Takayasu arteritis

Takayasu arteritis

Q. 1

Most common causeof renal artery stenosis in children in India is

 A

Takayasu Aortoarteritis

 B

Fibromedial hypertrophy

 C

Fibrointimal hyperplasia

 D

Polyarteritis Nodosa

Q. 1

Most common causeof renal artery stenosis in children in India is

 A

Takayasu Aortoarteritis

 B

Fibromedial hypertrophy

 C

Fibrointimal hyperplasia

 D

Polyarteritis Nodosa

Ans. A

Explanation:

Takayasu aortoarteritis [Ref Pediatric Nephrology by Bagga and Srivastava 471i/e p 132 Ghai 61h/e p 433; http//www.ncbi.nlm.gov/pmc Annals of pediatric cardiology department of cardiology AIIMS, Ishwarchadra malav and SS kottani]

The most common cause of renal artery stenosis in children is Takayasu’s Arteritis 



Q. 2 Takayasu arteritis commonly effects:
 A Coronary arteries
 B Spleenic artery
 C Radial artery
 D Renal arteries
Q. 2 Takayasu arteritis commonly effects:
 A Coronary arteries
 B Spleenic artery
 C Radial artery
 D Renal arteries
Ans. D

Explanation:

Renal arteries


Q. 3

All of the following condition are associated with granulomatous pathology, EXCEPT:

 A

Wegner’s Granulomatosis (WG)

 B

Takayasu Arteritis (TA)

 C

Polyarteritis Nodosa (Classic PAN)

 D

Giant Cell Arteritis (GCA)

Q. 3

All of the following condition are associated with granulomatous pathology, EXCEPT:

 A

Wegner’s Granulomatosis (WG)

 B

Takayasu Arteritis (TA)

 C

Polyarteritis Nodosa (Classic PAN)

 D

Giant Cell Arteritis (GCA)

Ans. C

Explanation:

Classic PAN is characterised by segmental transmural necrotizing inflammation of small to medium sized arteries in all organs except lung.
 
Ref: Robbin’s Basic Pathology, 7th Edition, Page 350, 347 – 352.

Q. 4

A 22 year old female Asian immigrant presents with complaints of malaise, fever, arm pain, loss of appetite, and visual problems. Her mother states that she fainted 1 week ago. The physician cannot palpate the patient’s lower extremity pulses and notes that the dial pulses are weak. The erythrocyte sedimentation rate (ESR) is elevated. Which of the following is the most likely diagnosis?

 A

Buerger’s disease

 B

Kawasaki’s disease

 C

Takayasu’s arteritis

 D

Thrombophlebitis

Q. 4

A 22 year old female Asian immigrant presents with complaints of malaise, fever, arm pain, loss of appetite, and visual problems. Her mother states that she fainted 1 week ago. The physician cannot palpate the patient’s lower extremity pulses and notes that the dial pulses are weak. The erythrocyte sedimentation rate (ESR) is elevated. Which of the following is the most likely diagnosis?

 A

Buerger’s disease

 B

Kawasaki’s disease

 C

Takayasu’s arteritis

 D

Thrombophlebitis

Ans. C

Explanation:

Takayasu’s arteritis is an uncommon disorder that is most prevalent in young Asian women. In Takayasu’s, an inflammatory process produces fibrous thickening of the aortic arch, causing narrowing or near obliteration of the origins of arteries that branch from the arch. Absent or diminished pulses are noted, especially in the upper extremities.
Involvement of the carotid circulation can produce ocular disturbances or other neurological dysfunction (syncope or, less commonly, stroke).
 
Buerger’s disease (thromboangiitis obliterans is a disease of young-to middle-aged adult (predominantly males) smokers. It is characterized by segmental thrombosis of arteries and veins, often in extremities.
 
Kawasaki’s disease is usually seen in children, and is characterized by rash, fever, conjunctivitis, and lymphadenopathy. Coronary vasculitis and aneurysms may occur.
Thrombophlebitis usually involves the deep veins of the legs and often occurs in association with predisposing factors such as immobilization, clotting disorders, heart disease, cancer, pregnancy, or tissue injury.
 
