Vasculitis

Question 1

What symptoms are shared by all Vasculitides?

Answer 1

Fever
Malaise/fatigue
Weight loss
Arthralgia/myalgia

Question 2

What is Polyarteritis Nodosa (PAN)?

Answer 2

disease of small and medium arteries that spares the lungs

Question 3

What diseases are associated with PAN?

Answer 3

Chronic Hepatitis B and C

Question 4

What common systems are involved in PAN?

Answer 4

GI
Renal
Neurological
Skin

Question 5

What are common GI findings in PAN?

Answer 5

Abd pain worse with eating due to vasculitis of mesenteric vessels

bleeding, nausea/vomiting

Question 6

What are common Neurological findings in PAN?

Answer 6

Peroneal Neuropathy leading to food drop is most common

look for a stroke in a young patient

Question 7

What are common Skin findings in PAN?

Answer 7

Lower extremity ulcers: most common

Livedo Recitularis, Purpura, nodules, gangrene

Question 8

What does the term Mononeuritis Multiplex mean?

Answer 8

Multiple simultaneous peripheral neuropathies of nerves large enough to have a name: ex radial and peroneal nerves

Question 9

What is the most accurate test for PAN?

Answer 9

Biopsy of symptomatic site

Question 10

What is seen on Angiography in PAN?

Answer 10

Abnormally dilated “beading” of involved arteries
-commonly renal, mesenteric, hepatic

Question 11

What should all patients with PAN be tested for?

Answer 11

Hepatitis b and C

Question 12

What is seen on UA for PAN?

Answer 12

Nonspecific protein and RBC

Question 13

How do you treat PAN?

Answer 13

Prednisone + Cyclophosphamide

Treat Hepatitis if present

Question 14

How does Polymyalgia Rheuamtica present?

Answer 14

Age >50 with pain and stiffness in the neck, shoulder and pelvic girdle
-difficulty arising from chair and issues combing hair

Question 15

What are the diagnostic testing options for Polymyalgia Rheuamtica?

Answer 15

Elevated ESR
Normochromic, Normocytic Anemia
No muscle destruction despite pain
Normal CPK and Aldolase

Question 16

How is Polymyalgia Rheumatic Treated?

Answer 16

Steroids

Question 17

What is another name for Giant Cell Arteritis?

Answer 17

Temporal Arteritis

Question 18

How does Giant Cell Arteritis present?

Answer 18

Visual symptoms
jaw claudication: pain when chewing
Scalp Tenderness
Headache
Decreased arm pulses, bruit near clavicle, AR

Question 19

What does Giant cell Arteritis lead to?

Answer 19

Large Vessel Aneurysm and Aortic Aneurysm

Question 20

What ist he most accurate test for Giant Cell Arteritis?

Answer 20

Biopsy of the affected artery

Question 21

How is Giant Cell arteritis treated?

Answer 21

Steroids: start right away before biopsy

Question 22

What medication is used to get patients off steroids in Giant Cell Arteritis?

Answer 22

Tociluzumab: anti-IL6

Question 23

What is another name for Granulomatosis with Polyangittis (GPA)?

Answer 23

Wegener Granuloamtosis

Question 24

How does GPA present?

Answer 24

Combination upper and lower respiratory tract findings with renal insufficiency

Question 25

What are common symptoms of GPA?

Answer 25

Sinusitis or nasal perforation Otitis media Mastoiditis Oral and Gingival Involvement Skin, joint and eye lesions

Question 26

What is the best initial test for GPA?

Answer 26

C-ANCA: anti-proteinase 3 (PR3) Antibodies

Question 27

What vasculitis is (+) for C-Anca?

Answer 27

GPA

Question 28

What vasculitides are (+) for P-Anca?

Answer 28

Churg-strauss and microscopic polyangiitis

Question 29

What is the most accurate test for GPA?

Answer 29

Biopsy -lung biopsy is best -renal biopsy okay -sinus biopsy is least accurate

Question 30

How do you treat GPA?

Answer 30

Prednisone and Rituximab

Question 31

What can be used as an alternative to Rituximab in GPA and Microscopic Polyangiitis?

Answer 31

Cyclophosphamide

Question 32

What is a clinical clue to lead you to the diagnosis of GPA?

Answer 32

Nonresolving Pneumonia that fails to improve with Antibiotics

Question 33

What is another name for Eosinophilic Granulomatosis with Polyangittis?

Answer 33

Churg-Strauss

Question 34

How does Churg-Strauss present?

Answer 34

Pulmonary-renal syndrome

Question 36

What are common symptoms in Churg-Strauss?

