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PEDIATRIC
VASCULITIDES
What is Vasculitis?
   The term vasculitis means inflammation of the
    wall of blood vessels.
   Various sizes of blood vessels may be involved.
   The inflammatory exudates may vary and be
    mononuclear, eosinophilic or neutrophilic.
How Common is Vasculitis in
Children?
   Vasculitis in children appears to have an
    incidence of about 50 cases per 100,000
    children per year.
   Henoch Schonlein purpura (HSP) and Kawasaki
    disease (KD) are more common in children (75–
    125 per 100,000 in Japanese children and 9 per
    100,000 in Caucasian children of USA.)
How is Vasculitis Classified in
Children?
   The aorta and its main branches are termed
    large vessels;
   The first branches of the aorta e.g., renal,
    mesenteric, coronary vessels are regarded as
    mid-sized;
   Arterioles, capillaries and venules are regarded
    as small-sized.
   Small vessel vasculitis (SVV) is sub-categorized
    into granulomatous and non-granulomatous
    varieties.
New classification of childhood
vasculitides
When Should a Pediatrician Suspect Vasculitis
in a Child?
Large vessel involvement:

1.   Absent pulses,
2.   Bruits over aorta and vessels,
3.   Hypertension,
4.   Discrepant four limb blood pressure,
5.   Congestive heart failure.
6.   Cardiomyopathy.
Medium vessel involvement:

1.   Hypertension (renal vessels).
2.   Abdominal pain (celiac axes),
3.   Chest pain (coronaries),
4.   Claudication (subclavian, femoral or iliac
     vessels),
5.   Deep skin changes, gangrene.
6.   Focal deficits (cerebrovascular
     insufficiency/sudden blindness),
7.   Orchitis (testicular) and
8.   Neuropathy (vasa vasorum).
Gangrene of the toes in systemic PAN
Small vessel involvement (symptoms in
richly vascularized organs):

1.   Purpura, superficial ulcers, mucosal ulcers,
2.   Pulmonary and/or renal syndrome.
3.   Ear-nose throat disease.
4.   Asthma.
What is the Investigative Approach for a Child
with Vasculitis with Regards to Radiology,
Histopathology and Serology?

    Radiology:
           Digital Subtraction Angiography (DSA)
    In large vessel vasculitis, conventional DSA is
     useful in demonstrating vessel stenoses or
     aneurysms.
    The major limitations of DSA
1.     Invasiveness,
2.      High contrast load,
3.     High-dose radiation exposure,
4.      Complications at the arterial injection site.
5.      Inability to delineate the arterial wall anatomy.
Digital subtraction angiography demonstrating stenoses
of a segment of the aorta and the left proximal renal
artery
MR Angiogram (MRA)

    MR angiography :
1.     Detects of early signs of large-vessel disease,
      and
2.     It has the added advantage of potentially
      revealing evidence of ongoing large arterial wall
      inflammation.

    Disadvantage :
1.    Its relative insensitivity to slow flow or in-plane
      flow because of saturation of the MR imaging
      signal intensity.
2.    This can lead to overestimation of the severity of
      the stenosis or to a false diagnosis of vascular
      occlusion .
Computerised Tomography (CT) and
Ultrasonography
(USG)
    USG provides a very good image of the following compared
     to CT or MRI.
1.    Carotid.
2.    Axillary.
3.    Brachial.
4.    Femoral arteries.

    Major advantages are
1.    Non invasiveness,
2.    No radiation,
3.    Wide availability and
4.    Low cost.
5.    Blood flow characteristics, wall elasticity, and plaques can be
      delineated.
    Relative Disadvantages : The image of the
    subclavian, iliofemoral, renal, superior and
    inferior mesenteric arteries, the celiac trunk, and
    the abdominal aorta is moderate.
   The assessment of thoracic aorta requires
    transesophageal USG.
   CT, Electron beam tomography and CT
    angiogram (CTA) have been reported as useful
    tools for assessing the aorta and its main
    branches.
   However, poor visualisation of the more distal
    branches and a high radiation exposure are
    possible disadvantages.
   Studies comparing Color Doppler Flow Imaging
    (CDFI) with MRA and conventional angiography
    have shown a high degree of correlation in
    delineating the extent of stenosis in the carotids
    and subclavian vessels.
    Positron emission tomography (PET) scanning
    with radioactive-labelled 18-fluorodeoxyglucose
    (FDG) has been shown to be useful in
    monitoring disease activity (by its extreme
    sensitivity to pick inflamed vessels) and
    response to treatment in preliminary studies.
2 D Echocardiogram


