Indian Journal of Pediatrics

https://doi.org/10.1007/s12098-023-04958-2

REVIEW ARTICLE

Childhood Vasculitis
Sujata Sawhney1

Received: 25 April 2023 / Accepted: 10 November 2023

© The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation 2023

Abstract
Of the primary vasculitis pediatricians are familiar with, Kawasaki disease and IgA vasculitis are the most common. The
other large, medium and small vessel vasculitis are seldom seen in practice. Though rare, early diagnosis and appropriate
management is critical for the best outcome. Primary vasculitis in the pediatric age group have several differential diagnoses
which range from infections to monogenic causes such as deficiency of Adenosine Deaminase -2. Each child, therefore,
needs a careful systematic approach.

Keywords Childhood vasculitis · IgA vasculitis · Kawasaki disease · Clinical approach to vasculitis in children

Introduction of less than 1 per 100000 [4, 5]. Anti-neutrophilic cyto-

plasmic antibody (ANCA) associated vasculitis (AAV) are
The term vasculitis implies inflammation of the blood vessel seldom seen in childhood with the reported incidence of
walls. This can involve blood vessels of any size and also 0.5–6.3 patients per million population/year [6, 7]. Finally,
involve either the arterial tree or venous channels. Inflam- Takayasu arteritis (TA) has an incidence of 2.6/million [8].
mation of the blood vessels causes reduction in the blood
supply to the distal organs that the vessel supplies and this
causes tissue ischemia or infarction [1]. The clinical mani- Classification and Pathogenesis
festations of vasculitis thus depend upon the site, size and
degree of vessel involvement. Vasculitis remains an enigma The current classification criteria for childhood vasculitis
for the practicing pediatrician. The reasons are many: the have been developed by European League Against Rheu-
clinical manifestations are protean. The mimics are many. matism/Pediatric Rheumatology European Society/Pedi-
Finally, there are no diagnostic tests for two of the most atric Rheumatology Trials Organization (EULAR/PReS/
common vasculitis of childhood: Kawasaki disease (KD) PRINTO), (Table 1) [9–11]. This classification system is
and IgA vasculitis. based upon the predominant vessel involved and is thus
classified into large, medium and small vessel vasculitis.
Additionally, there are several secondary causes of vas-
Epidemiology culitis listed as well. IgA vasculitis, granulomatosis with
polyangiitis (GPA), polyarteritis nodosa (PAN) and finally
Amongst the primary vasculitis, IgA vasculitis, (previously TA have validated classification criteria (Table 2) [9].
called Henoch Schönlein purpura) has a reported incidence The pathogenesis of vasculitis is complex; genetic pre-
of 29.9 per 100000 population [2]. The incidence of KD disposition in concert with environmental trigger leads to
varies from 315 patients per 100000 population in Japan to immune activation resulting in vascular inflammation and
4.5–25 per 100 thousand in the non-Asian population [3]. damage [13].
Polyarteritis nodosa (PAN) is very rare and has an incidence

Clinical Approach
* Sujata Sawhney
[email protected]
It can be simplified by keeping the classification in mind and
1
Division of Pediatric Rheumatology, Institute of Child by analyzing the clinical features in a systematic fashion.
Health, Sir Ganga Ram Hospital, New Delhi 110022, India

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 Indian Journal of Pediatrics

Table 1  EULAR/PReS/PRINTO classification of childhood vasculitis Kawasaki Disease

