盛行率較高，約90%在30歲以內發病。 臨床表現 在局部症狀或體征出現前數周，少數病人可有全身不適，易疲勞、發熱、食欲不振、噁心、出汗、體重下降和月經不調等症狀。根據病變部位可分為四種類型；1.頭臂動脈型（主動脈弓症候群）；2.胸腹主動脈型；3.廣泛型；4.肺動脈型。 輔助檢查 一、實驗室檢查  紅細胞沉降率增快 血沉是反映本病病變活動的一項重要指標。約43 %的患者血沉快，可快至130mm/小時。其中發病10年以內者，多數血沉增快，大於10年者則病情趨於穩定，血沉恢復正常。 Ｃ反應蛋白 其臨床意義與血沉相同，陽性率與血沉相似，均為本病病變活動的一項指標。 抗鏈球菌溶血素“Ｏ” 這類抗體的增加僅說明患者近期曾有溶血性鏈球菌感染，本病約半數患者出現陽性或可疑陽性反應。 二、胸部X線檢查 心臟改變 約1/3患者有不同程度的心臟擴大．胸主動脈的改變 常為升主動脈或弓降部的膨隆，凸出，擴張，甚至瘤樣擴張，可能係高血壓的影響或大動脈炎的表現，與病變類型及範圍有關。降主動脈，尤以中下段變細內收及搏動減弱等，提示胸降主動脈廣泛狹窄的重要證據。血管造影 ，包括數位減影血管造影(DSA)仍是確診大動脈炎的主要方法，主要顯示動脈管腔的改變。 【Reference】 Sheikhzadeh A, Tettenborn I, Noohi F, Eftekharzadeh M, Schnabel A: Occlusive thromboaortopathy (Takayasu disease): clinical and angiographic features and a brief review of literature. Angiology 2002; 53: 29-40 2. Featuring the complete contents of Harrison's Principles of Internal Medicine, 16th Edition 繼續教育考題 1. (C) 下列哪一種非為大血管炎(large vessel vasculitis) A  Temperal arteritis B  Takayasu's arteritis C  Polyarteritis nodosa D  Occlusive thromboaortopathy E  Giant cell arteritis 2. (E) 下列哪一種非為Takayasu's disease的診斷標準 (ISHIKAWA'S CRITERIA Diagnosis criteria) ? A  Age < 40 year B  subclavian artery lesion C  Carotid artery tenderness D  Pulmonary artery lesion E  Elevated c-reactive protein 3. (B) 何者是發生Takayasu's disease的高危險群? A  中年婦人 B  年輕女性 C  中年男性 D  年輕男性 4. (A) Takayasu's disease 病人的盛行率約為多少? A  1-5病例/每百萬人 B  5-10病例/每百萬人 C  10-15病例/每百萬人 D  15-20病例/每百萬人 5. (E) Takayasu's disease 病人的臨床表現何者最少見? A   Fever B  Hypertension C  Arthralgia D  Aortic aneurysm E  All of above 6. (B) Takayasu's disease 病人的實驗室診斷何者為非? A  elevated C-reactive protein levels B  antinuclear antibody and antineutrophil cytoplasmic antibodies positive C  left ventricular hypertrophy D  mild anemia, E  elevated immunoglobulin levels. 7. (A) Takayasu's disease侵犯的血管何者最常見? A  Subclavians B  Aortic arch C  Ascending aorta D  Renal artery 8. (B) Takayasu's disease 病人的臨床表現何者最少見? A  decrease or absence of peripheral pulses, B  ischemic optic neuropathy C  discrepancies in blood pressure, D  arterial bruits 9. (E) 何時該懷疑病人有Secondary hypertension? A  onset of hypertension of any severity in a patient under the age of 35 or over the age of 55 B  An elevated creatinine or blood urea nitrogen level, associated with proteinuria and hematuria C  the presence of signs or symptoms of a secondary cause such as hypokalemia or metabolic alkalosis that is not explained by diuretic therapy D  stable hypertension that becomes difficult to control E  All of above 10. (C) Takayasu's disease 該如何治療，何者為非? A  glucocorticoid therapy in doses of 40 to 60 mg prednisone per day B  aggressive surgical and/or angioplastic approach to stenosed vessels C  radiotherapy for arteritis D  correcting hypertension due to renal artery stenosis E  methotrexate in doses up to 25 mg per week

答案解說 ( C ) Polyarteritis nodosa is a mutisystem, necrotizing vasculitis of small and medium-sized muscular arteries in which involvement of the renal and visceral arteries is characteristic. ( E ) (1988 ISHIKAWA'S CRITERIA Diagnosis criteria)  Obligatory criterion         Age < 40 year  Two major criteria        (1) Left mid subclavian artery lesion         (2) Right mid subclavian artery lesion  Nine minor criteria        (1) High ESR        (2) Carotid artery tenderness        (3) Hypertension        (4) Aortic regurgitation or Anuloaortic ectasia        (5) Pulmonary artery lesion        (6) Left mid common carotid lesion        (7) Distal branchiocephalic trunk lesion        (8) Descending thoracic aorta lesion        (9) Abdominal aorta lesion ( B ) Takayasu's disease affects young females in the second and third decade of life. ( A ) Takayasu's arteritis is an uncommon disease with an estimated annual incidence rate of 1.2 to 2.6 cases per million. It is most prevalent in adolescent girls and young women. Although it is more common in Asia, it is neither racially nor geographically restricted. ( E ) Diagnosis of Takayasu arteritis (TA) is often delayed because of a non-specific clinical presentation. Ishikawa's criteria (1988) has been widely used for the diagnosis of this disease. The criteria proposed consists of three major criteria including left and right mid subclavian artery lesions and characteristic signs and symptoms of at least one month duration and ten minor criteria-a high erythrocyte sedimentation rate, carotid artery tenderness, hypertension, aortic regurgitation or annuloaortic ectasia, pulmonary artery lesion, left mid common carotid lesion, distal brachiocephalic trunk lesion, descending thoracic aorta lesion, abdominal aorta lesion and coronary artery lesion. Presence of two major or one major and two minor criteria or four minor criteria suggests a high probability of TA. ( B )The mean age was 34.7 and female:male ratio was 3.6:1. According to National Institute of Health criteria, 61.5% of patients were in the acute phase of disease with systemic symptoms such as fever, weight loss, malaise, and elevated C-reactive protein levels. Immunologic markers, such as antinuclear antibody and antineutrophil cytoplasmic antibodies, were negative. The tuberculin test result was positive in 47%. Vascular bruit was present in 89%. Almost all patients had stenoses, occlusions, or aneurysmatic changes of the aorta and its main branches. Hypertension was detected in 58% and left ventricular hypertrophy was initially present in 28% patients.1 ( A ) The frequency of arteriographic abnormalities and the potentially associated clinical manifestations Artery Percent of Arteriographic Abnormalities Potential Clinical Manifestations Subclavian 93 Arm claudication, Raynaud's phenomenon Common carotid 58 Visual changes, syncope, transient ischemic attacks, stroke Abdominal aortaa 47 Abdominal pain, nausea, vomiting Renal 38 Hypertension, renal failure      ( B ) The diagnosis of Takayasu's arter itis should be suspected strongly in a young woman who develops a decrease or absence of peripheral pulses, discrepancies in blood pressure, and arterial bruits. ( E ) ( C )