Vasculitis - Abu-Shakra

Question 1

What are the primary vasculitis syndromes that affect large vessels?

Answer 1

Takayasu’s arteritis:

• affects the aorta and its primary branches
• generally younger Asian patient with headache

Giant Cell Arteritis = Temporal Arteritis

• chronic vasculitis of large and medium size vessels, mainly the cranial branches of arteries originating from the aortic arch.
• relatively common
• Older female patient with headaches

Isolated Arteritis

• Nonspecific symptoms, thickened aorta (not many other features
• Granulomas, No autoantibodies, T-cell mediated

Question 2

What are vasculitis syndromes that affect medium sized vessels?

Answer 2

(Classic) Polyarteritis Nodosa (PAN)
-Systemic (fibro)necrotizing vasculitis of small and medium size muscular vessels.

Kawasaki Disease:

• Arteritis of medium and small arteries, particularly the coronary arteries
• Coronary infection at a young age
• Common in children with an MI

Question 3

What are vasculitis syndromes that affect small vessels?

What is distinct about where these antibodies attack?

Answer 3

ANCA (anti-neutrophilic cytoplasmic antibody) Associated (cytoplasm attack versus nucleus) Vasculitis:

Curg-Strauss Arteritis (CCS):
Vasculitis of medium and small sized muscular arteries, with vascular and extravascular granulomatosis.
Main features:
1. History of allergic rhinitis
2. Pulmonary infiltrate leading to eosinophilia
3. Vasculitis

Wegener’s Granulomatosis (WG):
vasculitis of medium and small arteries, as well venules and arterioles.
Granulomatous inflammation of the 1. URT, 2. LRT and 3. renal involvement (pauci-immune GN).
Sinuses, oral cavity, ear, trachea

Microscopic polyarteritis (MPA):
vasculitis which affects capillaries, venules, or
arterioles.

Question 4

What vasculitis syndromes affect the very small vessels (leucocytoclastic vasculitis)?

Answer 4

Mainly skin lesions

Cutaneous leukocytoclastic vasculitis:
– palpable purpura
– Hypersensitivity vasculitis (serum sickness)

Henoch-Schonlein Purpura (HSP):
systemic postcapillary venullitis
characterized by the deposition of IgA-containing immune complexes.
Age <16
URI followed by renal disease, abdominal vasculitis, and and purpura

Essential cryoglobulinemia vasculitis:
Cryoprecipitate after blood placed in fridge

Question 5

What is the pathogenic pathway of ANCA involvement in vasculitis?

Answer 5

1. Infections lead to secretion of cytokines
2. Priming of PMN by cytokines translocates the PR3 from the inside to the outside of the cell surface

If there is no autoimmune tendency the infection will be cured and that will be the end, but if autoimmune genes are present ANCA will bind to the PR3 on the cell wall and cause endothelial cell activation/lysis/destruction.

Question 6

What clinical features distinguish systemic vasculitis from other diseases?

Answer 6

Mononeuritis multiplex

Sensory peripheral neuropathy

Pulmonary hemorrhage

Nodules

Pansinusitis

Necrotizing GN

Bowel ischemia

Claudications (in the arm, jaw stiffness, etc. from ischemia)

CNS (seizures, strokes, altered consciousness)

Question 7

How is Microscopic Polyangitis (MPA) differentiated from Classic Polyarteritis Nodosa?

Answer 7

Classic is medium sized vessels only, MPA is small vessels

Renal involvement:
In MPA GN is typical with active sediments and casts
In classic form rarely nephritis but renal infarcts with high creatinine, mild proteinuria and hematuria

Pulmonary involvement only in MPA (hemorrhage)

Lividovasculitis is seen more in classical PAN.

Question 8

What is the classic tetrad of Henoch-Schonlein purpura?

Who is it most found in?

What is the equivalent in another population?

Answer 8

1. Rash/purpura: skin vasculitis
2. Arthralgia/arthritis
3. Abdominal pain: vasculitis
4. Renal disease

Usually in children following URI with IgA in serum

Same as hypersensitivity vasculitis in adults

Question 9

What is the diagnostic criteria of Behcet’s disease?

What is each manfestation treated with?

Answer 9

Must have recurrent oral ulcerations
Treated with anti-TNF

AND

2 of the following:

1. Recurrent genital ulceration
2. Eye lesions
   -uveitis
   -vasculitis
   Treated with anti-TNF (used to be Cyclosporine A)
3. Skin lesions
   -Erythema Nodosum
   -Folliculitis
   -Pustular acne
   Treated with Azothioprine and col.chicine
4. Pathergy

Can also have CNS symptoms, give steroids if MS like or meningitis