Childhood vasculitis encompasses several conditions involving inflammation of blood vessels. The most common types in children are Kawasaki disease, Henoch-Schonlein purpura, Takayasu arteritis, and polyarteritis nodosa. Kawasaki disease is a medium vessel vasculitis that predominantly affects coronary arteries. Henoch-Schonlein purpura is a small vessel vasculitis characterized by palpable purpura on the lower extremities and joints. Takayasu arteritis causes inflammation of large vessels like the aorta. Polyarteritis nodosa involves necrotizing inflammation of small and medium vessels.

1. CHILDHOOD
VASCULITIS
Surg Lt Cdr
Jayakrishnan VY
Moderator : Lt Col
Reema Solanki

2. REFERENCES
• Nelson Textbook of Pediatrics
• Kawasaki Disease – An Indian Perspective, Surjit
singh and *Tomisaku Kawasaki
• Pediatric Vasculitides: A Generalists Approach,
Raju P. Khubchandani & V. Viswanathan
• Henoch-schonlein purpura: The Chandigarh
Experience
• KIDIGO Clinical Practice Guidelines 2012

3. VASCULITIS
Inflammation in a blood vessel –primary process or secondary to an underlying
disease.
Vasculopathy -abnormality of blood vessels occurring due to inflammation,
degeneration or intimal proliferation.
50 cases per 100,000 children per year
Only HSP and KD are more common in children vs adults

4. CLASSIFICATION OF VASCULITIS
PREDOMINANT LARGE VESSEL VASCULITIS
PREDOMINANT MEDIUM VESSEL VASCULITIS
PREDOMINANT SMALL VESSEL VASCULITIS
OTHERS

6. KAWASAKI DISEASE
• Medium vessel vasculitis
• Special predilection for the coronary arteries
• Commonest vasculitis across all ages
• Typical constellation of sequential clinical findings
• Indian data - 0.51 to 4.54/100,000 children aged
below 15 years during the period 1994-2007
• Socioeconomic status – more affluent population

7. • 80% -under the age of 5 years
• Peak age of onset of KD in Japan is 6 to 11
months
• INDIA : 30% - above 5 years of age
• Male-to-Female ratio is 1.5:1.0.

8. ETIOLOGY
• Very reminiscent of an
infectious etiology
• Age profile of the disease
• Occur in epidemics
• Staphylococcal and
streptococcal superantigens
(exanthem and peripheral
desquamation)

9. PATHOLOGY
• Pan-arteritis - medium sized vessels
(coronaries)
• Acute phase- heart, meninges, lungs, lymph
nodes, and liver
• Initial phase - Polymorphonuclear infiltration-
saccular aneurysm
• Later- Mononuclear cells
• During recovery- inflammation subsides -
leaves fibrous connective tissue - with
proliferation of intima- stenosis
• CAA are often not seen in the first 5-7 days

10. • Patients are prone to develop
thrombosis later in life leading to
myocardial ischemia, myocardial
infarction and sudden death.
• Adult onset ischemic heart disease -
result of KD in childhood

11. DIAGNOSIS
• Remains purely clinical diagnosis
• Recognition of a typical temporal
sequence
• None of the features taken individually
being of any diagnostic significance
whatsoever
• Entire clinical spectrum is not seen at
any one particular point of time

12. CLINICAL CRITERIA
• Sensitivity and Specificity of criteria
has not been worked out
• Experienced clinician may be justified
in making a diagnosis of KD even
when all the criteria are not being
met

15. SEQUENCE OF ILLNESS
ACUTE
(0-10 days)
• Fever
• Extreme irritability
• Bilateral
conjunctival
injection
• Myocarditis
• IVIG most beneficial
SUBACUTE
(10-28 days)
• All acute phase signs
start disapearing
• Periungual
desquamation
• CAA
• Thrombocytosis
CONVALESCENT
• All clinical signs
have disappeared
• Till the acute phase
parameters
normalizes
• 6-8 weeks

16. FEVER
• High (≥38.3°C [101°F]), unremitting, and
unresponsive to antibiotics.
• May persist for 3-4 wk
• Not associated with the typical features of an
upper respiratory catarrh
• Responds promptly to administration of IVIG

17. CONJUNCTIVAL INJECTION
• Shortly after the onset of fever
• Non-exudative
• Bulbar conjunctiva > Palpebral
conjunctiva
• Perilimbal sparing

18. MUCOSAL CHANGES
• Redness of the mouth and lips with
dryness and fissuring
• Peeling of the lips
• Strawberry tongue
• Most Prominent - erythema of the lips

19. CHANGES IN THE PERIPHERY
• Redness of palms and soles
• ‘Indurative edema’ on the
dorsal aspects
• Only seen in the acute phase
• Periungual desquamation of
fingers and toes - SUBACUTE
• Beau’s lines- transverse ridged
grooves nail

