Vasculitis Syndromes

Question 1

What are vasculitis syndromes?

Answer 1

★ A clinicopathologic process of inflammation and damage to
blood vessels
★ Broad and heterogeneous group of syndromes
★ May be a primary disease or secondary component of another
disease
★ May be confined to a single organ or involve several organs

Question 2

Etiology of Vasculitis syndromes

Answer 2

❖ Likely a number of factors are involved
➢ Genetic predisposition
➢ Environmental exposure
➢ Immune response to certain antigens

Question 3

Eosinophilic Granulomatosis with
Polyangitis AKA

Answer 3

AKA “Churg-Strauss Syndrome”

Question 4

Eosinophilic Granulomatosis with
Polyangitis etiology

Answer 4

❖ Exact cause unknown
❖ Allergic mechanism seems to be
directly involved
❖ T lymphocytes seem to help
maintain inflammation
❖ Any organ can be affected

Question 5

Eosinophilic Granulomatosis with
Polyangitis: 3 phases

Answer 5

➢ Prodromal: persists for years. Allergic rhinitis,
nasal polyps, asthma or combination
➢ 2nd phase: peripheral blood and tissue
eosinophilia includes chronic eosinophilic
pneumonia and gastroenteritis
➢ 3rd phase: life threatening vasculitis. Systemic
symptoms fever, weight loss, malaise and fatigue

Question 6

Eosinophilic Granulomatosis with
Polyangitis neurologic symtoms

Answer 6

mononeuritis multiplex occurs in 75% of patient

Question 7

Eosinophilic Granulomatosis with
Polyangitis diagnosis

Answer 7

❖ Antineutrophil cytoplasmic Autoantibodies (ANCA) present in
40 % of cases
❖ Biopsy is dx tool of choice
❖ Treatment with systemic corticosteroids
❖ More severe cases may need immunosuppressant therapy with
methotrexate, cyclophosphamide or azathioprine

Question 8

Temporal Arteritis s/s

Answer 8

❖ Headache-dominate symptom
➢ Jaw pain
➢ Fever
➢ Malaise
➢ PMR
➢ Blurred vision

Question 9

50% of untreated patients
develop blindness with ____

Answer 9

Temporal Arteritis

Question 10

Headache coarse in temporal arteritis

Answer 10

➢ Unilateral or bilateral superficial
➢ Located temporally about 50%
➢ Gradual onset with peak usually explosive
➢ Dull with stabbing pains
➢ Scalp tenderness cannot lay head on pillow
➢ Tender red temporal nodules may be present
➢ Worse at night

Question 11

Temporal Arteritis diagnosis

Answer 11

➢ History and physical
➢ Erythrocyte sedimentation rate (ESR) often elevated
➢ Temporal artery biopsy

Question 12

Temporal arteritis treatment

Answer 12

Prednisone 80 mg daily for 4 to 6 weeks
➢ Tocilizumab (ACTEMRA)–MCA against the interleukin-6
receptor

Question 13

Eosinophilic Granulomatosis with
Polyangitis treatment

Answer 13

❖ Antineutrophil cytoplasmic Autoantibodies (ANCA) present in
40 % of cases
❖ Biopsy is dx tool of choice
❖ Treatment with systemic corticosteroids
❖ More severe cases may need immunosuppressant therapy with
methotrexate, cyclophosphamide or azathioprine

Question 14

POLYARTERITIS NODOSA

Answer 14

❖ NECROTIZING VASCULITIS AFFECTING MEDIUM AND SMALL VESSELS
❖ MULTI ORGAN DISEASE SPARES THE LUNGS
❖ Disease usually of young adults
❖ Vague symptoms progress rapidly to fulminant illness
❖ Weakness, malaise, myalgias, new onset hypertension
❖ Most commonly affects kidney

Question 15

POLYARTERITIS NODOSA treatment

Answer 15

➢ Combination of high dose prednisone and immunosupression
➢ Cyclophosphamide (Cytoxan) or Azathioprine (Imuran)

Question 16

POLYARTERITIS NODOSA prognosis

Answer 16

➢ Untreated 5-10% mortality at 5 years
➢ Mortality from GI or cardiovascular
➢ Relapse in treated patients 10-20%

Question 17

Granulomatosis With Polyangitis

Answer 17

❖ Granulomatous vasculitis of upper
and lower respiratory tracts
❖ Small vessel disease
❖ Uncommon disease rare in black pts
❖ 1:1 ratio male-to-female
❖ Can be seen at any age; mean age at
onset is 40