   Helps in detecting or excluding coronary artery
    aneurysms, intraluminal or mural thrombi,
    regurgitant cardiac valves, myocardial
    dysfunction, or pericardial effusion.
Histology


   The choice of tissue is guided by the clinical
    setting using the most accessible involved organ
    or tissue.
   Various organs biopsied include the skin, lungs
    and kidney (pulmonary-renal syndrome), sural
    nerve, and muscle.
   Skin is the most commonly biopsied area.
   A deep punch biopsy (to diagnose changes in the
    deeper vessels at the dermal level) is ideal.
   Cutaneous vasculitis is mostly characterized by
    involvement of the post-capillary venules with
    endothelial cell swelling, neutrophilic invasion of
    blood vessel walls, presence of disrupted
    neutrophils (leukocytoclasia), extravasation of red
    blood cells, and fibrinoid necrosis of the blood
    vessels walls.
   These features are termed cutaneous
    necrotizing vasculitis (CNV), which refers to
    leukocytoclastic vasculitis affecting the small
    and medium vessels supplying nutrients to the
    skin .
   Along with leukocytoclasia, the biopsy may
    reveal areas of panniculitis, particularly in PAN.
   Samples should be subjected to
    immunofluorescence particularly for possible
    HSP (IgA deposits), ANCA associated vasculitis
    (pauci-immune necrotising vasculitis) or
    secondary vasculitis like SLE (immunoglobulins
    and complement deposits at the dermo-
    epidermal junction).
   Renal biopsies are carried out if clinical features
    and urinalysis suggest renal involvement.
   Various changes from focal to segmental
    glomerulonephritis can be seen.
   Subepithelial deposits can be seen on electron
    microscopy
   As the yield from tissues like sinuses, nose,
    ears, and trachea is low (<50%), invasive
    procedures such as open or thoracoscopic lung
    biopsies or renal biopsies are often used to
    diagnose Wegeners granulomatosis (WG).
Serology


    ANCA are present with a high specificity of up to
     98% in the specific group of AAV.
    Two staining patterns for ANCA are detected by
     ELISA.
1.    Cytoplasmic ANCA (C-ANCA) representing
      antibody to proteinase 3 is present in 90% of
      patients with active diffuse WG i.e., with high
      specificity.
2.    Perinuclear ANCA (P-ANCA) caused by
      antimyeloperoxidase antibody is suggestive of
      involvement of small to medium sized arteries in
      conditions such as microscopic polyangiitis.
   The presence of Von Willebrand factor antigen
    is a surrogate marker for SVV.
   Elevated IgA levels may be found in 50–70% of
    HSP.
   Anti-streptolysin O titres may be elevated with
    cutaneous polyarteritis (C- PAN).
   Serology for Hepatitis B and C are relevant in
    cases of polyarteritis nodosa (PAN).
Differential Diagnoses
How are Children with Vasculitis
Managed?
What is the Prognosis of the Various Vasculitides?


   The majority of children with HSP make a full
    and uneventful recovery with no evidence of
    ongoing significant renal disease.
   Mortality is around 1–2% with severe
    gastrointestinal or renal involvement.
   Renal involvement is the most serious long-term
    complication of HSP and occurs in 5%.
   KD has a good prognosis, with 1–2% acute
    mortality rate due to myocardial infarction,
    having been reduced further to less than 1%
    due to increasing awareness of clinicians and
    prompt treatment.
   Ozen et al reported in a retrospective series of
    childhood PAN an improved outcome compared
    to that reported in adults with only one (1.1%)
    death and two (2.2%) patients with end-stage
    renal disease among 110 patients.
   C-PAN has a good prognosis, albeit with
    recurrent relapses and remissions.
   The AAV carry considerable disease-related
    morbidity and mortality mainly due to
    progressive renal failure or aggressive
    respiratory involvement.
   For Churg-Strauss syndrome (CSS) in children,
    the most recent series reports mortality of 18%,
    attributable to disease rather than to therapy.
   The risk of organ damage is related to the
    duration of active disease.
   With proper treatment, clinical remission is often
    achieved within the first year.
   The remission may be life-long, but long-term
    maintenance therapy is frequently required.
   Periods of disease remission may be interrupted
    with relapses requiring more intensive therapy.