I Predominantly large-vessel vasculitis
This medium vessel vasculitis characteristically occurs in
• Takayasu arteritis
young children majority being under 5 y of age [15]. The
II Predominantly medium-sized vessel vasculitis
criteria to diagnose KD are persistent fever for >5 d and 4/5
• Childhood polyarteritis nodosa
of the following criteria: non-purulent conjunctivitis with
• Cutaneous polyarteritis
perilimbal sparing, oral mucositis, polymorphous rash, uni-
• Kawasaki disease
lateral tender cervical lymphadenopathy, and peripheral limb
III Predominantly small-vessel vasculitis
changes which acutely presents with edema of the dorsum
(A) GRANULOMATOUS
of the hands and feet and in the subacute stage with peel-
• Wegener’s granulomatosis
ing, classically periungual (Fig. 1a). The challenge is to
• Churg-Strauss syndrome
recognize the child with incomplete disease (fever and <3
(B) NON-GRANULOMATOUS
criteria) by 5–7 d of illness. Sequential appearance of clini-
• Microscopic polyangiitis
cal features and irritability are important clinical clues. The
• Henoch-Schönlein purpura
latter is likely due to aseptic meningitis [13]. The 2017 KD
• Isolated cutaneous leucocytoclastic vasculitis
position paper stressed the importance of serum albumin,
• Hypocomplementic urticarial vasculitis
acute phase response, serum glutamate pyruvate transami-
IV Other vasculitides
nase (SGPT) and sodium in addition to echocardiography
• Behçet disease
• Vasculitis secondary to infection (including hepatitis B associated
for early diagnosis [3]. This is a monophasic illness and
polyarteritis nodosa), malignancies, and drugs, including recurrences are rare.
hypersensitivity
• Vasculitis associated with connective tissue diseases Polyarteritis Nodosa (PAN)
• Isolated vasculitis of the central nervous system
• Cogan syndrome It is a necrotising medium vessel vasculitis that presents
• Unclassified with fever, failure to thrive, arthralgia, myalgia, digital
gangrene (Fig. 1b), skin rashes including livedo reticularis
(Fig. 1c), vasculitic ulcers (Fig. 1d), significant gastrointes-
tinal complaints, involvement of the kidneys due to arcuate
Large Vessel Vasculitis (LVV) and lobular arterial involvement and occasionally neurologi-
cal involvement. Cutaneous PAN presents only with skin
TA which is a granulomatous pan arteritis affecting the aorta lesions.
through its length and also has the potential to involve the Among 69 patients, fever and skin rashes were seen in
coronary and pulmonary circulations is the only LVV seen in more than 85% children [14]. Majority do not have a relapse,
children. Depending upon the site of involvement TA is clas- however 35% may relapse [13]. Young age of onset, posi-
sified into five types. The disease has two phases: the acute tive family history and associated hematological or immuno-
inflammatory phase followed months to years later by the logical abnormalities needs testing for Adenosine deaminase
chronic phase with stenosis and aneurysmal dilatation of the 2 (ADA2) deficiency [16].
involved vessel. TA is suspected when an adolescent presents
with prolonged pyrexia, arthralgia, myalgia, limb claudica- Small Vessel Vasculitis
tion with asymmetric peripheral pulses, and hypertension. The
acute phase has non-specific features and this makes the diag- Small vessel vasculitis is inflammation of the arterioles, ven-
nosis a huge challenge. Fever, headache and heart failure are ules and capillaries. Except for IgA vasculitis, primary small
more common in pediatric TA as compared to adults. Retin- vessel vasculitis is rare.
opathy and skin rashes/nodules are other described features
[14]. This condition has a long indolent course and is prone to IgA Vasculitis (HSP)
periods of disease activity and then remission [13].
The classical patient is a young boy 4–8 y of age with a
Medium Vessel Vasculitis triad of lower limb symmetrical palpable purpura (Fig. 1e),
abdominal angina and predominant lower limb arthritis. In
In medium vessel vasculitis, there is involvement of the vis- many children this is preceded by an upper respiratory infec-
ceral arteries and veins and their initial branches. The two tion. Abdominal pain occurs in up to 70% of children. The
important medium vessel vasculitides are KD and PAN. acute complications in IgA vasculitis are gastrointestinal

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 Table 2  Classification criteria and key features of some childhood vasculitides
Disease Diagnostic criteria Comments Management Ref.
(EULAR/PRES/PRINTO)

HSP/IgA vasculitis Mandatory criterion: Purpura or petechiae with lower • Commonest vasculitis in childhood • Self-limiting in majority [12]
limb predominance • IgA deposition on skin biopsy helpful but not • Steroids for severe abdominal pain, orchitis, cer-
Additional: Minimum 1 out of 4 criteria mandatory ebral vasculitis, renal disease, pulmonary hemor-
1. Diffuse abdominal pain with acute onset • 87% specificity for diagnosis rhage
Indian Journal of Pediatrics