20. RASHES
• Diffuse non specific
MACULOPAPULAR
ERYTHEMATOUS rash
• Perineal attenuation
• Scarlatiniform rash and an
erythema multiforme-like rash
• Vesicular rash is never seen

21. CERVICAL
LYMPHADENOPATHY
• Seen in only 50-75% of
patients
• Febrile unilateral acute
cervical adenitis not
responding to antibiotics

22. ATYPICAL KD
&
INCOMPLETE
KD
• Clinical features not commonly associated with
• Significant hypertension, nephritis, seizures or a
cerebrovascular accident in a patient with KD
• Fever and fewer than four of the other clinical
features

23. LABORATORY FINDINGS
• Pyuria
• Polymorphonuclear leucocytosis
• ESR CRP
• Normocytic anemia
• Thrombocytosis
• Transaminitis
• CSF- Pleocytosis
• Echocardiography

24. CARDIOVASCULAR COMPLICATIONS
• 15% to 25% of untreated KD patients
• Diffuse dilatation (ectasia) and Aneurysm formation
• Majority of the aneurysms regress or show a decrease
• Coronary stenosis
• Premature atherosclerosis
• Myocardial ischemia-1.9%
• 39% of Persistent aneurysms
• Myocarditis/ valvulitis/ Pericardial effusion

25. TREATMENT
• IVIG- single dose of 2 g/kg
• Reduces the chances of development of CAA from
20-25% to 1-2%
• IVIG Resistant KD : 15% of KD , Febrile after 36 hrs
– Repeat IVIG / IV MethylPred/ Infliximab
• Defer parenteral virus vaccines for 3 months
• Aspirin : 80-100 mg/kg/d given every 6 hours
• After Acute Phase - 3 to 5 mg/kg/day till
convalescence
• Echo : At diagnosis > 2-3 weeks > 6-8 weeks

27. ECHO CRITERIA
• LAD /RCA Z Score ≥ 2.5
• Coronary arteries meet Japanese HM
criteria for aneurysms
• ≥3
• Perivascular Brightness
• Lack of tapering
• Decreased LV function
• MR
• Pericardial effusion
• CA Z score 2-2.49

28. HENOCH
SCHONLEIN
PURPURA

29. • Small vessel vasculitis
• Leukocytoclastic vasculitis
• IgA deposition (IgA1) – Skin, GIT, Joints , Kidney
• 14-20/100,000 children per year
• M:F 1.2-1.8 : 1
• 3 and 10 yr
• winter and spring

30. PATHOLOGY
• Vasculitis of the dermal capillaries
and postcapillary venules.
• Renal histopathology -
Endocapillary Proliferative
Glomerulonephritis
• Focal segmental process -
Extensive crescentic involvement
• Immunofluorescence - IgA
deposition in walls of small
vessels

31. PATHOGENESIS
• UNKNOWN
• Infectious triggers - GAS, Staphylococcus aureus,
Mycoplasma, and Adenovirus
• Genetic predisposition – Clusters in family

32. CLINICAL MANIFESTATIONS
• Hallmark :Palpable purpura
• Starting as pink macules or wheals and
developing into petechiae, raised purpura, or
larger ecchymoses.
• Symmetric
• Gravity-dependent areas (lower extremities)
• Pressure points (buttocks)
• lasting 3-10 days, may reccur
• Subcutaneous edema - dorsa of hands and feet,
periorbital area, lips, scrotum, or scalp

33. • Musculoskeletal (75%) – Arthralgia & Arthritis
Self-limited and Oligoarticular – lower
extremities – non erosive
• Gastrointestinal (80%)- Abdominal pain,
Vomiting, Diarrhea, Paralytic ileus and
Melena
• Intussusception, Mesenteric ischemia,
Intestinal Perforation
• Renal (50%) - Microscopic hematuria,
Proteinuria, Hypertension, Frank nephritis,
Nephrotic syndrome, Acute or Chronic renal
failure

34. SYSTEMATIC
REVIEW
34% Abnormal urinalysis (79% having isolated
hematuria with or without proteinuria)
21% of those with kidney involvement (or 7.2% of
all cases) developed a nephritic and/or nephrotic
syndrome.
90% - kidney involvement by 8 weeks
97% - kidney involvement by 6 months.
Recurrence of rash and other symptoms –
1/3 rd

35. • Neurologic – Intracerebral hemorrhage,
Seizures, Headaches and Behavior
changes
• Orchitis (25%)
• Carditis
• Inflammatory eye disease
• Testicular torsion
• Pulmonary haemorrhage.