Answer 36

Asthma, Eosinophilia fever, weight loss, joint pain, skin findings: nonspecific

Question 37

What medication is associated with Churg-Strauss?

Answer 37

Zafirlukast

Question 38

What is an initial test for Churg-Strauss?

Answer 38

P-ANCA: anti-myeloperoxidase (MPO) Ab

Question 39

What is the most accurate test for Churg-Strauss?

Answer 39

Biopsy

Question 40

How do you treat Churg-Strauss?

Answer 40

Prednisone + Cyclophosphamide

Question 41

What can be used to inhibit eosinophils with chronic steroids in Churg-Strauss?

Answer 41

IL-Inibitors: Benralizumab or Mepolizumab

Question 42

What does Cyclophosphamide increase the risk of?

Answer 42

Bladder bleeding and bladder cancer

Question 43

What is Microscopic Polyangiitis?

Answer 43

Systemic Vasculitis involving the lung, kidney, skin, joint, CNS and GI

Question 44

What are common symptoms of Microscopic Polyangiitis?

Answer 44

High ESR/CRP Anemia, weight loss, fatigue Otitis/Sinusitis

Question 45

What Ab is positive in Microscopic Polyangiitis?

Answer 45

P-ANCA

Question 46

What is the most accurate test for Microscopic Polyangiitis?

Answer 46

Biopsy

Question 47

How do you treat Microscopic Polyangiitis?

Answer 47

Steroids and Cyclophosphamide

Question 48

What population is Henoch-Schonlein Purpura (HSP) commonly seen in?

Answer 48

Children

Question 49

How does HSP typically present?

Answer 49

GI pain/bleeding, Purpura, arthralgia and hematuria

Question 50

What is the most accurate test for HSP?

Answer 50

Biopsy: shows leukocytoclastic vasculitis

Question 51

How is HSP treated?

Answer 51

Most cases resolve spontaneously Steroids: for severe abdominal pain or progressive renal insufficiency ACE-I for proteinuria

Question 52

What is Cryoglobulinemia most commonly associated with?

Answer 52

Hepatitis C

Question 53

What type of Antibodies are Cryoglobulins?

Answer 53

IgM

Question 54

How does Cryoglobulinemia Present?

Answer 54

Joint pain, Glomerulonephritis, Purpuric skin lesions and Neuropathy

Question 55

What do lab tests show for Cryoglobulinemia?

Answer 55

RF (+) Cold-precipitable immune complexes

Question 56

What C' protein is decreased in Cryogloulinemia?

Answer 56

C4

Question 57

How do you treat Cryoglobulinemia?

Answer 57

Treat the underlying cause: especially Hep C

Question 58

What is Behcet Syndrome?

Answer 58

widespread inflammation of vessels throughout the body

Question 59

What patient population is Behcet Syndrome more common in?

Answer 59

Asian or Middle Eastern

Question 60

What are common symptoms of Behcet Syndrome?

Answer 60

Painful oral and genital ulcers in association wiht erythema-nodosum like lesions of the skin

Question 61

What other symptoms are associated with Behcet Syndrome?

Answer 61

Ocular lesions: lead to uveitis and blindness Arthritis CNS lesions: mimic Multiple Sclerosis Aneurysm: Pulmonary and Coronary Arteries

Question 62

What is the most specific finding to help diagnose Behcet Syndrome?

Answer 62

Pathergy: production of sterile skin pustule after a needle is scraped against the skin

Question 63

How do you treat Behcet Syndrome?

Answer 63

Corticosteroids

Question 64

What medications are used to wean patients off Corticosteroids in Behecet Syndrome?

Answer 64

Colchicine Azathioprine Cyclophosphamide Thalidomide

Question 65

What drug can be used to relieve the oral ulcers in Behcet Syndrome?

Answer 65

Apremilast: PDE-inhibitor

Question 66

What is Takayasu Arteritis?

Answer 66

Granulomatous disease with massive intimal fibrosis blocking the subclavian arteries

Question 67

How does Takayasu Arteritis present?

Answer 67

Anemia, Fatigue, normocytic anemia, High ESR/CRP: all vasculitis Large vessel limb claudication with arterial bruits

Question 68

How do you diagnose Takayasu Arteritis?

Answer 68

Angiogram

Question 69

How do you treat Takayasu Arteritis?

Answer 69

Steroids

Question 70

What drugs can be used to wean patients off Steroids in Takayasu Arteritis?

Answer 70

Methotrexate Azathioprine Leflunomide Cyclophosphamide