Question 18

Granulomatosis With Polyangitis S/S

Answer 18

❖ Upper airway involvement in 95% of patients
➢ Paranasal sinus pain, purulent or bloody nasal discharge
➢ Nasal septal deviation resulting in saddle nose
➢ Serous otitis
➢ Subglottic stenosis
❖ Pulmonary involvement in 85-90%
➢ Cough, chest discomfort
➢ Hemoptysis, dyspnea
❖ Renal disease 77%
❖ Eye involvement 52%
❖ Skin lesions 46%
❖ Nervous system 23%
❖ Cardiac 8%

Question 19

Granulomatosis With Polyangitis diagnosis

Answer 19

➢ Elevated ESR, WBC, mild elevated Rheumatoid Factor
➢ 90% will have a positive anti-proteinase-3 ANCA if disease is active, only 60%
if inactive
➢ Necrotizing granulomatous vasculitis on biopsy- pulmonary tissue offers
highest yield
➢ Upper airway and renal biopsy rarely show the vasculitis

Question 20

Granulomatosis With
Polyangitis treatment

Answer 20

➢ Prior to onset of current treatment this was a universally fatal disease within
a few months of diagnoses
➢ Glucocorticoids improved symptoms but did not affect the ultimate outcome
➢ Cyclophosphamide (Cytoxin) changed outcome dramatically

Question 21

Two phases of treatment induction and maintenance for Granulomatosis With
Polyangitis

Answer 21

❖ Induction-severe disease
➢ Prednisone 1 mg/kg for one month then taper over 9 months
➢ Cyclophosphamide 2 mg/kg/day orally for 3 months then reduce to 1.5 mg/kg
daily
➢ Blood counts and renal function should be monitored every 2 weeks
➢ Rituximab (Rituxan) 375 mg/m2 once a week for 4 weeks in combination with
prednisone found to be superior to cyclophosphamide in recurrent cases
❖ Induction-non severe cases
➢ Prednisone 1 mg/kg for one month then taper over 9 months
➢ Methotrexate (Trexall) 0.3mg/kg not to exceed 15 mg/week single dose if
tolerated well after 2 weeks may increase by 2.5 mg weekly to a dose not to
exceed 25 mg week
❖ Maintenance phase (after 3-6 months)
➢ Methotrexate at above doses
➢ Azathioprine (Imuran) 2 mg/kg day

Question 22

Microscopic Polyangitis

Answer 22

❖ Nomenclature introduced in 1948 to recognize the presence of glomerulonephritis
in patients with polyarteritis nodosa
❖ 1992 was changed to connote a necrotizing vasculitis with few or no immune
complexes affecting capillaries and venules or arterioles (small vessel diseases)
❖ Glomerulonephritis is common
❖ Pulmonary capillaritis also occurs
❖ Absence of granulomatous inflammation differentiates it from Wegener’s

Question 23

Microscopic Polyangiitis diagnosis

Answer 23

➢ Elevated ESR, anemia, leukocytosis and thrombocytosis
➢ ANCA (antineutrophil cytoplasmic antibodies) are present in 75% of patients
➢ Defined with histologic evidence on biopsy

Question 24

Microscopic Polyangiitis treatment

Answer 24

➢ Same as Wegener’s
➢ 5 year survival rate is 74% with mortality from alveolar hemorrhage, GI,
cardiac or renal disease
➢ Relapse occurs in 34% of patients

Question 25

Henoch-Schonlein Purpura

Answer 25

❖ IgA Vasculitis -> small vessel vasculitis with palpable purpura,
usually on buttocks or lower extremities
❖ Usually seen in children ages 4-7 does not exclude infants and adults

Question 26

Henoch-Schonlein
Purpura pathophysiology

Answer 26

➢ Immune complex deposition
➢ IgA antibody most often seen with/after:
■ Upper respiratory infections
■ Insect bites
■ Foods
■ Immunizations

Question 27

Henoch-Schonlein Purpura

Answer 27

❖ Palpable purpura seen in virtually all patients
❖ Polyarthralgias
❖ 70% of pediatric patients experience colicky abdominal
pain associated with nausea, vomiting, bloody mucus in
diarrhea or hard stool bowel intussusception may occur
❖ Adult symptoms related to skin and joints
❖ Cardiac involvement rare in children
❖ Renal disease more severe and progressive in adults