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Pediatric vasculitides

  • 2. What is Vasculitis?  The term vasculitis means inflammation of the wall of blood vessels.  Various sizes of blood vessels may be involved.  The inflammatory exudates may vary and be mononuclear, eosinophilic or neutrophilic.
  • 3. How Common is Vasculitis in Children?  Vasculitis in children appears to have an incidence of about 50 cases per 100,000 children per year.  Henoch Schonlein purpura (HSP) and Kawasaki disease (KD) are more common in children (75– 125 per 100,000 in Japanese children and 9 per 100,000 in Caucasian children of USA.)
  • 4. How is Vasculitis Classified in Children?  The aorta and its main branches are termed large vessels;  The first branches of the aorta e.g., renal, mesenteric, coronary vessels are regarded as mid-sized;  Arterioles, capillaries and venules are regarded as small-sized.  Small vessel vasculitis (SVV) is sub-categorized into granulomatous and non-granulomatous varieties.
  • 5. New classification of childhood vasculitides
  • 6. When Should a Pediatrician Suspect Vasculitis in a Child?
  • 7.
  • 8. Large vessel involvement: 1. Absent pulses, 2. Bruits over aorta and vessels, 3. Hypertension, 4. Discrepant four limb blood pressure, 5. Congestive heart failure. 6. Cardiomyopathy.
  • 9. Medium vessel involvement: 1. Hypertension (renal vessels). 2. Abdominal pain (celiac axes), 3. Chest pain (coronaries), 4. Claudication (subclavian, femoral or iliac vessels), 5. Deep skin changes, gangrene. 6. Focal deficits (cerebrovascular insufficiency/sudden blindness), 7. Orchitis (testicular) and 8. Neuropathy (vasa vasorum).
  • 10. Gangrene of the toes in systemic PAN
  • 11. Small vessel involvement (symptoms in richly vascularized organs): 1. Purpura, superficial ulcers, mucosal ulcers, 2. Pulmonary and/or renal syndrome. 3. Ear-nose throat disease. 4. Asthma.
  • 12. What is the Investigative Approach for a Child with Vasculitis with Regards to Radiology, Histopathology and Serology?  Radiology: Digital Subtraction Angiography (DSA)  In large vessel vasculitis, conventional DSA is useful in demonstrating vessel stenoses or aneurysms.  The major limitations of DSA 1. Invasiveness, 2. High contrast load, 3. High-dose radiation exposure, 4. Complications at the arterial injection site. 5. Inability to delineate the arterial wall anatomy.
  • 13. Digital subtraction angiography demonstrating stenoses of a segment of the aorta and the left proximal renal artery
  • 14. MR Angiogram (MRA)  MR angiography : 1. Detects of early signs of large-vessel disease, and 2. It has the added advantage of potentially revealing evidence of ongoing large arterial wall inflammation.  Disadvantage : 1. Its relative insensitivity to slow flow or in-plane flow because of saturation of the MR imaging signal intensity. 2. This can lead to overestimation of the severity of the stenosis or to a false diagnosis of vascular occlusion .
  • 15. Computerised Tomography (CT) and Ultrasonography (USG)  USG provides a very good image of the following compared to CT or MRI. 1. Carotid. 2. Axillary. 3. Brachial. 4. Femoral arteries.  Major advantages are 1. Non invasiveness, 2. No radiation, 3. Wide availability and 4. Low cost. 5. Blood flow characteristics, wall elasticity, and plaques can be delineated.
  • 16. Relative Disadvantages : The image of the subclavian, iliofemoral, renal, superior and inferior mesenteric arteries, the celiac trunk, and the abdominal aorta is moderate.  The assessment of thoracic aorta requires transesophageal USG.
  • 17. CT, Electron beam tomography and CT angiogram (CTA) have been reported as useful tools for assessing the aorta and its main branches.  However, poor visualisation of the more distal branches and a high radiation exposure are possible disadvantages.
  • 18. Studies comparing Color Doppler Flow Imaging (CDFI) with MRA and conventional angiography have shown a high degree of correlation in delineating the extent of stenosis in the carotids and subclavian vessels.  Positron emission tomography (PET) scanning with radioactive-labelled 18-fluorodeoxyglucose (FDG) has been shown to be useful in monitoring disease activity (by its extreme sensitivity to pick inflamed vessels) and response to treatment in preliminary studies.