Ref: Wyatt C., Butterworth IV J.F., Moos P.J., Mackey D.C., Brown T.G. (2008). Chapter 9. Vascular Pathology. In C. Wyatt, J.F. Butterworth IV, P.J. Moos, D.C. Mackey, T.G. Brown (Eds), Pathology: The Big Picture.

Q. 5

Most common cause of renovascular hypertension in children in India is:

 A

Takayasu Aortoarteritis

 B

Fibromedial hypertrophy

 C

Fibrointimal hyperplasia

 D

Polyarteritis nodosa

Q. 5

Most common cause of renovascular hypertension in children in India is:

 A

Takayasu Aortoarteritis

 B

Fibromedial hypertrophy

 C

Fibrointimal hyperplasia

 D

Polyarteritis nodosa

Ans. A

Explanation:

Takayasu’s arteritis is believed to be the commonest cause of renovascular hypertension in India.

 

 

 

Takayasu’s arteritis most often involves the aorta and its major branches, although the pulmonary arteries may be affected. Involvement of the renal artery may cause renovascular hypertension. Takayasu’s arteritis is most common in Asia, and rarely occurs in patients older than 40 years. Clinically, it often presents with diminished pulses, vascular bruits, claudication, and renovascular hypertension. 

 

 

 

Ref: Essential paediatrics by OP Ghai, 6th edition, Page 586

 


Q. 6

Aortic arch syndrome is also known as:

 A

Takayasu’s arteritis

 B

Kawasaki disease

 C

Churg Strauss syndrome

 D

Giant cell areritis

Q. 6

Aortic arch syndrome is also known as:

 A

Takayasu’s arteritis

 B

Kawasaki disease

 C

Churg Strauss syndrome

 D

Giant cell areritis

Ans. A

Explanation:

Takayasu’s arteritis is an inflammatory and stenotic disease of medium and large-sized arteries characterized by a strong predilection for the aortic arch and its branches.

For this reason, it is often referred to as the aortic arch syndrome.

Ref: Harrison’s principle of internal medicine 17th edition, chapter 319.


Q. 7

Large vessel vasculitis is/are:

 A

Takayasu disease

 B

Temporal arteritis

 C

PAN

 D

a and b

Q. 7

Large vessel vasculitis is/are:

 A

Takayasu disease

 B

Temporal arteritis

 C

PAN

 D

a and b

Ans. D

Explanation:

Ans. is ‘a’ i.e., Takayasu disease; ‘b’ i.e., Temporal arteritis

Large vessel vasculitis are Giant cell arteritis, Takayasu arteritis and Cogan syndrome.


Q. 8

All of the following condition are associated with granulomatous pathology, except –

 A

Wegner’s granulomatosis (WG)

 B

Takayasu arteritis (TA)

 C

Polyarteritis nodosa (Classic PAN)

 D

Giant cell arteritis (GCA)

Q. 8

All of the following condition are associated with granulomatous pathology, except –

 A

Wegner’s granulomatosis (WG)

 B

Takayasu arteritis (TA)

 C

Polyarteritis nodosa (Classic PAN)

 D

Giant cell arteritis (GCA)

Ans. C

Explanation:

Ans. is ‘c’ i.e., Polyarteritis nodosa (classic PAN)

o Granulomatous vasculitis is seen in giant cell arteritis (temporal arteritis), Takayasu’s arteritis, wegener’s granulomatosis, churg strauss syndrome.


Q. 9

Pulseless disease is –

 A

Giant cell arteritis

 B

Takayasu arteritis

 C

Kawasaki disease

 D

Polyarteritis nodosa

Q. 9

Pulseless disease is –

 A

Giant cell arteritis

 B

Takayasu arteritis

 C

Kawasaki disease

 D

Polyarteritis nodosa

Ans. B

Explanation:

Ans. is ‘b’ i.e., Takayasu arteritis

Takayasu arteritis

o Takayasu arteritis is gran u lomatous vasculitis of large and medium arteries.

o It is characterized principally by ocular disturbance and marked weakening of pulses in the upper extremities —> Pulseless disease.

o It is also characterized by a strong predilection for aortic arch and its branches —> Aortic arch syndrome. o It is more common in Adolescent girls and young women.

o Morphological changes in vessel wall are same as in giant cell arteritis.

