Vasculitis

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Vasculitis
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• Vasculitis refers to a heterogeneous group of
  disorders that are characterized by inflammatory
  destruction of blood vessels.
• Both arteries and veins are affected.
  Lymphangitis is sometimes considered a type of
  vasculitis.
• Solitary inflammation of veins (phlebitis) or
  arteries (arteritis), although both occur in
  vasculitis, on their own are separate entities.
  Vasculitis affects both arteries and veins.
  Vasculitis is primarily due to leukocyte
  migration and resultant damage

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• Types of systemic vasculitis
• Non-infective
• Large
• Giant cell arteritis  
• Takayasu's arteritis  
• Medium
• Classical polyarteritis nodosum (PAN)  
• Kawasaki's disease
•  Small
• Microscopic polyangiitis  
• Wegener's granulomatosis ANCA
  associated
•  Churg-Strauss syndrome
•  Henoch-Schönlein purpura
•  Cutaneous leucocytoclastic vasculitis  
• Essential cryoglobulinaemia

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• Other conditions associated with vasculitis
• Infective e.g. Subacute infective endocarditis
• Non-infective
• Vasculitis with rheumatoid arthritis
•  Systemic lupus erythematosus  
• Scleroderma  
• Polymyositis/dermatomyositis  
• Behçet's disease  
• Goodpasture's syndrome
•  Serum sickness
• Paraneoplastic syndromes  
• Inflammatory bowel disease

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• The disorders are characterized by inflammation
  in or through a blood vessel wall, with fibrinoid
  necrosis with or without granuloma formation.
• Histologically there are several different
  patterns necrotizing vasculitis giant cell
  arteritis and granulomatous angiitis. The
  clinical manifestations are due to ischaemic
  necrosis and vary with the size and type of blood
  vessel affected. The vasculitides are systemic
  diseases which affect the skin and
  musculoskeletal, renal and gastrointestinal
  systems. Skin lesions are palpable.

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• LARGE VESSEL VASCULITIS
• Polymyalgia rheumatica (PMR) and giant cell
  (temporal) arteritis are systemic illnesses of
  the elderly.
• Both are associated with the finding of a giant
  cell arteritis on temporal artery biopsy.
• Polymyalgia rheumatica (PMR) PMR causes a sudden
  onset of severe pain and stiffness of the
  shoulders and neck, and of the hips and lumbar
  spine a limb girdle pattern. These symptoms are
  worse in the morning, lasting from 30 minutes to
  several hours. The clinical history is usually
  diagnostic and the patient is always over 50

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• Patients develop systemic features of tiredness,
  fever, weight loss, depression and occasionally
  nocturnal sweats.
• Symptom patterns in some muscle disorders
• Polymyositis - proximal muscle ache and weakness
• Polymyalgia rheumatica - proximal morning
  stiffness and pain
• Myopathy - weakness, but no pain or stiffness

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• Investigation of PMR
• A raised ESR and/or CRP is a hallmark of this
  condition. It is rare to see PMR without an
  acute-phase response.
• Anaemia (mild normochromic, normocytic) is often
  present.
• Temporal artery biopsy shows giant cell arteritis
  in 10-30 of cases, but is not usually performed.

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• Giant cell arteritis (GCA)
• GCA is inflammatory granulomatous arteritis of
  large arteries which occurs in association with
  PMR. The patient may have current PMR, a history
  of recent PMR, or be on treatment for PMR. It is
  extremely rare under 50 years of age. It may
  present with the symptoms of GCA with severe
  headaches, tenderness of the scalp (combing the
  hair may be painful) or of the temple,
  claudication of the jaw when eating, tenderness
  and swelling of one or more temporal or occipital
  arteries, and can cause sudden painless temporary
  or permanent visual loss . Systemic
  manifestations of severe malaise, tiredness and
  fever occur.

