Question

A 12 year old boy presented with weak pulses in upper limbs. His BP was 90/60 mmHg. He also had retinal haemorrhages. Most likely diagnosis is

• A. PAn
• B. Microscopic polyangiitis
• C. Takayasu arteritis
• D. HSP

Hint:

Think of the large-vessel vasculitis known as pulseless disease affecting young patients.

Answer 1

The Correct Option is C

Step 1: Identify key clinical features. The patient is a 12-year-old boy with weak pulses in the upper limbs, hypotension (BP 90/60 mmHg), and retinal haemorrhages. These features point to large-vessel vasculitis affecting the aorta and its major branches.

Step 2: Understand Takayasu arteritis. Takayasu arteritis (also called pulseless disease or Martorell syndrome) is a chronic granulomatous large-vessel vasculitis predominantly affecting the aorta and its major branches. It typically affects young females and children. The hallmark is absent or weak peripheral pulses due to progressive stenosis and occlusion of vessels, particularly the subclavian arteries supplying the upper limbs.

Step 3: Eliminate other options. Polyarteritis nodosa (PAN) is a medium-vessel vasculitis that does not typically cause upper limb pulse deficits. Microscopic polyangiitis is a small-vessel vasculitis presenting mainly with glomerulonephritis and pulmonary haemorrhage. Henoch-Schonlein Purpura (HSP) is a small-vessel IgA vasculitis in children presenting with purpura, arthritis, abdominal pain, and renal involvement -- not pulseless disease.

Step 4: Confirm diagnosis. Weak upper limb pulses + hypotension + retinal haemorrhages in a young patient = Takayasu arteritis. Retinal haemorrhages occur due to occlusive retinopathy secondary to reduced blood flow from the affected carotid/vertebral arteries.