Clinical presentation

  • Symptoms are mainly according to the involved vessel –
  • Subclavian artery :

o Most commonly involved vessel

o Symptoms are – Arm claudication, lower BP and weaker pulse in upper limb then in lower limb, Raynaud’s phemomenon.

 Common carotid -+ Visual disturbances, stroke, TIA, syncope.

 Abdominal aorta or coeliac artery or superior mesentric artery —> Abdominal pain, nausea, vomiting

 Renal                                         –>           Hypertension, Renal failure

 Aortic root                                 —>         Aortic regurgitation, CHF

 Vertebral                                                   Visual disturbances

  Iliac                                            –               Leg claudication

 Pulmonary                                —>         Pulmonary hypertension, dysnea, chest pain.

 Coronary                                                   Angina, MI

  Constitutional symptoms      —>         As with other vasculitis malaise, fatigue, anarexia and weight loss are common.

 Elevated ESR and Anemia (as with other vasculitis)


Q. 10

Most common cause of renal artery stenosis in children in India is –

 A

Takayasu Aortoarteritis

 B

Fibromedial hypertrophy

 C

Fibrointimal hyperplasia

 D

Polyarteritis Nodosa

Q. 10

Most common cause of renal artery stenosis in children in India is –

 A

Takayasu Aortoarteritis

 B

Fibromedial hypertrophy

 C

Fibrointimal hyperplasia

 D

Polyarteritis Nodosa

Ans. A

Explanation:

Ans. is ‘a’ i.e., Takayasu aortoarteritis


Q. 11

All of the following factors predispose to Aortic dissection, Except:

 A

Systemic hypertension

 B

Coarctation of aorta

 C

In Ist trimester pregnancy

 D

Takayasu’s arteritis

Q. 11

All of the following factors predispose to Aortic dissection, Except:

 A

Systemic hypertension

 B

Coarctation of aorta

 C

In Ist trimester pregnancy

 D

Takayasu’s arteritis

Ans. C

Explanation:

Answer is C (In 1st trimester pregnancy)

Aortic dissection is predisposed during Third trimester of pregnancy and not during the first trimester of pregnancy Risk factors / Predisposing factors for Aortic Dissection

  • Systemic Hypertension and cystic medial necrosis
  • Connective Tissue Disorders

–         Marfan’s Syndrome

–         Ehler’s Danlos Syndrome

  • Inflammatory Aortititis

–         Takayasu arteritis

–         Giant cell arteritis

  • Congenital Aortic Valve abnormalities

–         Bicuspid Aortic Valve

  • Coarctation of Aorta
  • Aortic trauma
  • Third trimester of pregnancy

Q. 12

A young female presents with diminished pulses in the upper limb and hypertension. The most likely diagnosis is:

 A

Takayasu Arteritis

 B

Coarctation of Aorta

 C

Giant Cell Arteritis

 D

Polyarteritis Nodosa

Q. 12

A young female presents with diminished pulses in the upper limb and hypertension. The most likely diagnosis is:

 A

Takayasu Arteritis

 B

Coarctation of Aorta

 C

Giant Cell Arteritis

 D

Polyarteritis Nodosa

Ans. A

Explanation:

Answer is A (Takayasu Arteritis):

Diminished pulses in upper extremities and hypertension in a young female suggests a diagnosis of Takayasu arteritis. Coarctation is associated with diminished pulses in the lower extremities and Hypertension.

Takayasu arteritis is typically prevalent in adolescent girls and young women (<40 years) and is more common in Asia. Obstruction at the level of proximal branches of the aortic arch (Subclavian and Common Carotids) leads to diminished pulses in the upper extremity (Reversed Coarctation). Hypertension is a major complication of the disease and most commonly arises from renal artery stenosis. Since upper limb pulses are typically difficult to feel it may be dcult to record the blood pressure and hypertension may be easily missed. High blood pressure may be recorded in the lower limbs.

