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
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