Takayasu arteritis

Takayasu aortoarteritis [Ref Pediatric Nephrology by Bagga and Srivastava 4⁷¹ⁱ/e p 132 Ghai 6^1h/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 

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.

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

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

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.

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)

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

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

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 (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

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

Answer is C (Takayasu Arteritis):

Takayasu arteritis is also known as ‘Reversed Coarctation’.

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%)

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

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.

Ans. Polyarteritis nodosa

Ans. is ‘c’ i.e., Takayasu arteritis

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.

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

Ans is D

 This condition 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. Nonspecific Aortoarteritis
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