S 24 PEDIATRIC RHEUMATOLOGY

24
PEDIATRIC RHEUMATOLOGY
M A R C H 2 0 1 0 • R H E U M AT O L O G Y N E W S
ASK THE EXPERT
Pediatric Vasculitis Poses Diagnostic Challenges
S
RHEUMATOLOGY NEWS: What are the
most frequent systemic vasculitides that
affect children?
Dr. Gedalia: There are two systemic
vasculitides that affect children most frequently: Henoch-Schönlein purpura, a
nongranulomatous small-vessel vasculitis, and Kawasaki disease, a mediumsize vessel vasculitis. These are self-limiting diseases. Children do develop such
chronic vasculitides as giant cell arteritis,
Wegener’s granulomatosis, microscopic
polyangiitis, Churg-Strauss syndrome,
polyarteritis nodosa, and Takayasu’s arteritis, but these occur much more rarely
than they do in adults.
RN: What are the most important diagnostic criteria that distinguish these conditions from other inflammatory disorders that might mimic them?
Dr. Gedalia: As its name suggests, vasculitis is a condition associated with inflammation of the blood vessels. This
arteries and is now the leading cause of Dr. Gedalia: Most children with
acquired heart disease in children in the Henoch-Schönlein purpura do well with
United States. The disease affects mostly supportive care and observation because
infants and young children, and rarely this is a self-limiting disease, from which
teenagers. It is characterized by fever, most patients recover. Some patients, espolymorphic rash, conjunctivitis, mu- pecially those with joint involvement
cositis, changes in
and painful inflamthe hands and feet,
matory soft-tissue
The criteria classify the
and unilateral cerviedema,
require
common childhood
cal lymphadenopaanalgesics and nonthy. However, the
steroidal anti-invasculitides by vessel size
hallmark of this disflammatory drugs.
and histopathology, which is
ease is the coronary
The arthritis assoartery abnormalities
ciated
with
expected to be useful to
(mainly aneurysms)
Henoch-Schönlein
rheumatologists in practice.
that are developed
purpura is tranin approximately
sient and not asso20%-25% of untreated patients. A patient ciated with any permanent joint disease;
with Kawasaki disease has to present with therefore, the use of steroids is reserved
fever lasting 5 or more days without oth- for patients with severe gastrointestinal
er more reasonable explanation and, ac- involvement.
cording to the classification system, must
Standard treatment for Kawasaki disfulfill at least four of the following five cri- ease includes intravenous immunoglobteria:
ulin treatment (IVIG). In IVIG-resistant
Kawasaki disease, treatment with the
씰 Have bilateral conjunctival injection.
anti–tumor necrosis factor–alpha agent
씰 Demonstrate at least one mucous
membrane change, such as injected or infliximab has been proved effective in
fissured lips, injected pharynx, or straw- early studies.
©C USTOM M EDICAL S TOCK P HOTO, A LL R IGHTS R ESERVED
ystemic vasculitis is a multisystem process can occur as a primary disorder
disease that without proper man- or as secondary to the underlying disagement can be associated with sig- eases. In both situations, vasculitis can
nificant morbidity and mortality. Diag- lead to vascular tissue injury such as vasnosing systemic vasculitis in children can cular leak, aneurysm formation, stenosis,
be especially challenging, given the wide occlusion, rupture, and necrosis that may
impact the involved organ.
variation in the type and loThe above disorders have
cation of vessel involvement.
unique criteria that are helpThe degree of inflammation
ful to distinguish them from
and the resultant vessel wall
other inflammatory diseases
damage may also confound
and establish the diagnosis.
diagnosis. Moreover, the earIn the case of Henochly findings in affected chilSchönlein purpura, the condren—such as fever, skin ledition is a multisystem IgAsions, and inflammation—
mediated vasculitis with
are neither specific nor senself-limited course affecting
sitive for the condition, and
ABRAHAM
the skin, joints, gastrointestias such can mimic multiple
G E D A L I A , M . D.
nal tract, and kidneys. Other
other childhood infectious
organs such as lungs, brain,
diseases.