2. Histopathology showing leukocytoclastic vasculitis, • Mild renal involvement common • Management of renal disease per the renal biopsy
or proliferative glomerulonephritis with predomi- • 1% develop end stage renal disease • There is no role of prophylactic steroid to prevent
nant immunoglobulin A (IgA) deposits • Follow for renal involvement for 6 mo from onset renal involvement
3. Arthritis or arthralgia of acute onset
4. Renal involvement in the form of proteinuria or
hematuria
PAN Characteristic histopathology or typical angiographic • Deep tissue biopsy of the skin may help to clinch Patients with systemic disease are treated with an [9]
abnormalities (mandatory criteria) plus 1/5 the diagnosis induction regimen of either IV cyclophosphamide
1. Skin involvement • Percutaneous renal biopsy should be avoided or mycophenolate mofetil followed by a prolonged
2. Myalgia/muscle tenderness because of the risk of bleed from renal aneurysms remission phase with either mycophenolate mofetil
3. Hypertension • Catheter digital substraction angiography is the or Azathioprine
4. Peripheral neuropathy investigation modality of choice, however, CT angi-
5. Renal involvement ography is more often done for practical reasons
• STING associated vasculopathy of infancy (SAVI)
and deficiency of Adenosine deaminase -2 (DADA-
2) are important differential diagnosis
GPA 3/6 needed to classify pediatric vasculitis as GPA: • Classification criteria alone should not be used for The patients are treated in two phases:
1. Histopathology (granulomatous inflammation diagnosis • Induction of remission: With Rituximab or IV
within the arterial wall or in the perivascular or • The ANCA testing should be two step: on indirect CYC. Some patients may benefit with PLEX
extravascular area) immunofluorescence and ELISA for the MPO and • Maintenance phase up to 2 y with IV Rituximab
2. Upper airway involvement (chronic purulent or PR3 assay every 6 mo
bloody nasal discharge or recurrent epistaxis/crusts/
granulomata, nasal septum perforation or saddle
nose deformity, chronic or recurrent sinus inflam-
mation)
3. Laryngo-tracheo-bronchial stenoses (subglottic,
tracheal or bronchial stenosis)
4. Pulmonary involvement (chest X-ray or CT
showing the presence of nodules, cavities or fixed
infiltrates)
5. ANCA positivity by IIF or ELISA (P-ANCA/MPO-
ANCA or C-ANCA/PR3- ANCA)
6. Renal involvement (proteinuria >0.3 g/24 h or >30
mmol/mg of urine albumin/creatinine ratio on a spot
morning sample, hematuria or red blood cell casts in
the urinary sediment or ≥2+ on dipstick or necrotis-
ing pauci immune GN

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 Indian Journal of Pediatrics

bleeding, perforation and intussusception. Isolated acute

GPA Granulomatosis with polyangiitis, HSP Henoch Schönlein purpura, IIF Indirect immunofluorescence, MMF Mycophenolate mofetil, MPO Myeloperoxidase, MTX Methotrexate, PAN Pol-
ANCA Anti neutrophil cytoplasmic antibody, CYC​ Cyclophosphamide, ELISA Enzyme-linked immunosorbent assay, EULAR European League Against Rheumatism, GN Glomerulonephritis,
Ref. abdominal pain without rash or arthritis can be a diagnostic
challenge. Intussusception or gut perforation should be ruled
Induction with CYC or Methotrexate and mainte- out [13]. Older adults with this condition should be screened
for malignancies [12]. IgA vasculitis associated nephritis
occurs in 20%-60% and is usually mild, but may occur up to
6 mo after onset, thus close follow-up with urine examina-
nance with MTX or MMF are used

tion and blood pressure monitoring is required. Nephritis

yarteritis nodosa, PLEX Plasma exchange, PRES Pediatric Rheumatology European Society, PRINTO Pediatric Rheumatology Trials Organization, PR3 Proteinase-3
may vary from microscopic hematuria to acute nephritic or
nephrotic presentation. The Single Hub and Access for Pedi-
atric Rheumatology in Europe (SHARE) consortium sug-
gests nephrology review including proteinuria more than 1 g
at 4 wk, the presence of nephritic or nephrotic syndrome,
Management

