Vasculitis -Dr. Lee

Question 1

51 yo man admitted for 2 week history of fevers, night sweats, cough with hemoptysis, malaise. He works in agriculture, noticed lots of rat droppings. No recent travel or ill contacts
CXR: diffuse infiltrates and chest CT image follows

What do you do next…..?
A. ANA
B. ANCA
C. Anti-dsDNA
D. Rheumatoid factor (RF)

Answer 1

B. ANCA

Question 2

What is Behcet’s disease?

Answer 2

Recurrent aphthous oral ulceration (painful) plus two of the following:

• Recurrent genital ulceration
• Eye lesions (anterior/posterior uveitis or cells in vitreous or retinal vasculitis)
• Positive pathergy test

Question 3

What is the most common cause of Cryoglobulinemic Vasculitis?

Answer 3

Hepatitis C

Question 4

What is often the underlying cause of polyarteritis nodosa? What are some symptoms seen in polyarteritis nodosa?

Answer 4

Hepatitis B

need a biopsy to diagnose

• polyarteritis nodosa:
• weight loss, lived reticular is, testicular pain, myalgias or mm weakness, neuropathy, elevated BUN/creatinine, Hep B, small or medium artery biopsy with PMN
• multiple organs*

Question 5

What is the diagnostic criteria for Granulomatosis with Polyangiitis? (GPA) (also known as Wegener’s Granulomatosis)

Answer 5

Must have at least 2 of the 4 criteria present:

• Nasal/oral inflammation (oral ulcers or bloody nasal drainage) (upper respiratory disease)
• Abnormal chest radiograph (nodules, fixed infiltrates, cavities)(lung issues–> lower respiratory tract)
• Urinary sediment (>5 RBC/hpf or RBC casts) with glomerulonephritis (kidney involvement)
• Granulomatous inflammation and Giant Cells on biopsy (in wall of artery or arteriole, perivascular, or extravascular)

Question 6

What is the best tissue to biopsy to diagnose granulomatosis?

Answer 6

lung

Question 7

What is the only FDA drug available for GPA?

Answer 7

Rituximab

Question 8

What is Churg-Strauss Syndrome (CSS)?

Answer 8

Must have at least 4 of the 6 criteria present.

• Adult onset Asthma
• Peripheral Eosinophilia > 10%
• Neuropathy, mono or poly
• Pulmonary infiltrates, non-fixed
• Paranasal sinus abnormality
• Extravascular eosinophils

“Wegener’s with asthma, eosinophilia”

Question 9

What is Polymyalgia rheumatica (PMR)? What is the age of onset?

Answer 9

• Pelvic and shoulder girdle aching
• Morning stiffness
• Rapid response to low doses of corticosteriods
• Anemia
• Elevated ESR and C-reactive protein

blood vessels to the bursa causing inflammation

always patients who are older ** (>50-60)

most common vasculitis subtype that was mentioned

Question 10

How will PMR react to prednisone?

Answer 10

*immediate results

Initial 10-15 mg prednisone daily x 1 month

Question 11

What do most pts with GCA (giant cell arteritis) present with? How do we confirm diagnosis?

Answer 11

• new onset headache
• > 50 yo
• an also have elevated ESR and temporal artery physical abnormality

-temporal artery biopsy

Question 12

How are PMR and GCA related?

Answer 12

40-60% of pts with GCA have PMR symptoms

tx of GCA=LARGER dose of steroids than PMR does

Question 13

What is vasculitis? When should this be suspected?

Answer 13

Heterogeneous set of diseases characterized by inflammatory destruction of blood vessels

• suspect if:
• multisystem disease
• unexplained constitutional signs and symptoms
• inflammation
• skin lesions
• ischemic vascular changes
• mononeuritis multiplex
• pain

Question 14

What is henoch-schonlein purpura?

Answer 14

a red raised rash

responds well to steroids

Question 15

What is cryoglobulinemic vasculitis?

Answer 15

immunoglobulins undergo irreversible precipitation in the cold

-reaynauds–> ulceration and necrosis

Question 16

What clinical findings are seen with Wegener’s granulomatosis?

Answer 16

• saddle nose
• pulmonary nodules (lesions with fluid in it)
• cavitation
• initially present with upper airway/sinus/ear infection