Question 28

Henoch-Schonlein Purpura diagnosis

Answer 28

➢ Mild leukocytosis, normal platelets, mild eosinophilia
➢ Serum complement components are normal
➢ IgA levels elevated only in 50% of cases

Question 29

Henoch-Schonlein
Purpura Treatment

Answer 29

➢ Prognosis-mortality exceedingly rare
➢ Most do not require any treatment
➢ Tapered Prednisone starting at 1 mg/kg daily if needed to treat symptoms
➢ Severe glomerulonephritis treated with intensive plasma exchange
➢ Recurrence in 10-40% of patients

Question 30

1930’s Turkish Dermatologist Hulusi Behcet first physician to describe the triad of _____

Answer 30

aphthous oral ulcers, genital lesions, and
recurrent eye inflammation

Question 31

Behcet’s Syndrome S/S

Answer 31

❖ Recurrent painful aphthous ulcers of mouth and genitals
❖ Erythema nodosum-like lesions: follicular rash; and the pathergy phenomenon (sterile pustule at sight of needle stick)*
❖ Anterior or posterior uveitis- leading cause of blindness in Japan
❖ Neurological lesions that can mimic MS on MRI of brain
❖ Aneurysms of lung arteries rupture may lead to massive lung hemorrhage
❖ Joint pain without swelling
❖ Ulcerations of GI tract from mouth to anus
❖ Scrotal lesion in male vulvar lesions in female similar to oral but deeper

Question 32

Behcet’s Syndrome treatment

Answer 32

❖ Colchicine 0.6 mg 1 to 3 times daily
❖ Corticosteroids 1 mg/kg/day orally are mainstay treatment
❖ Thalidomide 100 mg/day orally for aphthous lesions
❖ Azathioprine 2 mg/kg/day orally effective non steroid tx
❖ Cyclosporine is indicated for severe ocular or central nervous
system involvement

Question 33

Kawasaki’s Disease etiology

Answer 33

❖ Etiology remains unknown but evidence indicates causative
agent is probably infectious.
❖ Autoimmune reactions and genetics are predisposing factors
❖ Siblings of those with kawasaki have 10 to 20 x higher
probability of becoming infected.

Question 34

85-90% of cases of _____ occur in children less than 5 years

Answer 34

Kawasaki’s Disease

Question 35

Kawasaki’s Disease pathophysiology

Answer 35

❖ Generalized vasculitis that affects small and
medium sized arteries.
❖ Most pronounced in coronary vessels
❖ Surpassed rheumatic heart disease as leading
cause of acquired heart disease in US <5 years
❖ 20 to 25 % of patients develop cardiac problems

Question 36

Kawasaki’s Disease symptoms

Answer 36

➢ Irritability
➢ Nonexudative
bilateral
conjunctivitis
➢ Anterior uveitis
➢ Perianal erythema
➢ Fever
➢ Enanthema (rash of mucous membranes)
➢ Bulbar conjunctivitis
➢ Rash
➢ Internal organ involvement
➢ Lymphadenopathy
➢ Extremity changes

Question 37

Kawasakis disease PE findings

Answer 37

Sterile pyuria
Erythema and edema of hands and
feet
Strawberry tongue
Hepatic renal GI dysfunction
Myocarditis
Lymphadenopathy single large
cervical lymph node 1.5 cm

Question 38

Kawasaki’s Disease diagnosis

Answer 38

➢ No specific lab testing
➢ Echocardiogram
■ At time of diagnoses
■ 2 weeks after diagnosis
■ 6-8 weeks after onset of illness

Question 39

Kawasaki’s Disease treatment

Answer 39

➢ Principle treatment is to prevent coronary
artery dz and to relieve symptoms
➢ Full dose of IVIG (IGG from 1000+ donors)
➢ ASA high dose followed by low dose
➢ Corticosteroids

Question 40

Buerger’s Disease aka

Answer 40

“Thromboangiitis obliterans”

Question 41

Buerger’s Disease

Answer 41

❖ Claudication in feet and/or hands
❖ Tingling or numbness of limbs
❖ Raynaud’s phenomenon
❖ Caused by tobacco
❖ Postulated that this is an autoimmune response
triggered by some constituent product of tobacco

Question 42

Buerger’s Disease diangosis

Answer 42

❖ Diagnosed by angiograms of upper and lower extremities and
have characteristic “corkscrew” appearance to the arteries in
the wrists and ankles secondary to vascular damage

Question 43

Buerger’s Disease treatment

Answer 43

❖ No effective treatment
❖ Immediate cessation and abstinence from all tobacco products
will prevent progression.