or the presence of deranged renal function [17]. Orchitis,

cerebral vasculitis and rarely pulmonary hemorrhage may
rarely occur. Recurrence occurs in about one-third of chil-
dren usually within 4 mo of remission. Upto 2% of children
may have chronic renal disease [13, 18]. Acute hemorrhagic
Careful clinical examination, including 4 limb blood

edema of infancy is likely a variant of HSP and presents as

pressure are important to identify these patients

a triad of fever, edema and rosette shaped purpura. Biopsy

shows leukocytoclastic vasculitis with abundant neutrophils.

Anti‑Neutrophilic Cytoplasmic Antibody (ANCA)

Associated Vasculitis (AAV)

The three small vessel vasculitides of this category are GPA,

eosinophilic granulomatosis with polyangiitis (EGPA) and
microscopic polyangiitis (MPA) [1, 4]. Broadly, AAV should
Comments

be suspected in a patient with pyrexia of unknown origin,

vasculitic skin rash, nasal crusting and bleeding, peripheral
or central nervous system involvement, scleritis, unexplained
arthritis, myalgia, serositis, unexplained pulmonary, gastro-
Typical angiographic abnormalities of the aorta or its
main branches and pulmonary arteries (mandatory

intestinal, cardiovascular or renal disease [19]. These patients

are systemically unwell. Children with GPA present with
sinusitis, nasal crusting and bleeding early on followed by
2. Blood pressure discrepancy in any limb

fever, fixed pulmonary lesions, either nodular or cavitating,

scleritis and renal involvement in the form of active urinary
criterion) plus one of five criteria:

sediment and hypertension. An important clinical feature in a

5. Elevated acute phase reactant
1. Pulse deficit or claudication

child with GPA is the presence of sub-glottic stenosis which is

(EULAR/PRES/PRINTO)

rarely reported in adult patients [1]. Patients with microscopic

polyangiitis present with predominant renal disease, often rap-
Diagnostic criteria

idly progressive glomerular nephritis. The child with EGPA

4. Hypertension

has shifting pulmonar y shadows, eosinophilia and prolonged

pyrexia with a background of asthma. EGPA is a rarity in clin-
3. Bruits

ical practice. Patients with AAV can also present with multi-
ple skin lesions- nodular lesions, purpura, livedo and ulcers
[1]. Children with AAV tend to have more aggressive disease,
Table 2  (continued)

more relapses and accrue more organ damage as compared to

adults [20]. The largest pediatric AAV cohort of 105 patients,
Aortoarteritis

the ARChiVe (A Registry for Childhood Vasculitis: e-entry)

Disease

cohort found that 42% achieved remission by 12 mo and 61%

had inactive disease on higher doses of corticosteroids [21].

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Indian Journal of Pediatrics

Fig. 1  (a) Periungual peeling a b c

seen in the subacute phase of
Kawasaki Disease (KD). (b)
Digital gangrene in a case of
polyarteritis nodosa (PAN). (c)
Livedo reticularis in a case of
PAN. (d) Vasculitic ulcer in a
child with PAN. (e) Palpable
purpura in a child with IgA
vasculitis. (f) CT angiogram in
a case of PAN showing aneu-
rysms in branches of the renal
arteries

