Download Vasculitis

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.
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.
Vasculitis
• Diseases characterized by inflammation and
necorosis of artery
• Primary vasculitis: damage:
• Large vessels
• Medium vessels
• Small vessels
• Histological, patogenetic aspects and clinical
feature
Vasculitis
• Cranial arteritis
• – prevalence 15-30/100 000
• - incidence 18/100 000
Vasculitis
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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
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
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.
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.
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
Primary CNS angiitis (PACNS)
Primary CNS angiitis (PACNS)
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.
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.