autoimmune disease

1. TAKAYASU’S ARTERITIS
Dr. Aishwarya

2. DEFINITION
TAKAYASU’S ARTERITIS is an inflammatory and
stenotic disease of medium and large sized
arteries characterised by a strong prediliction for
the aorta and its branches .
AORTIC ARCH SYNDROME
PULSELESS DISEASE.

3. HISTORY
Dr. Takayasu described the retinal changes of
the disease in 1905 at the Annual
Ophthalmology Society meeting in Japan, and
his abstract was subsequently published in
1908. The condition is now called Takayasu
arteritis in his honor.

4. INCIDENCE AND PREVALENCE
Uncommon disease.
Annual incidence rate 1.2-2.6 cases/million
Prevalent in adolescent and young women
M.C. in Asia
No racial/geographical distribution.

5.  Race
Takayasu arteritis is observed more frequently in
patients of Asian or Indian descent. Japanese have a
higher incidence of aortic arch involvement. india report
higher incidences of abdominal involvement.
 Sex
 80% of patients - women; however, the high female-to-
male ratio seems to decrease west of Japan. In India, the
female-to-male ratio is as low as 1.6:1.
Age
Most patients are aged 4-63 years, mean age of onset -
30 years. <15% of cases present in individuals older than
40 years.

6. PATHOLOGY
Panarteritis,inflammmatory mononuclear
infiltrates,occasionally giant cells
Marked intimal proliferation ,fibrosis ,scarring
and vascularisation of media
disruption,degeneration of elastic lamina
Narrowing of lumen +/- thrombus

7. Pathology in the chronic phase of Takayasu’s Arteritis showing
fibrosis in all the layers of the vessel wall and markedly
thickened intima

8. Contd.
Immunopathologcial mechanism are
suspected ,uncertain
Circulating immune complexes-
demonstrated but their pathological
signficance is unclear.

9. 



10. Constitutional symptoms :
Headache (50%-70%)
Malaise (35%-65%)
Arthralgias (28%-75%)
Fever (9%-35%)
Weight loss (10%-18%)

11. Cardiac and vascular features
 Bruit, with the most common location being the carotid artery
(80%)
 Blood pressure difference of extremities (45%-69%)
 Claudication (38%-81%)
 Carotodynia or vessel tenderness (13%-32%)
 Hypertension (28%-53%; 58% with renal artery stenosis in one
series)
 Aortic regurgitation (20%-24%)
 Raynaud’s syndrome (15%) .Pericarditis (< 8%) ,Congestive
heart failure (< 7%) , Myocardial infarction (< 3%).

12. NEUROLOGICAL FEATURES
Headache (50%-70%)
Visual disturbance (16%-35%) - Strong
association with common carotid and
vertebral artery disease
Stroke (5%-9%)
Transient ischemic attacks (3%-7%)
 Seizures (0%-20%)

13. DERMATOLOGICAL
MANIFESTATIONS
Erythema nodosum (6%-19%)
Ulcerated subacute nodular lesions (< 2.5%)
Pyoderma gangrenosum (< 1%)

14. Particular attention to peripheral pulses, blood pressure
in all 4 extremities, and an ophthalmologic examination.
The most discriminatory finding is a systolic blood
pressure difference (>10 mm Hg) between arms.
Hypertension due to renal artery involvement is found in
approximately 50% of patients.
Absent or diminished pulses are the clinical hallmark of
Takayasu arteritis, but pulses are normal in many patients
and upper limbs are affected more often Than lower
limbs.

15. Physical examination contd…
 Carotidynia may be present.
 Bruits are often noted.
 Aortic regurgitation is a common finding.
 Ophthalmologic examination may show retinal
hemorrhages, cotton-wool exudates, venous dilatation
and beading, microaneurysms of peripheral retina, optic
atrophy, vitreous hemorrhage, and classic wreathlike
peripapillary arteriovenous anastomoses (extremely rare).
 Skin changes resembling erythema nodosum or ulcerating
nodular lesions may be seen.