36. DIAGNOSIS

37. LAB
FINDINGS
Leucocytosis
Thrombocytosis
Mild Anemia
Elevated CRP & ESR
Occult Blood in Stool
Hypoalbuminemia
S. IgA elevated
RFT
USG – Bowel wall odema/ Intussuseption
Skin & Kidney Biopsy

38. TREATMENT
• Supportive
• Hydration, Nutrition, Analgesia
• Steroids: Prednisolone 1mg/kg/day x 1-2
wks – tapering
• Indication : CGN / GI bleed
• Persistent Proteinuria >0.5-1 g/day/1.73
m2 - ACE-I or ARBs
• > 1 g/day/1.73 m2 and GFR >50
ml/min/1.73 m2- 6 month course of
corticosteroid therapy
• Other Immunosuppressants

39. TAKAYASU ARTERITIS
• Pulseless disease
• Chronic large vessel vasculitis
• Inflammation starting @ vas vasorum
• Infiltrated by T cells, Natural killer cells, Plasma cells,
Macrophages
• Giant cells and granulomatous inflammation – Media
• Aneurysms - Subclavian, Renal, and Carotid arteries
• Pre-pulseless : Fever, malaise, weight loss, headache,
hypertension, myalgias, arthralgias, dizziness, and
abdominal pain

40. • Diminished pulses, Asymmetric BP,
claudication, Raynaud phenomenon,
Renal failure, and symptoms of pulmonary
or cardiac ischemia
• Aortic Insufficiency
• Pericardial Effusion, Pericarditis, Pleuritis,
Splenomegaly, and Arthritis

41. TREATMENT
• 1-2 mg/kg/day of prednisone - gradual
dosage tapering.
• Steroid-sparing therapy : Methotrexate
or Azathioprine.
• Severe cases : Cyclophosphamide
• Antihypertensives

42. POLYARTERITIS
NODOSA
• Systemic Necrotizing Vasculitis – rare in
pediatrics
• Small & Medium Vessels
• Limited to Skin – Cutaneous PAN
• Boys & Girls equally affected
• Post infectious AI response – HBV, HCV,
GAS, TB, CMV, Parvo B19
• Segmental involvement – usually at
bifurcation
• Non Granulomatous
• Various stages of inflammation –
Fibrinoid necrosis

43. • Vessels – thickened & Narrow –
end organ damage

44. DIAGNOSIS &
TREATMENT
• Biopsy – Necrotizing vasculitis
• Gold Standard – Conventional Arteriography- “
beads on string appearance”
• Non-Specific lab findings
• HBV & HCV testing in all patients
• Rx: Steroid : Prednisolone : 1-2mg/kg/day or IV
pulse 30mg/kg/day
• Usually combined with Cyclophosphamide (Oral/IV)
• Life threatening : Plasmapheresis
• Cutaneous nodules- ulcerate, HTN, CKD, Mesentric
vasculitis, CVA

45. ANCA–ASSOCIATED VASCULITIS
• Small vessel
• Circulating ANCAs
• Pauci-immune complex deposition
• 3 Distinct forms
GRANULOMATOSIS WITH POLYANGITIS
EOSINOPHILIC GRANULOMATOSIS (CHURG
STRAUSS)
MICROSCOPIC POLYANGITIS

46. GRANULOMATOSIS WITH POLYANGITIS (GPA)
• Necrotizing granulomatous small and medium vessel vasculitis
• Female predominance of 3-4 : 1
• Neutrophils and monocytes are activated PROTEINASE-3 (PR3)
URT LRT RENAL/ OTHERS
SINUSITIS COUGH OCCULAR
NASAL ULCER WHEEZ NEUROPATHIES
EPISTAXIS DYPSNOEA HEMATURIA
OTITIS MEDIA HEMOPTYSIS HYPERTENSION
HEARING LOSS PULMONARY HMG PURPURA/ ULCERS
SADDLE NOSE SUBGLOTIC STENOSIS

48. • ESR/ CRP/ ANEMIA/ LEUCOCYTOSIS/
THROMBOCYTOSIS
• c-ANCA /anti-PR3 antibodies
• Rx : 2 mg/kg/day oral or
• 30 mg/kg/day × 3 days IV Steroid
• PLUS Oral Cyclophosphamide (2 mg/kg/day)
• Step down to – MTX/ MMF/ AZA by 3-6
months
• Septran Prophylaxis against P.Jeroveci
• If only RTI – Oral Prednisolone + MTX

49. CHURG STRAUSS SYNDROME
• Small vessel necrotizing granulomatous
• Refractory Asthma and Peripheral Eosinophilia
• Perivascular eosinophilic infiltrates
• Inflammation of the upper and lower
respiratory tracts
• C/c rhinitis/sinusitis, Nasal polyposis and
difficult to treat asthma
• Renal involvement uncommon
• p-ANCA - MPO

52. THANK YOU