Question

A patient presents with respiratory symptoms, that is, cough, hemoptysis, and glomerulonephritis. His c-ANCA levels in serum are found to be raised. The most likely diagnosis is

• A. Goodpasture's Syndrome
• B. Classic Polyarteritis Nodosa
• C. Wegener's Granulomatosis
• D. Kawasaki syndrome

Hint:

c-ANCA (anti-PR3) with lung and kidney disease points to Wegener's granulomatosis, unlike Goodpasture's which is anti-GBM antibody driven.

Answer 1

The Correct Option is C

The clue set here is cough, coughing up blood, kidney inflammation, and a positive c-ANCA test. The antibody type is the fastest way to sort the vasculitis options.

1. Goodpasture's Syndrome: This also causes bleeding into the lungs plus glomerulonephritis, but the antibody responsible attacks the glomerular basement membrane directly (anti-GBM antibody), not neutrophil proteinase 3, so a positive c-ANCA does not fit this diagnosis.
2. Classic Polyarteritis Nodosa: This vasculitis hits medium sized arteries, especially in the kidney, gut and skin, but it spares the lungs and does not cause glomerulonephritis, and it is usually ANCA negative, so it does not match.
3. Wegener's Granulomatosis: This is a small vessel vasculitis that is well known for attacking the upper airway, lungs and kidneys together, and c-ANCA (against proteinase 3) is its signature antibody. The cough, hemoptysis, glomerulonephritis and raised c-ANCA all point straight to this diagnosis, now called granulomatosis with polyangiitis.
4. Kawasaki syndrome: This is a childhood vasculitis of the coronary arteries with fever and mucocutaneous features, unrelated to ANCA and unrelated to glomerulonephritis.

Since the antibody is c-ANCA and the organs hit are lungs and kidneys, the disease that matches on both counts is Wegener's granulomatosis.

Let's summarize:

• c-ANCA (anti-PR3) links to Wegener's granulomatosis / GPA.
• p-ANCA (anti-MPO) links to microscopic polyangiitis and eosinophilic GPA, not this case.

The correct answer is Wegener's granulomatosis.