d e f

Childhood Primary Angiitis of the Central Nervous may be warranted. The most consistent biomarker in patients
System (cPACNS) with IgA Vasculitis is serum levels of Galactose deficient
IgA1 (Gd-IgA1) [18].
The primary pathology is inflammation of the cerebral Based on clinical suspicion ANCA is ordered. Protein-
vessels. These children present with encephalopathy, sei- ase-3 (PR3) antibodies are seen in up to 67% of children
zures, stroke and sometimes psychiatric features. Early with GPA and myeloperoxidase antibodies are present in
recognition by doing magnetic resonance (MR) angiogram up to 55% of children with MPA [23]. If lupus is suspected
and sometime brain biopsy and adequate immunosuppres- then antinuclear antibody (ANA), anti-double stranded
sion are critical for survival [22]. DNA, C3 and C4 are requested. In a patient suspected
to have PAN, CT angiography can show medium vessel
involvement. These patients demonstrate aneurysms in the
Laboratory Evaluation renal (Fig. 1f), mesenteric, splenic and hepatic arteries. If
CT angio is negative, sometime conventional angio may be
Investigations are planned to diagnose the condition, exclude needed. CT angio is also used to demonstrate the involve-
the mimics, and evaluate the extent of disease. Routine base- ment of the aorta and its branches in patients suspected to
line blood counts, liver and renal tests, urine exam, X-ray have TA. Both CT and MR angiography give good infor-
chest, ultrasound abdomen and 2D echo are needed in most mation about the vessel lumen. MR angiographic study,
children. Echo can show aortic involvement in TA, coro- specifically for TA is preferable as vessel wall inflam-
nary artery changes in KD, and rarely a left atrial myxoma mation can be delineated well and there is no radiation
that may mimic systemic vasculitis [14]. Additionally, HIV, exposure. Due to radiation exposure PET-CT is avoided
Hepatitis B screening and in many children anti streptolysin in children. PET MRI of available may be preferable [19].
O (ASO) titre are asked for. In the appropriate clinical set- Tissue biopsy [e.g. kidney (light and electron micros-
ting, blood culture and PCR testing for bacterial infections copy), lung, nasopharynx or sinuses] and associated

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 Indian Journal of Pediatrics

immunohistochemistry are recommended to confirm the molecular weight Heparin to use of only low dose Aspi-
diagnosis of AAV [19]. Of note, in patients with suspected rin. This depends upon the coronary artery lesion size: z
PAN, renal biopsies should be avoided because of risk of score of >10 warrants combined therapy with low molecu-
bleeding. In a child with IgA vasculitis, the skin biopsy lar Heparin and Aspirin ± Clopidogrel. All children with
should be sent for immunofluorescence in addition to KD warrant long-term follow-up with tests for myocardial
histopathology. ischemia depending upon the maximum size of the coro-
As the differential diagnosis of PAN requires considera- nary artery lesion at presentation [3, 26].
tion of inherited autoinflammatory syndromes such as ADA-2
deficiency, STING associated vasculopathy of infancy (SAVI)
IgA Vasculitis
and chronic atypical neutrophilic dermatosis with lipodystro-
phy and elevated temperature (CANDLE) syndrome in the
The treatment is usually symptomatic. Non-steroidal
appropriate clinical setting, whole exome sequence may need
anti-inflammatory drugs (NSAIDs) can help the arthri-
to be done.
tis. Short course of steroid may be given for the patient
with severe abdominal angina or bothersome purpura.
There is no evidence to suggest that preemptive steroids
Assessment of Severity of Pediatric Vasculitis will prevent renal involvement. In the presence of sig-
nificant proteinuria or deranged renal function, the most
Paediatric Vasculitis Activity Score (PVAS) is a weighted
common renal pathology noted is a focal segmental pro-
score from 0–63, with higher scores indicating higher
liferative glomerulonephritis. With rapidly progressive
disease activity. The Paediatric Vasculitis Damage Index
disease there may be crescentic glomerulonephritis as
(PVDI) is similarly based on a modified version of the adult
well. For the child with severe renal involvement, high
VDI tool; the score ranges from 0 to 73 and represents per-
dose steroids and immunosuppressive drugs like CYC,
manent organ damage [24, 25]. These outcome measures
(occasionally with plasmapheresis) remains the mainstay
capture objective disease assessment and damage and are
of treatment [13, 17].
important for use in clinical trials.

