Vasculitis

Question 1

2 types of large vessel vasculitis

Answer 1

GCA and Takayasu

Question 2

2 types of medium vessel vasculitis

Answer 2

POlyarteritis nodosa and Kawasaki Disease

Question 3

2 categories of small vessel vasculitis

Answer 3

ANCA-associated vasculitis and Immune complex vasculitis

Question 4

3 types of ANCA-associated vasculitis and type of ANCA

Answer 4

Microscopic polyangitis: pANCA, MPO
GPA: cANCA, PR3
Eosinophilic granulomatosis w/ polyangiitis: either ANCA

Question 5

4 types of immune complex vasculitis

Answer 5

cryoglobulinemic vasculitis, anti-glomerular BM disease (Goodpasture), IgA vasculitis, hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)

Question 6

Treating small / medium vessel vasculitis

Answer 6

High dose corticosteroids and immune suppressive meds (azathriprine, Mtx, mycophenolate mofetil

Question 7

Treating large vessel vasculitis

Answer 7

corticosteroids

Question 8

treating life threatening / organ-threatening vasculitis

Answer 8

High dose steroids + cyclophosphamide or rituximab

Question 9

Giant Cell Arteritis
Age
How common?
Sxs
Association
Clinical clues
Diagnosis
Treatment

Answer 9

• Age >50
• By far the most common systemic vasculitis
• Sxs – new headache, constitutional sxs, proximal myalgia, painful shoulder / hip movement, jaw claudication, scalp tenderness, visual sxs (may cause blindness)
• Associated w/ polymyalgia rheumatica (50%)
• Look for dilation of temporal artery and loss of pulse.
• Diagnosis – obtain biopsy as soon as possible
• Shows granulomatous or mononuclear infiltrate in artery w/ fragmentation of internal elastic lamina.
• Treatment – begin high dose steroids right away if suspected.

Question 10

Takayasu's Arteritis
Gender
Age
Clinical manifestations
Treatment

Answer 10

• 9x more common in women.
• Avg age of onset under 40.
• Clinical manifestations – claudication of extremities, “pulseless” (esp in upper extremities), bruit over subclavian artery / abdominal aorta, abnormal angiogram / MRA
• Treat w/ high dose steroids

Question 11

Polyarteritis Nodosa (PAN)
4 affected organ systems
Associated diseases
Diagnosis
Treatment

Answer 11

• Skin – purpura, livedo reticularis
• Myalgia / arthralgia
• Nerve – mononeuropathy / multiple mononeuropathies
• Kidney – HTN, elevated Cr, proteinuria
• Associated w/ Hep B
• Diagnosis – biopsy or angiography (shows focal / segmental vasculitis)
• Treatment – steroids, cyclophosphamide if severe. Use steroids + anti-viral if pos for Hep B.

Question 12

Cryoglobulinemic vasculitis
Clinical manifestations
Associated disease
Diagnosis
Treatment

Answer 12

• Clinical manifestations – palpable purpura, arthralgias, LFTs, elevated Cr, proteinuria, low C4 / RF, cryoglobulins
• Associated w/ Hep C (>90%)
• Diagnose w/ biopsy
• Treatment – steroids + anti-viral +/- plasmapheresis. Use rituximab for severe cases.

Question 13

cANCA
Major Ag
Disease
Appearance

Answer 13

• Major Ag: serine protease PR3
• Disease: GPA
• Granular staining

Question 14

pANCA
Major Ag
Diseases (6)

Answer 14

• Major Ag: myeloperoxidase (MPO)
• Diseases: microscopic polyangiitis, Eosinophilic granulomatosis w/ polyangiitis (Churg-Strauss), IBD, chronic liver disease, HIV, lupus, etc.

Question 15

GPA
Clinical manifestations
Labs
Treatment

Answer 15

• Clinical manifestations – sinusitis, epistaxis, otitis media, anemia of chronic disease, RBC casts
• Lungs: infiltrates, nodules, cavitary lesions → hemoptysis, dyspnea,
• Kidney: HTN, pauci-immune glomerulonephritis, kidney failure
• Labs: cANCA, PR3, elevated ESR / Cr
• Treatment – high dose prednisone + (cyclophosphamide or rituximab)

Question 16

Microscopic Polyangiitis (MPA)
Clinical manifestations
Biopsy
Treatment

Answer 16

• Clinical manifestations – pulmonary infiltrates, proteinuria, hematuria, RBC casts, pANCA, MPO. Usually no lung involvement as w/ GPA.
• Biopsy shows pauci-immune glomerulonephritis and necrotizing vasculitis
• Treatment – steroids → cyclophosphamide or rituximab

Question 17

Eosinophilic granulomatosis w/ polyangiitis (EGPA, Churg-Strauss)
Clinical manifestations (8)
Biopsy
Treatment

Answer 17

• Clinical manifestations – asthma, allergic rhinitis, nasal polyposis, eosinophilia, non-fixed pulmonary infiltrates, cardiomyopathy, MI, pANCA
• Biopsy shows extravascular eosinophils
• Treatment – steroids → cyclophsophamide

Question 18

Behcet's Disease
Epidemiology
Pathology
Clinical manifestations
Prognosis
Treatment

Answer 18

• More common in men from Mediterranean and Far East. Onset 2nd-3rd decade.
• Chronic vasculitis of arteries and veins of all sizes. Large vessel disease is more rare but more serious. Affects veins more than other diseases.
• Clinical manifestations – recurrent painful ORAL / SCROTAL ULCERS, erythema nodosum (panniculitis of small vessels), superficial / deep vein thrombosus, uveitis / retinal vasculitis → blindness, bowel disease like Crohn’s, aseptic meningitis, meningoencephalitis.
• Prognosis – increased mortality, esp in young men. Mortality mainly from large vessel disease, esp bleeding pulmonary artery / GI aneurysms and CNS involvement.
• Treatment
• Local corticosteroids / cochicine for mucocutaneous ulcers
• Azathioprine for eye involvement

Question 19

Polymyalgia Rheumatica
Age
Heritage
Clinical manifestations
Association
Prognosis
Treatment

Answer 19

• Avg age 60-80.
• More common in Northern European heritage.
• Clinical manifestations – pain / stiffness of neck, shoulder girdle, and pelvic girdle (symmetric). Malaise, fever, weight loss.
• Associated w/ GCA.
• Prognosis – no increase in mortality. Usually remits in 2-3 years.
• Treatment – extremely responsive to low dose prednisone. Monitor ESR / CRP. Educate regarding risk for GCA.