  • 19. 2 D Echocardiogram  Helps in detecting or excluding coronary artery aneurysms, intraluminal or mural thrombi, regurgitant cardiac valves, myocardial dysfunction, or pericardial effusion.
  • 20. Histology  The choice of tissue is guided by the clinical setting using the most accessible involved organ or tissue.  Various organs biopsied include the skin, lungs and kidney (pulmonary-renal syndrome), sural nerve, and muscle.
  • 21. Skin is the most commonly biopsied area.  A deep punch biopsy (to diagnose changes in the deeper vessels at the dermal level) is ideal.  Cutaneous vasculitis is mostly characterized by involvement of the post-capillary venules with endothelial cell swelling, neutrophilic invasion of blood vessel walls, presence of disrupted neutrophils (leukocytoclasia), extravasation of red blood cells, and fibrinoid necrosis of the blood vessels walls.
  • 22. These features are termed cutaneous necrotizing vasculitis (CNV), which refers to leukocytoclastic vasculitis affecting the small and medium vessels supplying nutrients to the skin .
  • 23. Along with leukocytoclasia, the biopsy may reveal areas of panniculitis, particularly in PAN.  Samples should be subjected to immunofluorescence particularly for possible HSP (IgA deposits), ANCA associated vasculitis (pauci-immune necrotising vasculitis) or secondary vasculitis like SLE (immunoglobulins and complement deposits at the dermo- epidermal junction).
  • 24. Renal biopsies are carried out if clinical features and urinalysis suggest renal involvement.  Various changes from focal to segmental glomerulonephritis can be seen.  Subepithelial deposits can be seen on electron microscopy
  • 25. As the yield from tissues like sinuses, nose, ears, and trachea is low (<50%), invasive procedures such as open or thoracoscopic lung biopsies or renal biopsies are often used to diagnose Wegeners granulomatosis (WG).
  • 26. Serology  ANCA are present with a high specificity of up to 98% in the specific group of AAV.  Two staining patterns for ANCA are detected by ELISA. 1. Cytoplasmic ANCA (C-ANCA) representing antibody to proteinase 3 is present in 90% of patients with active diffuse WG i.e., with high specificity. 2. Perinuclear ANCA (P-ANCA) caused by antimyeloperoxidase antibody is suggestive of involvement of small to medium sized arteries in conditions such as microscopic polyangiitis.
  • 27. The presence of Von Willebrand factor antigen is a surrogate marker for SVV.  Elevated IgA levels may be found in 50–70% of HSP.  Anti-streptolysin O titres may be elevated with cutaneous polyarteritis (C- PAN).  Serology for Hepatitis B and C are relevant in cases of polyarteritis nodosa (PAN).
  • 29. How are Children with Vasculitis Managed?
  • 30. What is the Prognosis of the Various Vasculitides?  The majority of children with HSP make a full and uneventful recovery with no evidence of ongoing significant renal disease.  Mortality is around 1–2% with severe gastrointestinal or renal involvement.  Renal involvement is the most serious long-term complication of HSP and occurs in 5%.
  • 31. KD has a good prognosis, with 1–2% acute mortality rate due to myocardial infarction, having been reduced further to less than 1% due to increasing awareness of clinicians and prompt treatment.
  • 32. Ozen et al reported in a retrospective series of childhood PAN an improved outcome compared to that reported in adults with only one (1.1%) death and two (2.2%) patients with end-stage renal disease among 110 patients.  C-PAN has a good prognosis, albeit with recurrent relapses and remissions.
  • 33. The AAV carry considerable disease-related morbidity and mortality mainly due to progressive renal failure or aggressive respiratory involvement.  For Churg-Strauss syndrome (CSS) in children, the most recent series reports mortality of 18%, attributable to disease rather than to therapy.  The risk of organ damage is related to the duration of active disease.
  • 34. With proper treatment, clinical remission is often achieved within the first year.  The remission may be life-long, but long-term maintenance therapy is frequently required.  Periods of disease remission may be interrupted with relapses requiring more intensive therapy.