Vasculitis

1
Vasculitis

• Vasculitis arises when immune system mistakenly
  attacks blood vessels.
• What causes this attack isn't fully known, but it
  can result from infection or certain medications.
• Severe forms of vasculitis can be caused by the
  rare autoimmune diseases microscopic polyangiitis
  and granulomatosis with polyangiitis.

2
Vasculitis

• People with these conditions produce harmful
  antibodies (anti-neutrophil cytoplasmic
  antibodies, or ANCAs) that attack immune cells
  known as neutrophils.
• Resulting inflammation in small- to medium-sized
  blood vessels can cause severe organ damage and
  sometimes death.

3
Vasculitis

• Diseases characterized by inflammation and
  necorosis of artery
• Primary vasculitis damage
• Large vessels
• Medium vessels
• Small vessels
• Histological, patogenetic aspects and clinical
  feature

4
Vasculitis

• Cranial arteritis
• prevalence 15-30/100 000
• - incidence 18/100 000

5
Vasculitis

• Polyarteritis nodosa medium and small vessels
• Fever, fatigue, loss of weight, artritis, skin
  changes
• Myalgia, polyneuropathy
• Involvement of brain cca 20
• Wegener granulomatosis small vessels
• Nekrotisans granulomatomas compression of
  cranial nerves
• Meningitis, hydrocephalus
• Polyneuropathy, myelopathy, cerebrovascular
  diseases
• Kidney, lung

6
Vasculitis

• Churg-Strauss syndrom
• CNS 6-8 pacients
• Stroke ischemic, hemorrhage, SAH
• Behcet disease
• Multisystem, chronic-relapsing vasculitis, damage
  predominantly venous system
• Oral ulcerations genital ulcerations, uveitis,
  erythema nodosum,
• 30 - CNS lesion of pyramidal tract, stroke,
  headache, venous sinuses thrombosis

7
Therapy

• Combination of immunosuppressive drugs to control
  the inflammation.
• These drugs are commonly high dose steroids
  (prednisolone) and additional treatment with
  drugs such as cyclophosphamide or methotrexate
  may be given.
• The amount of steroid treatment will be reduced
  quickly over the first few weeks and then more
  slowly.
• The current standard of care for ANCA-related
  vasculitis requires daily doses of the harsh
  immunosuppressant drug cyclophosphamide for 3 to
  6 months.

8
Therapy

• Daily doses of another immunosuppressant,
  azathioprine, then follow for a year or more.
• This standard therapy usually clears the
  vasculitis, but relapse is common.
• In addition, this treatment suppresses the immune
  system in a non-specific way and has potentially
  severe side effects.
• Rituximab is specifically targeted to deplete the
  type of immune cells thought to produce ANCA.

9
Primary CNS angiitis (PACNS)

• Multifocal or diffuse damage of CNS with
  remittent or progredient clinical course
• Leasion of the spinal cord
• CSF increased elements, proteins
• AG narroving and dilatation of the vessels
• MRI ischemic, hemoragic, tumor-like lesions,
  enhancement of meninges after gadolínium
• Brain biopsy

10
Primary CNS angiitis (PACNS)
11
Primary CNS angiitis (PACNS)
12
Primary CNS angiitis (PACNS)

• Therapy
• Combined immunosuppressive therapy is the
  treatment of choice for PACNS.
• This therapy was initially proposed after its
  success in patients with systemic vasculitis such
  as Wegner granulomatosis and polyarteritis nodosa
  
• but is not supported by evidence from controlled
  trials in PACNS.

13
Primary CNS angiitis (PACNS)

• An induction regimen for 9-12 weeks
  Cyclophosphamide 2.5 mg/kg/d coupled with
  intravenous methylprednisolone, 1 g/d for 3 days,
  then oral prednisolone, 60 mg/d, to be decreased
  by 10 mg at weekly intervals to reach a dose of
  10 mg/d, if possible.
• A maintenance regimen for further 10 months
  Alternate day steroids (10-20 mg prednisolone)
  along with azathioprine, 2 mg/kg/d, substituted
  for cyclophosphamide.