ANCA Associated Vasculitis (AAV)

Management Approach
Broadly, the management of these children is divided into
SHARE consortium has published easy to use guidelines the induction phase which aims to get the disease into
for the common and rare vasculitis in childhood [17, 19, remission and the maintenance phase which aims to reduce
26]. There is a lack of randomized clinical trial data for the relapses [27–32]. The management decisions for treat-
management of childhood vasculitis. This is predominantly ing a child with AAV are dependent upon the age of the
because of the rarity of these conditions [13]. Broadly, the patient, the presence of life-threatening organ involvement,
aim is to reduce childhood exposure of cytotoxic drugs whether the patient is a new case or a relapsed patient, and
such as cyclophosphamide (CYC) and avoid long courses any other co-morbidities that the patient may have.
of high dose steroids. Trial data from the adult literature suggests that there
is no significant difference in between the use of IV CYC
or Rituximab (RTX) for induction. Additionally, as per the
Kawasaki Disease
CYCLOPS trial, there was no advantage of using oral CYC
over IV CYC. The RITUXVAS trial confirmed that there
KD is unique as it occurs in infants less than one year
was no value in combining RTX and IV CYC for induction.
of age and stands out as it is the only vasculitis treated
The RAVE trial suggested that IV RTX was non-inferior to
with intravenous immunoglobulin (IVIG). Patients with
oral CYC for induction remission. The MEPEX and PLEXI-
KD can present with many complications. Some of them
VAS trials addressed the role of plasma exchange (PLEX)
are the presence of coronary artery lesions, presentation
for the acutely unwell patient with respect to renal outcomes.
with shock, association with hemophagocytic lymphohis-
Data suggested that PLEX was useful acutely, but there was
tiocytosis or co-associated infections. Thus, it is impor-
no definite long-term beneficial effect. With limited disease,
tant to identify the patient with KD who will benefit with
induction therapy in non-renal patients may be tried with
upfront primary intensification of treatment with steroid
Methotrexate (MTX).
or infliximab. In addition to the anti-inflammatory therapy,
Data from the MAINRITZAN trial suggests that RTX
the American Heart Association suggested antithrombotic
is significantly better for maintenance as compared to
treatment for these patients. This can range from low

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Indian Journal of Pediatrics

Azathioprine (AZA). Once the patient achieved remission Mimics of Primary Systemic Vasculitis [15]
RTX is given at week 0 and 2 and then every 6 mo until a
total follow up of 18 mo [27–32]. EGPA and MPA are very Infections
rare in the pediatric age group and usually the same proto-
cols as detailed above is followed [33]. Severe infections such as meningococcal sepsis may present
with extensive skin purpura and necrosis. Infective endo-
Polyarteritis Nodosa (PAN) carditis is an important mimic and needs screening with
2D echo and blood cultures. HIV, hepatitis B and C may
The MYPAN trial results are the only international, open- be important differentials and should be excluded prior to
label, Bayesian randomised controlled trial to investigate the immunosuppression.
effectiveness of CYC and Mycophenolate mofetil (MMF)
for remission induction in childhood PAN. The study was Vasculitis Secondary to Connective Tissue Disease
conducted in 11 children with PAN and confirmed that MMF such as Lupus
was non-inferior in remission induction at 6 mo and for a
follow-up of 18 mo [34]. The last SHARE guidelines pre- It is seen in a child with severe disease presentation, but
ceded this publication and recommended induction with IV with other features such as skin rash, arthritis, serositis and
CYC and maintenance with AZA or MMF. For the relapsed supporting lupus serology.
or resistant patient biologic therapy with TNF-alpha, IL-6
blockade or B cell depletion could be considered [19]. When
streptococcal infection is implicated penicillin may be effec- Auto Inflammatory Diseases
tive [13].
These are diseases of the innate immune system that present
with episodic local or systemic inflammation. There are a
Takayasu Arteritis (TA) few that have vasculitis as one of the main clinical manifes-
tations. Important amongst these are:
This is a challenging condition to manage and needs a team
assessment of the patients often involving cardiology and Deficiency of ADA2 DADA2 often presents as a PAN-like
vascular surgeons. Common induction regimens involve disease early on in life. The hallmarks of this condition are
CYC or MTX, combined with high dose glucocorticoid ther- persistent livedoid rash, early CNS involvement, positive
apy. Use of TNF-blockade, anti-IL-6 therapy and/or RTX for family history, non-response to conventional therapy and
severe disease is reported. Maintenance agents can include finally association of hematological abnormalities including
MTX, AZA or MMF. Accurate blood pressure measurement common variable immunodeficiency.
is a challenge and warrants 4 limb BP readings [19]. Sur-
gical procedures specifically for uncontrolled hypertension SAVI (STING Associated Vasculopathy of Infancy) It is an
or critical organ ischemia are challenging in children. As interferonpathy where patients present with severe skin
surgical correction needs revision with growth of the pedi- lesions including ulceration necrosis and amputation of dis-
atric patient, the preferred technique is endovascular balloon tal digits, ear cartilage and tip of the nose. The clinical hall-
angioplasty with stent placement [14]. mark of these patients is early onset interstitial lung disease.