1990 criteria of American College of Rheumatology for the classification of Takayasu arteritis

Criteria

Definition

Age at disease onset in year

Development of symptoms or findings related to Takayasu arteritis at age <40 years.

Claudication of extremities

Development and worsening of fatigue and discomfort in muscles of one or more extremity

while in use, especially the upper extremities.

Decreased brachial artery pulse

Decreased pulsation of one or both brachial arteries

BP difference >10mmHg

Difference of >10mmHg in systolic blood pressure between arms

Bruit over subclavian arteries or aorta

Bruit audible on auscultation over one or both subclavian arteries or abdominal aorta

Arteriogram abnormality

Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large

arteries in the proximal upper or lower extremities, not due arteriosclerosis, fibro-muscular

dysplasia, or similar causes: changes usually focal or segmental

Takayasu arteritis is diagnosed if at least three of six criteria are present.


Q. 13

Reversed Coarctation is seen in:

 A

Giant cell Arteritis

 B

Polyarteritis Nodosa

 C

Takayasu Arteritis

 D

Kawasaki Disease

Q. 13

Reversed Coarctation is seen in:

 A

Giant cell Arteritis

 B

Polyarteritis Nodosa

 C

Takayasu Arteritis

 D

Kawasaki Disease

Ans. C

Explanation:

Answer is C (Takayasu Arteritis):

Takayasu arteritis is also known as ‘Reversed Coarctation’.

Condition

Coarctation of Aorta

Takayasu Arteritis (Reversed Coarctation)

Pathology (Site of Obstruction)

Obstruction is most commonly found just distal to the origin of the left Subclavian artery thereby sparing the upper limb vessels

Obstruction is most commonly seen in proximal aspect of branches of the aortic arch including the Subclavian and Common Carotid thereby affecting the upper limb vessels

Pulses

Absence or diminished pulse in the lower limbs

Absence or diminished pulse in the upper limbs

Blood Pressure

Increased blood pressure in the upper limbs

Decreased blood pressure in the upper limbs


Q. 14

Takayasu arteritis mainly affects?

 A

Pulmonary artery

 B

Celiac artery

 C

Subclavian artery

 D

SMA

Q. 14

Takayasu arteritis mainly affects?

 A

Pulmonary artery

 B

Celiac artery

 C

Subclavian artery

 D

SMA

Ans. C

Explanation:

Answer is C (Subclavian Artery):

Subclavian artery is the single most common artery involved in Takayasu arteritis.

Takayasu arteritis : Most common sites affected

  • Takayasu arteritis typically involves medium and large sized arteries
  • It has a strong predilection for the aortic arch and its branches.
  • The involvement of the major branches of the aorta is much more marked at their origin than distally

The most commonly affected arteries as seen by arteriography in order of frequency

 

  • Coronary (<10%)
  • Vertebral (35%)
  • Coeliac axis (18%)
  • Pulmonary (10-40 %)
  • Superior Mesenteric (18%)
  • Iliac (17%)
  • Subclavian (93%)
  • Common Carotid (58%)
  • Abdominal Aorta (47%)
  • Renal (38%)

 


Q. 15

Renal artery stenosis may occur in all of the following, except :

 A

Atherosclerosis

 B

Fibromuscular dysplasia

 C

Takayasu’s arteritis

 D

Polyarteritis nodosa

Q. 15

Renal artery stenosis may occur in all of the following, except :

 A

Atherosclerosis

 B

Fibromuscular dysplasia

 C

Takayasu’s arteritis

 D

Polyarteritis nodosa

Ans. D

Explanation:

Answer is D (Polyarteritis nodosa)

Amongst the options provided renal artery stenosis is least likely to be seen in association with Polyarteritis nodosa.

Atherosclerosis and Fibromuscular disease

–   Renal artery stenosis is produced predominantly by atherosclerotic occlusive disease (80% to 90% of patients) or tibromuscular dysplasia (10-15% of patients).- CMDT’06 p 460

–  he common cause of renal artery stenosis in the middle aged and elderly is an atheromatous plaque at origin of renal artery. Harrison

  In younger women stenosis is due to intrinsic structural abnormalities of the arterial wall caused by a heterogenous group of lesions called ‘fibromuscular dysplasia’. – Harrison 16`” /1707

PAN or Takayasu arteritis ? 