16. Artery percent Clinical manifestations
1.Subclavian 93 ARM CLAUIDACTION ,RAYNAUD’S
PHENOMENON
2.Common
carotid
58 VISUAL
CHANGES,TIA,STROKE,SYNCOPE
3.Abdominal
aorta
47 ABDOMINAL PAIN,NAUSEA
,VOMITING
4.Renal 38 HTN,RENAL FAILURE
5.Aortic
arch/root
35 AORTIC INCOMPETENCE,CCF

17. ARTERY
% CLINICAL MANIFESTATIONS
1. VERTEBRAL 35 Visual changes
Dizziness
2. COELIAC AXIS 18 Abd pain
Nausea,vomiting
3. SUPERIOR MESENTRIC 18 Abd
pain,nausea,vomiting
4. ILIAC 17 Leg claudication
5. PULMONARY 10-40 Atyp chestpain,dyspnea
6. CORONARY <10 Chest pain,MI

18. DIFFERENTIAL DIAGNOSIS
 AORTIC COARCTATION
 ATHEROSCLEROSIS
 BEHCET’S DISEASE
 BUERGER’S DISEASE (THROMBANGITIS OBLITERANS)
 GIANT CELL ARTERITIS
 KAWASAKI DISEASE
 RHEUMATOID ARTHRITIS
 SARCOIDOSIS

19. AMERICAN COLLEGE OF
RHEUMATOLOGY – CLASSIFICATION
CRITERIA
 3 OF 6 NECESSARY
 1.Age < 40 yr onset
 2.Claudication of extremities
 3.Decreased pulsation in one/both brachial arteries
 4.Diff of at least 10mm of hg SBP between arms
5.Bruit over one/both sub cl art /abd aorta
6.Arteriografic narrowing/occlusion of entire abd
aorta,its primary br. Or large arteries of both UL and LL

20. LABORATORY STUDIES
The acute-phase reactants and clinical
parameters generally used to define active inflammatory
disease do not universally reflect active blood vessel
inflammation in Takayasu arteritis.
 The ESR is elevated in most but not all patients
during active inflammatory disease. Therefore, it is
not a consistently reliable marker of disease activity,
with both false-positive and false-negative
associations reported.]

21.  Tests:
 Arteriogram
 Angiogram
 Complete blood count (CBC)
 C-reactive protein (CRP)
 Electrocardiogram (ECG)
 Erythrocyte sedimentation rate (ESR)
 Magnetic resonance angiography (MRA)
 Magnetic resonance imaging (MRI)
 Ultrasound
 X-ray of the chest

22. Imaging Studies
While imaging studies (CT scanning, MRI) show typical
patterns of stenosis or aneurysms of the arteries,
angiography remains the standard for diagnosis and
evaluation of the extent of disease. recent studies
suggest that noninvasive imaging modalities such as
MRI, ultrasonography,and 18F-fluorodeoxyglucose
positron emission tomography (18F-FDG-PET) allow
diagnosis of Takayasu arteritis earlier in the disease
course than standard angiography and provide a means
for monitoring disease activity.
Angiography is used to evaluate only the appearance of
the lumen and cannot be used to differentiate between
active and inactive lesions.

23. Takayasu arteritis can be divided into 6 types based on
angiographic involvement, as follows:
Type I - Branches of the aortic arch
Type IIa - Ascending aorta, aortic arch, and its branches
Type IIb - Type IIa region plus thoracic descending aorta
Type III - Thoracic descending aorta, abdominal aorta,
renal arteries, or a combination
Type IV - Abdominal aorta, renal arteries, or both
Type V - Entire aorta and its branches

24.  Other tests
 MRA is equally or more sensitive than angiography for revealing
lesions in the aorta .
 CT helical scanning angiography is a sensitive and specific
diagnostic tool.
 Color Doppler ultrasonography provides details of the vascular
wall, lumen, and flow and is a useful tool for screening and follow-
up, particularly forcarotid and subclavian arteries.
 PET scanning with radioactive-labeled 18-fluorodeoxyglucose
(FDG) has been shown to be useful in monitoring disease activity
and response to treatment in preliminary studies. Presence or
absence of FDG uptake correlates well with clinical state and MRI
findings. Its use in patients with Takayasu arteritis requires further
investigation.
 MRA or CT angiography and FDG-PET may be useful to monitor
vascular response to treatment.