Historically, the lack of an acceptable and genitourinary system may be inclassification system for childhood vas- volved. Based on the revised classificaculitis has led rheumatologists to rely on tion system, a patient with Henochadult classification criteria that are sub- Schönlein purpura has to fulfill at least
optimal for use in children. In 2006, an two of the following four criteria, as deinternational consensus group of the fined by the American College of
European League Against Rheumatism Rheumatology:
(EULAR) and the Pediatric Rheumatol- 씰 Exhibit palpable purpura, not related
ogy European Society (PRES) proposed
a pediatric vasculitis classification that
would bring the existing criteria into
line with clinical practice (Ann. Rheum.
Dis. 2006;65:936-41).
The proposed criteria, which are currently being validated, classify the common childhood vasculitides by vessel
size and histopathology. This approach is
expected to be useful to rheumatologists
and pediatricians in practice, as well as to
those who are involved in epidemiologic studies and clinical trials of pediatric
vasculitis, according to Dr. Abraham
Gedalia.
In this month’s column, Dr. Gedalia
discusses the clinical features, management, and prognosis of the common
childhood vasculitides, and offers insight
into the most important classification revisions.
In this color-enhanced angiogram of a child with Kawasaki disease, note the
aneurysms (red) of the weakened small arteries that come off the aorta.
to thrombocytopenia.
Be age 20 years or younger at disease
onset.
씰 Present with abdominal pain, usually
with gastrointestinal bleeding.
씰 Have biopsy-proven granulocytes in
the vessel wall (arterioles or venules).
Detection of the classic, palpable, purpuric rash and its distribution in children
improves the accuracy of the diagnosis
of Henoch-Schönlein purpura. Skin
biopsy is rarely needed, except in very
young children (aged 2 years and
younger) in whom the rash is not classically distributed.
Kawasaki disease is an acute, self-limited, systemic vasculitis of unknown origin
that predominantly involves the coronary
씰
berry tongue.
씰 Exhibit at least one extremity change,
such as erythema of the palms or soles,
edema of the hands or feet, or generalized periungual desquamation.
씰 Present with a polymorphous rash.
씰 Present with cervical lymphadenopathy, with at least one lymph node measuring 1.5 cm or greater in diameter.
Patients who have coronary artery involvement (detected on echocardiography) in addition to fever may present
with fewer than four of these five criteria; the exact number is currently being
determined.
RN: What are the primary therapies for
vasculitis in children?
RN: What is the prognosis for children
with these conditions?
Dr. Gedalia: The prognosis is good in
children with Henoch-Schönlein purpura. However, the long-term outcome of
Henoch-Schönlein purpura is complicated when patients develop renal involvement. Among patients with
Kawasaki disease, up to 2% die from
coronary abnormalities, and 20%-25% of
untreated patients develop coronary
artery abnormalities or ectasia. In the absence of detectable coronary artery abnormalities, data from long-term followup studies conducted 10-20 years after
the onset of Kawasaki disease have
shown that patients’ morbidity and mortality are similar to those in the normal
pediatric population.
RN: What are some of the most pressing
short- and long-term challenges in the
management of vasculitis in children?
Dr. Gedalia: The short-term challenges
are to find the most efficacious and safe
treatments of the serious manifestations,
such as renal involvement in HenochSchönlein purpura and coronary involvement in Kawasaki disease. As affected
children are surviving longer—often
thanks to the increasing use of biologic
agents as second-line treatments after the
failure of standard first-line therapies—
managing the long-term cardiovascular
morbidity in Kawasaki disease patients is
a particular challenge.
—Diana Mahoney
DR. GEDALIA is professor of pediatrics and
head of the division of pediatric
rheumatology at Louisiana State
University Health Sciences Center and
Children’s Hospital in New Orleans. He
reported having no potential conflicts of
interest.
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