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• Investigation of GCA
• Normochromic, normocytic anaemia.
• ESR is usually raised (in the region of 50-120
  mm/h) and the CRP very high.
• A temporal artery biopsy from the affected side
  is the definitive diagnostic test. This should be
  taken before, or within 7 days of starting, high
  doses of corticosteroids. Start the drug first if
  the patient is very ill, in severe pain or has
  experienced visual loss or stroke. The lesions
  are patchy and the whole length of the biopsy (gt
  1 cm long) must be examined.

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• The histological features of GCA are
• intimal hypertrophy
• inflammation of the intima and sub-intima
• breaking up of the internal elastic lamina
• giant cells, lymphocytes and plasma cells in the
  internal elastic lamina.
• Treatmentof PMR or GCA Corticosteroids produce
  a dramatic reduction of symptoms within 24-48
  hours of starting treatment, provided the dose is
  adequate.
• In GCA, corticosteroids are obligatory because
  they significantly reduce the risk of
  irreversible visual loss and other focal
  ischaemic lesions, but much higher doses are
  needed.

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• Takayasu's arteritis
• This is a granulomatous inflammation of the
  aorta and its major branches. It is a rare
  disease, predominantly, affect young women.
• Features include systemic symptoms,with weight
  loss and fever. Vessel inflammation results in
  stenosis, which may be demonstrated
  angiographically.
• Features of arterial stenosis include
  dizziness,fainting,exertional dyspnea and reduced
  or absent peripheral pulse.
• The acute symptoms may respond to steroid
  therapy,but vascular reconstruction may helpful
  in later stages of the disease.

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• MEDIUM-SIZED VESSEL VASCULITIS
• Polyarteritis nodosa (PAN)
• Classical PAN is a rare condition which, unlike
  other vasculitic diseases, usually occurs in
  middle-aged men.
• It is accompanied by severe systemic
  manifestations, and its occasional association
  with hepatitis B antigenaemia suggests a
  vasculitis secondary to the deposition of immune
  complexes. Pathologically, there is fibrinoid
  necrosis of vessel walls with microaneurysm
  formation, thrombosis and infarction.

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• Clinical features These include fever, malaise,
  weight loss and myalgia. These initial symptoms
  are followed by dramatic acute features that are
  due to organ infarction.
• Neurological - mononeuritis multiplex is due to
  arteritis of the vasa nervorum.
• Abdominal - pain due to arterial involvement of
  the abdominal viscera, mimicking acute
  cholecystitis, pancreatitis or appendicitis.
  Gastrointestinal haemorrhage occurs because of
  mucosal ulceration.
• Renal - presents with haematuria and proteinuria.
  Hypertension and acute/chronic renal failure
  occur.
• Cardiac - coronary arteritis causes myocardial
  infarction and heart failure. Pericarditis may
  occur.
• Skin - subcutaneous haemorrhage and gangrene
  occur. A persistent livedo reticularis is seen in
  chronic cases. Cutaneous and subcutaneous
  palpable nodules occur, but are uncommon.
• Lung - involvement is rare.

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• Investigations and treatment Blood count.
  Anaemia, leucocytosis and a raised ESR occur.
• Biopsy material from an affected organ.
• Angiography. Demonstration of microaneurysms in
  hepatic, intestinal or renal vessels if
  necessary.
• Other investigations as appropriate (e.g. ECG and
  abdominal ultrasound), depending on the clinical
  problem.
• Treatment is with corticosteroids, usually in
  combination with immunosuppressive drugs such as
  azathioprine.

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• Kawasaki's disease
• This is an acute systemic vasculitis involving
  medium-sized vessels, affecting mainly children
  under 5 years of age. It is very frequent in
  Japan, and an infective trigger is suspected. It
  occurs world-wide.