CMDT do not mention PAN or Takayasu arteritis as a cause for renal artery stenosis.

However, Takayasu arteritis is certainly a more common cause of renal artery stenosis than PAN.

Takayasu arteritis (Aorto-arteritis) is beleiveds to be the most common of renovascular hypertension in India and China. – Diseases of Kidney and Urinary Tract 8th/1279

A number of research publications, however show Takayasu arteritis as a cause for renal artery stenosis.

PAN

Although renal involvement is seen in upto 60% of patients with PAN its pathology does not involve renal artery stenosis.

The pathology involves arteritis without glomerulonephritis and is characterised by aneurysms of small and medium sized arteries. Stenosis of main renal artery is an unusual association’.

Causes of Renal artery stenosis :

  1. Atherosclerosis
  2. Fibromuscular dysplasia
  3. Non specific Aorto arteritis
  4. Takayasu arteritis and giant cell arteritis (Takayasu > Giant cell)
  5. Antiphospholipid syndrome
  6. Transplant renal artery stenosis
  7. Renal artery embolism
  8. Dissecting aneurysm of aorta
  9. Radiation arteritis

Q. 16

Renal artery stenosis is not associated with:

March 2009

 A

Marfan’s syndrome

 B

atherosclerosis

 C

polyarteritis nodosa

 D

takayasu arteritis

Q. 16

Renal artery stenosis is not associated with:

March 2009

 A

Marfan’s syndrome

 B

atherosclerosis

 C

polyarteritis nodosa

 D

takayasu arteritis

Ans. A

Explanation:

Ans. A: Marfan’s syndrome

The most common cause of renal artery stenosis is an atheromatous plaque.

Marfan Syndrome:

  • Marfan syndrome (MFS) is a spectrum of disorders caused by a heritable genetic defect of connective tissue that has an autosomal dominant mode of transmission.
  • The defect itself has been isolated to the FBN1 gene on chromosome 15, which codes for the connective tissue protein fibrillin. Abnormalities in this protein cause a myriad of distinct clinical problems, of which the musculoskeletal, cardiac, and ocular system problems predominate.
  • The skeleton of patients with MFS typically displays multiple deformities including arachnodactyly (ie, abnormally long and thin digits), dolichostenomelia (ie, long limbs relative to trunk length), pectus deformities (ie, pectus excavatum and pectus carinatum), and thoracolumbar scoliosis.
  • Finally, blindness may result from unrecognized and untreated glaucoma, retinal detachment, and cataracts.
  • In the cardiovascular system, aortic dilatation, aortic regurgitation, and aneurysms are the most worrisome clinical findings. Mitral valve prolapse that requires valve replacement can occur as well.
  • Ocular findings include myopia, cataracts, retinal detachment, and superior dislocation of the lens.

Takayasu arteritis is also known as pulseless disease because of frequent occlusion of arteries arising from the aorta.It may also occlude renal arteries.

Polyarteritis nodosa is a multisystem necrtizing vasculitis of small and medium sized muscular arteries in which involvement of the renal and visceral arteries is characteristic.There is fibrinoid necrosis with compromise of the lumen.


Q. 17

Renal artery stenosis may occur in all of the following, except:

 A

Atherosclerosis

 B

Fibromuscular dysplasia

 C

Takayasu’s arteritis

 D

Polyarteritis nodosa

Q. 17

Renal artery stenosis may occur in all of the following, except:

 A

Atherosclerosis

 B

Fibromuscular dysplasia

 C

Takayasu’s arteritis

 D

Polyarteritis nodosa

Ans. D

Explanation:

Ans. Polyarteritis nodosa


Q. 18

Reversed Coarctation is seen in

 A

Giant cell arteritis

 B

Polyarteritis nodosa

 C

Takayasu arter

 D

Polyarteritis nodosa

Q. 18

Reversed Coarctation is seen in

 A

Giant cell arteritis

 B

Polyarteritis nodosa

 C

Takayasu arter

 D

Polyarteritis nodosa

Ans. C

Explanation:

Ans. is ‘c’ i.e., Takayasu arteritis


Q. 19

Incorrect about takayasu arteritis

 A

Spares pulmonary artery

 B

Renovascular hypertension

 C

Blood pressure difference between left and right limbs

 D

Strongly positive mantoux

Q. 19

Incorrect about takayasu arteritis

 A

Spares pulmonary artery

 B

Renovascular hypertension

 C

Blood pressure difference between left and right limbs

 D

Strongly positive mantoux

Ans. A

Explanation:

Ans. is ‘a’ i.e., Spares pulmonary artery

Takayasu arteritis

  • It is granulomatous vasculitis affecting medium and larger arteries.
  • The disease has a strong predeliction for the aortic arch and its branches. C.) Takayasus arteritis classically involves the “aortic arch”.

Histological changes

Transmural involvement (all three layers are involved).

Media               →       Mononuclear infiltrates to granulomatous inflammation with giant cells

Adventitia         →       Mononuclear infiltrates

Vasa vasorum   →       Perivascular cuffing

  • As the disease progresses collagenous scarring with admixed chronic infiltrates occur in all three layers of the vessel wall.
  • Narrowing of the lumen occurs with or without thrombosis pathological changes in various organs reflect the compromise of blood flow through the involved vessels.
  • Aortic lesions of Takayasu arteritis share many attributes with Giant cell arteritis including clinicalfeatures and histology.

The distinction is typically made on the basis of the age of the patient

> 50 years       →          Giant cell Arteritis

< 50 yrs          →          Takayasu disease

Clinical features of Takayasu’s Arteritis :‑

o Nonspecific features of Vasculitis

  • Fatigue, weight loss and fever
  • Vascular symptoms (Due to luminal narrowing of the aortic arch).
  • Marked weakening of pulses in upper extremities It is also knowns as “pulseless disease”.
  • Reduced blood pressure in upper extremitiese
  • Ocular disturbances
  • Visual defects
  • Retinal hemorrhages
  • Total blindness
  • Takayasu disease can also cause Myocardial infarction.
  • Due to narrowing of the coronary arteries.
  • Hypertension in 50% cases
  • Narrowing of renal arteries lead to hypertension in half the cases.
  • Diminished pulsein upper extremities and hypertension in a young female suggests a diagnosis of Takayasu arteritis.

Criteria

Definition

Age at disease onset in year

Development of symptoms of findings related to Takayasu arteritis at age < 40 years

Claudication of examination

Development and worsening of fatigue and discomfort in muscles of one or more extremity while in use, especially the upper extremities.

 Decreased brachial artery pulse  Decreased pulsation of one or both brachial arteries

BP differences> 10 mmHg

 Difference of> 10 mmHg in systolic blood pressure between arms

Bruitover subclavian arteries or aorta

Bruit audible on auscultation over one or both subclavian arteries or abdominal aorta

Arteriogram abnormality


Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not due arteriosclerosis, fibro-muscular dysplasia, or similar causes: changes usually focal or segmental 

Takayasu arteritis is diagnosed if at least three of six criteria are present.


Q. 20

This condition as shown in image  may occur in all of the following, except

 A

Atherosclerosis

 B

Fibromuscular dysplasia

 C

Takayasu’s arteritis

 D

Polyarteritis nodosa

Q. 20

This condition as shown in image  may occur in all of the following, except

 A

Atherosclerosis

 B

Fibromuscular dysplasia

 C

Takayasu’s arteritis

 D

Polyarteritis nodosa

Ans. D

Explanation:

Ans is D

 This conditition  is Renal artery stenosis

Amongst the options provided renal artery stenosis is least likely to be seen in association with Polyarteritis nodosa.

Causes of Renal artery stenosis :

  1. Atherosclerosis
  2. Fibromuscular dysplasia
  3. Non specific Aorto arteritis
  4. Takayasu arteritis and giant cell arteritis (Takayasu > Giant cell)
  5. Antiphospholipid syndrome
  6. Transplant renal artery stenosis
  7. Renal artery embolism
  8. Dissecting aneurysm of aorta
  9. Radiation arteritis




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