25. a. MRA b. Conventional angiogram

26. MEDICAL CARE
Assessing disease activity in patients with Takayasu arteritis
is frequently challenging, since clinical, biologic, and
radiologic information do not always correlate.
Prospective study criteria established by Kerr et al (NIH) are
used to assess disease activity in patients with Takayasu
arteritis. New onset or worsening of two or more of the
following features indicates active disease:
1.systemic features, such as fever and arthralgias (no
identified cause)
2. erythrocyte sedimentation rate
3.Features of vascular ischemia or inflammation, such as
claudication, diminished or absent pulse, bruit, carotodynia,
or asymmetric blood pressure in either upper or lower
limbs (or both)
4.Typical angiographic features

27. Treatment
Treatment of Takayasu arteritis is difficult, but
patients who do have the right treatment can see
positive results. Early detection is important.
Most patients are treated with steroids and
immunosuppressive drugs. Surgery is reserved for
complications caused by narrowed arteries.
Surgery to bypass narrowed arteries --
angioplasty or stent placement -- may be needed to
supply blood or open up the constriction.

28.  Therapeutic intervention
includes corticosteroid
therapy with or without
cytotoxic agents.

29. Corticosteroids are the mainstay of therapy for active
Takayasu arteritis, and some patients may require
additional cytotoxic agents to achieve remission and
taper of chronic corticosteroid treatment.
Oral corticosteroids are started at 1 mg/kg daily or
divided twice daily and tapered over weeks to
months as symptoms subside.
Long-term low-dose corticosteroid therapy may be
required.
Osteoporosis prevention when patients are started
on corticosteroids should be seriously considered

30. Cytotoxic agents are used for patients whose
disease is steroid-resistant or relapsing. These
agents are usually continued for at least one year
after remission and are then tapered to
discontinuation. The following agents with their
respective doses are as follows:
Methotrexate - 7.5-25 mg/wk oral
Azathioprine - 1-2 mg/kg/d oral
Cyclophosphamide - 2 mg/kg/d oral (should be
reserved for patients with the most severe and
refractory disease states)

31. Strict management of traditional
cardiovascular risk factors such as
dyslipidemia, hypertension, and lifestyle
factors is mandatory to minimize secondary
cardiovascular complications, which are the
major cause of death in this disease.
Additionally, low-dose aspirin may have a
therapeutic effect in large vessel vasculitis.
•Strict management of traditional cardiovascular risk factors such as dyslipidemia, hypertension, and lifestyle factors is mandatory to minimize secondary cardiovascular complications, which are the major cause of death in this disease. Additionally, low-dose aspirin ma

32. ANTI –TNF AGENTS
 In an uncontrolled series of 15 patients, adjunctive treatment with
anti–tumor necrosis factor (TNF) agents was effective in patients with
active, relapsing Takayasu arteritis despite treatment with steroids and
multiple other immunosuppressive agents.
 The initial dose of etanercept was 25 mg twice weekly (7 patients),
and infliximab (11 patients [3 were switched from etanercept to
infliximab]) was given at 3 mg/kg initially and at 2 weeks, 6 weeks, and
every 8 weeks thereafter. I
 In 9 of the 14 responders, an increase in the anti-TNF dosage was
required to sustain remission.
 The preliminary results suggest that anti-TNF therapy may be a require
further studies. A larger randomized controlled study of anti-TNF
therapy for Takayasu arteritis is puseful adjunct to corticosteroids in
the treatment of patients with Takayasu arteritis and Planned

33. SURGICAL CARE Critical stenotic lesions should be treated by angioplasty or surgical
revascularization during periods of remission.
 Indications for surgical repair or angioplasty are as follows:
1.Renovascular stenosis causing hypertension
2.Coronary artery stenosis leading to myocardial ischemia
3.Extremity claudication induced by routine activity
4.Cerebral ischemia and/or critical stenosis of 3 or more cerebral
vessels
5.Aortic regurgitation
6.Thoracic or abdominal aneurysms larger than 5 cm in diameter
7.Severe coarctation of the aorta
8.Percutaneous transluminal coronary angioplasty is followed by
restenosis at the angioplasty site within 1-2 years in a substantial
number of patients.
9.Bypass graft procedures have the best long-term patency rates