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• Wegenergranulomatosis.
• Signs and symptoms
• Initial signs are extremely variable, and
  diagnosis can be severely delayed due to the
  nonspecific nature of the symptoms. Rhinitis is
  generally the first sign in most patients.
• Upper airway, eye and ear disease
• Nose pain, stuffiness, nosebleeds, rhinitis,
  saddle-nose deformity due to a perforated septum
• Ears conductive hearing loss due to auditory
  tube dysfunction, sensorineural hearing loss
  (unclear mechanism)
• Oral cavity strawberry gingivitis, underlying
  bone destruction with loosening of teeth,
  non-specific ulcerations throughout oral mucosa
• Eyes pseudotumours, scleritis, conjunctivitis,
  uveitis, episcleritis
• Trachea subglottal stenosis
• Lungs pulmonary nodules (referred to as "coin
  lesions"), infiltrates (often interpreted as
  pneumonia), cavitary lesions, pulmonary
  hemorrhage causing hemoptysis, and rarely
  bronchial stenosis.
• Kidney rapidly progressive segmental necrotising
  glomerulonephritis (75), leading to chronic
  renal failure
• Arthritis Pain or swelling (60), often
  initially diagnosed as rheumatoid arthritis
• Skin nodules on the elbow, purpura.
• Nervous system occasionally sensory neuropathy
  (10)
• Heart, gastrointestinal tract, brain, other
  organs rarely affected.

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• ChurgStrauss syndrome (also known as allergic
  granulomatosis) is a medium and small vessel
  autoimmune vasculitis, leading to necrosis. It
  involves mainly the blood vessels of the lungs
  (it begins as a severe type of asthma),
  gastrointestinal system, and peripheral nerves,
  but also affects the heart, skin and kidneys.
• It is a rare disease that is non-inheritable,
  non-transmissible.
• Churg-Strauss syndrome was once considered a
  type of Polyarteritis nodosa (PAN) due to their
  similar morphologies.

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• Behcets disease
• The cardinal clinical feature is recurrent oral
  ulceration. The international criteria for
  diagnosis require oral ulceration and any two of
  the following genital ulcers, defined eye
  lesions, defined skin lesions, or a positive skin
  pathergy test
• Oral ulcers can be aphthous or herpetiform. The
  eye lesions include an anterior or posterior
  uveitis or retinal vascular lesions.
• Cutaneous lesions consist of erythema nodosum,
  pseudofolliculitis and papulopustular lesions.
• Other manifestations include a self-limiting
  peripheral mono- or oligoarthritis affecting
  knees, ankles, wrists and elbows
  gastrointestinal symptoms of diarrhoea, abdominal
  pain and anorexia pulmonary and renal lesions a
  brainstem syndrome, organic confusional states
  and a meningoencephalitis.
• All the common manifestations are self-limiting
  except for the ocular attacks. Repeated attacks
  of uveitis can cause blindness.
• The pathergy reaction is highly specific to
  Behçet's disease. Skin injury, by a needle prick
  for example, leads to papule or pustule formation
  within 24-48 hours.

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Oral ulcer
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Diagnosis of vasculitis

• Laboratory tests of blood or body fluids are
  performed for patients with active vasculitis.
  Their results will generally show signs of
  inflammation in the body, such as increased
  erythrocyte sedimentation rate (ESR), elevated
  C-reactive protein (CRP), anemia, increased white
  blood cell count and eosinophilia. Other possible
  findings are elevated antineutrophil cytoplasmic
  antibody (ANCA) levels and hematuria.
• Other organ functional tests may be abnormal.
  Specific abnormalities depend on the degree of
  various organs involvement.
• The definite diagnosis of vasculitis is
  established after a biopsy of involved organ or
  tissue, such as skin, sinuses, lung, nerve, and
  kidney. The biopsy elucidates the pattern of
  blood vessel inflammation.
• An alternative to biopsy can be an angiogram ,It
  can demonstrate characteristic patterns of
  inflammation in affected blood vessels

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Treatment.

• Treatments are generally directed toward stopping
  the inflammation and suppressing the immune
  system. Typically, cortisone-related medications,
  such as prednisone, are used. Additionally, other
  immune suppression drugs, such as
  cyclophosphamide and others, are considered.
• Additionally, affected organs (such as the heart
  or lungs) may require specific medical treatment
  intended to improve their function during the
  active phase of the disease.