Takayasu arteritis is a chronic inflammatory disease that causes stenosis, occlusion, dilation or aneurysm of the aorta and its branches. It most commonly affects adolescent girls and young women. Symptoms include limb claudication, decreased brachial pulse, hypertension, bruits, and vascular ischemia. Diagnosis is based on meeting criteria such as age of onset under 40, decreased pulse, blood pressure difference between arms, and angiographic evidence of vascular involvement. Treatment involves glucocorticoids which can control inflammation and symptoms, though relapses may occur. Additional immunosuppressants may be needed, and management of hypertension is important, especially during pregnancy which carries higher risks but fertility is not affected.

2.  A chronic, progressive, inflammatory,
occlusive disease of the aorta and its branches.
The inflammatory process results in stenosis,
occlusion, dilation or aneurysm formation in
the arterial wall.
 Aortic arch syndrome
 Pulseless disease

3.  prevalent in adolescent girls and young women
 1.2–2.6 cases per million in US
 In Asia
 Adolescent girls and young women
 Common age at presentation is 10 to 40 years

4.  There is panarteritis with inflammatory
mononuclear cell infiltrates and occasionally
giant cells.
 Marked intimal proliferation and fibrosis,
scarring and vascularization of the media, and
disruption and degeneration of the elastic
lamina.
 Narrowing of the lumen occurs with or
without thrombosis.

5.  circulating immune complexes have been
demonstrated, but their pathogenic significance
is unclear.
 Inflammatory lesions originates in Vasa
vasorum followed by cellular infilteration
maily T cells but NK cells, dentritic cells,
monocytes and neutrophils also invade outer
layer of media.
 Interlukin 6 & 1 are released to maintain
abberrant inflammatory process

6.  Triggering Antigen is unknown
 Antibodies found in TA are AECA(Antibodies
against endothelial cell)
 AECA may have role but not yet proved.

7.  CONSTITUNAL:
Dominate the presentation in 1/3rd of patients these include
Asthenia
headache
weight loss
fever
myalgia
arthralgia
Prominent back pain, especially in the thoracic region,

8.  caused by vascular damage (stenosis, occlusion or
dilataion of vessels)
 Bruit
 Claudication(upper extremity >Lower extremity)
 Hypertension
 Light headedness
 Unequal blood pressure in extremities
 Carotidynia
 Absent pulses

9.  Bruits at carotid arteries, supraclavicular or
infraclavicular space (subclavian disease)
flexor surface of the upper arm
(axillary artery disease)
abdomen (from renal or mesenteric artery
vasculitis)

10.  Angina
 Aortic Regurgitation
 Mitral regurgitation (secondary to left
ventricular dilatation due to AR)
 Congestive heart failure ( myocarditis or by
chronic aortic with or without mitral
regurgitation)

11.  Headache
 Visual Disturbance
 Stroke
 TIA
 Seizures

12.  Erythema Nodosum
 Ulcerated sabacute nodular lesion
 Pyoderma gangrenosum

13.  Rheumatic diseases :
Giant cell arteritis
Cogan syndrome
Relapsing polychrondritis,
Ankylosing spondylitis,
Rheumatoid arthritis
Sysstemic lupus erythematosus
Buergr disease and
Behcet s disease

14. Infectious disease: Syphilis
Other diseases:
Atherosclerosis
inflammatory abdominal aortic aneurysm
ergotism
radiation-induced
damage
retroperitoneal fibrosis
IBD
sarcoidosis, neurofibromatosis,
congenital coarctation, Marfan syndrome

15.  Measuring blood pressure in both arms,
carefully palpating pulses in all extremities,
and listening
for bruits in the abdomen and chest and along
the carotids
and supraclavicular and axillary areas provide
the best clinical tools in early diagnosis of
Takayasu arteritis.

16.  1. Onset at age <40 years
2. Limb claudication
3. Decreased brachial artery pulse
4. Unequal arm blood pressures (>10 mm Hg)
5. Subclavian or aortic bruit
6. Angiographic evidence of narrowing or
occlusion of the aorta or its primary branches, or
large limb arteritis
 The presence of three or more of the six criteria was sensitive
(91%) and specific (98%) for the diagnosis of Takayasu arteritis.

17.  TA does not cause any specific blood test or
urinary abnormalities, however
 ESR and CRP are raised, non specific tests. ESR
may be normal in high disease activity in 30%
patients and high in 40% patients with inactive
disease.
 Pentraxin-3 may more accurately reflect disease
activity than ESR or CRP

18.  Anemia
 Thrombocytosis
 Raised Creatinine
 Mild proteinuria or hematuria on UCE

19.  MRI and MRA
 CT and CTA
 Vascular ultrasonography
 Conventional aortography are abnormal in
virtually all patients with TA.
 Conventional angiography is the “gold
standard” for precisely delineating the
stenoses, occlusions and thrombosis.

20.  Biopsies of the aorta or other actively affected
arteries show a
granulomatous vasculitis with giant cells.

22. 2 or more features :
(1)fever
(2) elevated ESR
(3) symptoms or signs of vascular ischemia or
inflammation (e.g.,claudication, absent pulse,
carotidynia)
(4)typical angiographic features
.

23. GLUCOCORTICOIDS are very effective in
suppressing vascular inflammation as well as
constitutional symptoms within days to few
weeks; prednisone (1 mg/kg) for 1 to 3 months
and then tapered to 10 mg/d over 4–6 months.
Anemia, thrombocytosis, and elevated ESRs
also usually respond promptly

24.  Resolution of signs, symptoms, and laboratory
markers of
inflammation, as well as lack of progression of
angiographic
abnormalities, is seen in most patients who
receive glucocorticoid therapy.

25. Many patients with TA experience relapses of
symptoms or progression of vascular disease that
necessitate restarting high-dose prednisone therapy.
 resistant to steroids/ recurrent disease once
corticosteroids are tapered
cyclophosphamide (1-2 mg/kg/day), not preffered
choice in young females.
azathioprine (1-2mg/kg/day), or
methotrexate (0.3 mg/kg/week)
Mycophenolate mofetil/ anti TNF α agents-
infliximab

26.  Tricky in patients with extensive Takayasu
arteritis.
 The physician must accept compromises in
blood pressures That sustain perfusion of
critical organs or tissues.

27.  Pregnancy does not alter disease character
 Complications of HTN mainly affect.
 Outcome usually favourable
 Management of HTN essential
 Measurement of BP in UL impossible/unreliable
more accurate in legs
 HTN in 2nd stage Labour –risk for ICH-shortening
stage .
 pre-eclampsia, CCF, progressive RF,CVA
 Fertility not affected

28. According to new reseaches 13-fold higher rate of
obstetric complications compared to pregnancies
before TA diagnosis
Obstetric complications: 40% including pree
clampsia/eclampsia , premature delivery and
intrauterine fetal growth restriction or death.
Maternal complications: 39% of pregnancies and
included mainly new-onset or worsening
hypertension.

29.  Antihypertensive agents that are safely used
with proven success in pregnancy are labetolol,
hydralazine, and alpha-methyldopa