34. Response Of Aortitis To
Treatment

35. MORTALITY/MORBIDITY
 Takayasu arteritis is a chronic relapsing and remitting disorder.
 10-year survival rate is approximately 90%; however, this rate is
reduced in the presence of major complications.[6]
 Complications of Takayasu arteritis include valvular heart disease,
stroke, heart failure, retinopathy, and renovascular hypertension. The
5- and 10-year survival rates are approximately 69% and 36%,
respectively, in patients with two or more complications. The 5- and
10-year survival rates associated with one or fewer complications are
100% and 96%, respectively.
 A 2008 study assessing quality of life with Takayasu arteritis shows
worse scores for physical and mental health compared with many
other chronic diseases associated with peripheral vascular disease.
Disease remission is the only factor that positively influences both
physical and mental quality of life.Patients with RHEUMATOID
ARTHRITIS or rate their quality of life as similar to those with
Takayasu arteritis.

36.  In cortisone-resistant patients, stronger
medications which suppress the immune system
(immunosuppressive drugs), thereby further
decreasing active inflammation of the arteries,
have been used. Examples
include prednisone, prednisolone,methotrexate (R
heumatrex,
Trexall),cyclosporine, cyclophosphamide (Cytoxan)
, and azathioprine (Imuran). Strict control
ofelevated blood pressure (hypertension) is
important.

37. Pictured below is a close–up view of an
angiogram of the left vertebral and subclavian
arteries in a patient with Takayasu’s arteritis.
Note the narrowing and irregularities that
occur at several sites, and the “corkscrew”
configuration of one vessel segment near the
junction of the two arteries. These changes,
caused by inflammation in the blood vessel
wall, sometimes cause complete blockage of
the artery.

39. Pictured below is a normal aortic arch on
the left, with narrow, smooth blood
vessels. On the right is an example of an
abnormal aortic arch in a patient with
Takayasu’s, with obvious dilation of the
ascending aorta on the left side of the
picture

41. Although the lung involvement in
Takayasu’s is frequently overshadowed by
involvement of systemic large blood vessels,
the pulmonary arteries may also be affected
in this disorder. Pictured below is a
pulmonary angiogram demonstrating
beading and cut–off lesions of the right
pulmonary arteries, and a large aneurysm
of the left pulmonary artery.

43. Indian journal of ophthalmology,vol
50,2002.
Bilateral anterior ischaemic optic neuropathy
in Takayasu arteritis Abstract This report
describes a case of a young male with
bilateral, asymmetrical AION. He was
subsequently diagnosed with Takayasu's
disease


44. Publication of neurology society of
India,vol 49,2001.
 Single stage bilateral common carotid artery stenting in a patient
of Takayasu arteritis.
 » Abstract
Carotid angioplasty and stenting is increasingly becoming a safe
and efficacious modality of treatment in the management of
carotid artery stenosis. Although atherosclerosis is the
predominant cause of this morbid disease, Takayasu arteritis
assumes special importance in south east Asia. The diffuse nature
of this disease with associated inflammation and scarring of the
vessel make revascularisation difficult. We report a case of
Takayasu arteritis tid stenting was done in a single sitting.

45. JAPI, feb 2006
Coexistence of Takayasu’s Arteritis with Ulcerative
Colitis
Abstract
The association of ulcerative colitis with Takayasu’s
arteritis is rarely reported. The occurrence of the
two together is possibly related to a common
pathophysiology involving alteration in immune
mechanisms.
Takayasu's arteritis is more prevalent in Japan and
South East Asia whereas Ulcerative Colitis is more
inWestern countries. The coexistence of these two
diseases is uncommon and hence this report.

46. Clinical journal of immunology,vol
2009
 Research Article
 Lack of Antilipoprotein Lipase Antibodies in Takayasu's
Arteritis
 Abstract
 Background. Antilipoprotein lipase (anti-LPL) antibodies were
described in rheumatic diseases. In systemic lupus
erythematosus they were highly associated with
inflammatory markers and dyslipidemia, and may ultimately
contribute to vascular damage. The relevance of this
association in Takayasu's arteritis, which is characterized by
major inflammatory process affecting vessels, has not been
determined.

