Vaculitis

Question 1

What are the types of large vessel vasculitis?

Answer 1

• giant cell arteritis

- takayasu arteritis

Question 2

What vessels are affected in giant cell arteritis?

Answer 2

Large & medium sized vessels of the extracranial branches of the carotid artery
Seen after 50 years of age

Question 3

What are the predisposing factor to giant cell arteritis?

Answer 3

• genetic predisposition
• viral infections
• 50% also have PMR
• over 50 years of age

Question 4

What is the most important pathophysiological characteristic of giant cell arteritis?

Answer 4

Focal granulomatous inflammation

Question 5

What are the clinical features of giant cell arteritis?

Answer 5

• constitutional symptoms
• headaches
• scalp tenderness
• JAW CLAUDICATION
• VISION LOSS
• symptoms of PMR

Question 6

What will be seen on lab diagnostics in case of giant cell arteritis?

Answer 6

• increased ESR & CRP
• TEMPORAL ARTERY BIOPSY (gold) -> mononuclear cell infiltration with giant cells
• duplex US of temporal arteries

If suspected do NOT WAIT FOR RESULTS & TREAT IMMEDIATELY

Question 7

How is giant cell arteritis managed?

Answer 7

High dose corticosteroids for 1-2 years titrated based on symptoms & ESR

Question 8

What are the complications of giant cell arteritis?

Answer 8

• vision loss

- aortic aneurysm

Question 9

What are the vessels involved in Takayasu arteritis?

Answer 9

Pulseless aortitis & vasculitis of the large vessels & branches of the aorta

Seen in women in east asia

Question 10

What are the symptoms of Takayasu arteritis?

Answer 10

• fever
• malaise
• myalgia
• arthralgia
• weight loss
• progressive claudication (in limbs)

Question 11

How is Takayasu arteritis diagnosed & managed?

Answer 11

Diagnosed by -> angiography of aorta

Treated -> high dose corticosteroids, methotrexate, TNF inhibitors

Question 12

What are the types of medium vessel vasculitis?

Answer 12

• polyarteritis nodosa

- thromboangitis obliterans

Question 13

What are the clinical features of Polyarteritis Nodosa?

Answer 13

• fever, weight loss, malaise, muscle & joint pain
• KIDNEY: hypertension & renal impairment
• SKIN: livedo reticularis, nodules, ulcerations
• neuro: mononeuropathies
• GI: abdominal pain, melena, nausea, vomiting

Question 14

How is PAN diagnosed?

Answer 14

• increased ESR & CRP
• • ANCA
• • hepatitis B

Angiography -> aneurysmal dilations of affected arteries (Rosary beads) usually in renal & mesenteric

Question 15

How is PAN treated?

Answer 15

• high dose corticosteroids
• cytotoxic immunosuppressive agents (cyclophosphamide)
• antiviral therapy for HBV infection

Question 16

What is the classic triad of thromboangitiis obliterans?

Answer 16

1- intermittent claudication
2- Raynaud phenomena
3- migratory thrombophlebitis

Usually in male smokers
Treatment is discontinuing smoking

Question 17

What are the organs involved in granulomatosis with polyangiitis?

Answer 17

Necrotizing granulomatous arteritis mostly in the kidneys & upper & lower respiratory tract

Question 18

What are the clinical features of GPA?

Answer 18

• upper respiratory tract -> sinusitis, epistaxis, otitis media, gingivitis, stridor, mastoiditis, saddle nose
• lungs -> cough, hemoptysis, diffuse alveolar hemorrhage, dyspnea, tracheal stenosis
• kidneys -> rapidly progressive crescentic glomerulonephritis, hematuria
• arthritis, scleritis, skin rashes

Question 19

How is GPA diagnosed?

Answer 19

• ESR increased
• C ANCA (anti proteinase 3) positive
• biopsy -> necrotizing granulomatous
• x ray -> pulmonary nodules or cavities
• UA with active sedatives

Question 20

How is GPA managed?

Answer 20

INDUCTION -> cyclophosphamide + corticosteroids and/or rituximab
REMISSION -> methotrexate, azathioprine

Question 21

What are the complications of GPA?

Answer 21

• kidney failure

- diffuse alveolar hemorrhage could be life-threatening

Question 22

What organs are most involved in eosinophilic granulomatosis with polyangitis (Churg-Strauss syndrome)?

Answer 22

small and medium vessel necrotizing vasculitis & eosinophilia most commonly in LUNGS & SKIN

Question 23

What are the clinical features of EGPA?

Answer 23

• asthma, nasal polyps, allergic rhinitis
• mononeuritis multiplex
• cough, dyspnea, angina pectoris

Question 24

How is EGPA diagnosed & treated?

Answer 24

• EOSINOPHILIA
• MPO-ANCA & pANCA
• XRAY -> fleeting pulmonary infiltrates
• biopsy -> tissue eosinophilia

Treat with immunosuppression + glucocorticoids +/- cyclophosphamide

Question 25

What are the characteristics of microscopic polyangitis?

Answer 25

NONGRANULOMATOUS vasculitis of small vessels mostly in lungs, kidneys, & skin

Question 26

How is microscopic polyangitis diagnosed & treated?

Answer 26

Diagnosis - BIOPSY: fibrinoid necrosis with infiltration of neutrophils & no granuloma - MPO-ANCA & pANCA Treatment - immunosuppression with corticosteroids & cyclophosphamide

Question 27

What are the characteristics of Behcet disease?

Answer 27

- oral ulcers - recurrent painful genital ulcerations - eye or cutaneous lesions - + parhergy test TREAT WITH systemic corticosteroids