Vasculitis & Alveolar Hemorrhage

Question 1

What is the former name for granulomatosis with polyangiitis?

Answer 1

Wegner’s

Question 2

What ANCA antibotidy & target are positive in granulomatosis with polyangiitis?

Answer 2

cANCA, targeting PR3

Question 3

What ANCA antibody & target are positive in microscopic polyangiitis?

Answer 3

pANCA, targeting MPO

Question 4

Symptoms of granulomatosis with polyangiitis? (7)

Answer 4

• Lung nodules
• Saddle nose
• Tongue ulcer
• Strawberry gums
• Skin lesions
• Lacrimal inflammation
• Subglottic tracheal stenosis.

Question 5

Overall treatment of vasculitic lung diseases?

Answer 5

• Induce remission with steroids + immunosuppressant

- Maitenance therapy with immunosuppressant

Question 6

BAL findings with DAH (Diffuse Alveolar Hemorrhage)?

Answer 6

• Serially bloodier aliquots

- Hemosiderin-laden macrophages > 20% (Prussian blue staining)

Question 7

Diagnosis of idiopathic pulmoary hemosiderosis?

Answer 7

surgical lung biopsy to exclude capalleritis

Question 8

Clinical picture of idiopathic pulmonary hemosiderosis?

Answer 8

• Recurrent hemoptysis & GGOs
• Iron-deficient anemia
• Bland histopathology

Question 9

Treatment of idiopathic pulmonary hemosdierosis?

Answer 9

Steroids + Immunosuppressant

Question 10

Treatment of DAH?

Answer 10

• Address underlying vasculitic disease

- Cytotoxic agents, plasma exchange

Question 11

What is Lane-Hamilton Syndrome?

Answer 11

Idiopathic pulmonary hemosiderosis + Celiac disease.

-Treated with gluten-free diet.