47. Pub MED.gov ,jul 9,2001
 [Takayasu's disease: interest in methotrexate treatment].
Abstract
 We report a case in which methotrexate proved to be
effective. CASE REPORT: A 6-year-old girl presented with
Takayasu's disease with elevated blood pressure of
renovascular origin. Corticosteroids controlled the
inflammatory syndrome but not the renal involvement and
stopped the growth. Methotrexate (10 mg/m2/week)
resulted in the control of the disease, the reduction of
steroids and normal growth. CONCLUSION: In Takayasu's
disease, steroids should be given as first-line therapy. In
case of failure, side effects or steroid dependency, small
doses of methotrexate may facilitate the disease's control
and weaning from the steroids

48. Oxford journal of
Rheumatology,nov 8,2005.
 Post-interventional immunosuppressive treatment and
vascular restenosis in Takayasu's arteritis
 Abstract
 Objective. To investigate the outcome of vascular interventions
and the effect of post-interventional immunosuppressive
treatment on the occurrence of vascular restenosis in patients
with Takayasu's arteritis (TA).
 Conclusion. Restenosis occurred in 31.7% of TA patients after
intervention. A lower restenosis rate was observed when the
vascular interventions were performed at the stable stage and
when post-interventional immunosuppressive treatment was
implemented.

49. American journal of Rheumatology,April
2008
Successful treatment of a patient with takayasu arteritis
using a humanized anti–interleukin-6 receptor antibody
Abstract
Takayasu arteritis (TA) is a chronic inflammatory disease
that involves the aorta and its major branches. Since
overproduction of interleukin-6 (IL-6) seems to play a
pathogenic role in TA, we used the anti–IL-6 receptor (IL-
6R) antibody tocilizumab to treat a 20-year-old woman
with refractory active TA complicated by ulcerative colitis
(UC). Treatment with tocilizumab improved the clinical
manifestations of TA and the abnormal laboratory findings
in this patient and ameliorated the activity of UC. These
results indicate that IL-6R inhibition with tocilizumab might
be a future treatment option for .

50. JOURNAL OF PAEDIATRICS,Sept
2008
Treatment of Takayasu's Arteritis with Tumor Necrosis
Factor Antagonists
Four children with Takayasu's arteritis were treated with
tumor necrosis factor antagonists because of disease
relapse during conventional therapy or as a first-line
agent. Two patients went into remission; in the other 2,
the response was partial. Anti-tumor necrosis factor
agents can have a role in the treatment of Takayasu's
arteritis; further controlled studies are required.

51. Curr Opin
Rheumatology.2005;17(1) 2005
Advances in the medical and surgical treatment
of Takayasu arteritis.
 SUMMARY: In the future, greater therapeutic
success may be achieved by addressing both the
inflammatory and the myointimal proliferative
components of Takayasu arteritis. New drugs
that target intimal hyperplasia, as well as drug-
eluting stents, deserve to be studied for possible
utility as adjuncts to present treatments.

52. Journal of invasive cardiology,Nov
2009
Covered Stent for the Treatment of Coronary
Aneurysm after Sirolimus-Eluting Stent
Implantation a Patient with Takayasu’s
Arteritis

ABSTRACT:
 Drug-eluting stents (DES) have been used
successfully to treat both de-novo and
restenotic lesions in these patients.

53. Curr Opin Rheumatology,2009
Advances in the use of biologic agents for the
treatment of systemic vasculitis.
 RECENT FINDINGS: The greatest amount of
experience with these agents for the treatment of
systemic vasculitis is with antitumor necrosis factor
agents, pooled intravenous immunoglobulin, and
anti-B-cell therapies such as rituximab. SUMMARY:
Biologic agents represent the next evolution in
treatment for the primary systemic vasculitides.

54. Clinical Rheumatology
journal.,2008
Mycophenolate mofetil in Takayasu’s arteritis
Abstract
Mycophenolate mofetil (MMf) has recently been
reported as a useful alternative immunosuppressive
drug in autoimmune diseases.This study is the
largest series till date establishing the use of
mycophenolate as a safe and effective steroid-
sparing immunosuppressant in Takayasu's arteritis

55. Indian jrnl of Paediatrics,july
2002.
Recent advances in the management of non-
specific aorto-arteritis.
 Today, percutaneous transluminar balloon
angioplasty (PTBA) has emerged as a non-surgical,
safe, effective alternative therapeutic option and
endovascular stents have revolutionised the
management. The disease must be suspected and
diagnosed precisely with echocardiography and
angiography before it is too late to manage.