Otulipenia The patient presents with painful subcutaneous

General Measures nodules which are associated with vasculitis.

During immunomodulatory treatment for management of Haplo Insufficiency of A20 It presents with clinical features
severe systemic vasculitis (AAV, PAN, TA), other agents very similar to Behcet's disease.
that should be considered are: antiplatelet agents, antibi-
otic prophylaxis (specifically against Pneumocystis jiroveci Behcet's Disease It is a polygenic auto inflammatory disease
pneumonia, osteoporosis prophylaxis and gastric protection. with features of oral and genital ulcers and unique vasculitis
CYC should be administered with MESNA unless there is affecting both arteries and veins of all sizes [35].
hypersensitivity to the latter. Sperm cryopreservation may
be considered for all post-pubertal males prior to receiv- Immunodeficiencies TAP1 deficiency patients can present
ing CYC [19]. There is no formal recommendation for with granulomatous lesions of the skin, sinuses and lungs.
gonadotropin-releasing hormone (GnRH) analogues to halt This can look like GPA and it worsens with immunosup-
ovulation in adolescent girls. pression [36].

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 Indian Journal of Pediatrics

Conclusions 12. Hetland LE, Susrud KS, Lindahl KH, Bygum A. Henoch-
Schönlein purpura: a literature review. Acta Derm Venereol.
2017;97:1160–6.
The common childhood vasculitidis are IgA vasculitis and 13. Eleftheriou D, Brogan PA. Vasculitis in children. Paediatr Child
KD. Recognition of incomplete KD can be a challenge. Health. 2018;28:P57-63.
Prolonged pyrexia associated with arthralgia and myalgia 14. Di Santo M, Stelmaszewski EV, Villa A. Takayasu arteritis in
paediatrics. Cardiol Young. 2018;28:354–61.
are non-specific features of several systemic vasculitis. 15. Ozen S, Sag E. Childhood vasculitis. Rheumatology (Oxford).
Consideration of vasculitis in differential diagnosis, pattern 2020;59:iii95–100.
recognition and appropriate tests guide one to the correct 16. Bilginer Y, Ozen S. Polyarteritis nodosa. Curr Opin Pediatr.
diagnosis. Exclusion of mimics are important before immu- 2022;34:229–33.
17. Ozen S, Marks SD, Brogan P, et al. European consensus-based
nosuppression. The SHARE guidelines are a good resource recommendations for diagnosis and treatment of immunoglobu-
to guide specific therapies for these rare but challenging lin A vasculitis-the SHARE initiative. Rheumatology (Oxford).
group of diseases. 2019;58:1607–16.
18. Jelusic M, Sestan M, Giani T, Cimaz R. New insights and chal-
lenges associated with IgA vasculitis and IgA vasculitis with
nephritis-is it time to change the paradigm of the most common
Declarations systemic vasculitis in childhood? Front Pediatr. 2022;10:853724.
19. de Graeff N, Groot N, Brogan P, et al. European consensus-based
Conflict of Interest None.
recommendations for the diagnosis and treatment of rare paediat-
ric vasculitides - the SHARE initiative. Rheumatology (Oxford).
Guarantor SS will act as guarantor for this manuscript.
2019;58:656–71.
20. Iudici M, Pagnoux C, Quartier P, et al. Childhood- versus adult-
onset ANCA-associated vasculitides: a nested, matched case-
control study from the French Vasculitis Study Group Registry.
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