Pediatrics Tea

mWor
43
K
7
Common Pediatric
Rheumatic Diseases
Done by:
Elham Alami Ghadah Alqarni
Kawasaki disease topic
includes also dr. Mohammed
Revised by: alghamdi slides and notes
from acquired heart disease
Aseel Badukhon lecture to avoid repeating
topics!

Team Leader:
Aseel Badukhon

Notes Previous Notes Book Important!

 Approach to Arthritis

Arthritis(how to know the joint is inflamed): swelling within a joint, or limitation

in the range of joint movement with joint pain or tenderness, which persists for at
least 6 weeks, is observed by a physician, and is not due to primarily mechanical
disorders or other identifiable causes.

Differential diagnosis of arthritis:

Reactive: 1. Post-enteric. 1. Juvenile idiopathic arthritis.

2. Rheumatic fever. Inflammatory: 2. Inflammatory bowel disease.
3. Post-streptococcal. 3. Sarcoidosis.

1. Kawasaki disease.
2. Behcet’s disease. Malignancy:
Systemic: 3. Henoch-Schonlein purpura. 1. Leukemia.
4. Serum sickness. 2. Neuroblastoma.
5. Systemic lupus erythematosus. 3. Malignant bone tumors.
6. Dermatomyositis.

1. Septic: Single joint , fever ,

Infection: high WBC, look sick
2. Osteomyelitis. Benign bone tumors
3. Viral.
4. Bacterial sacroiliitis..

Trauma

-As part of infectious causes don’t

forget about brucellosis and TB ( ask
in Hx ; contact w/ animal ,sick ppl ….)
-Undifferentiated if it doesn’t fit any
category

A nice article about Fever evaluation in pediatrics:

https://www.aafp.org/afp/2013/0215/afp20130215p254.pdf
 Juvenile Idiopathic Arthritis

Overview:

Old names It is a group of disorders characterized by

chronic arthritis (6 wks in duration to diagnose JIA)
Juvenile
It is the most common chronic rheumatic
Rheumatoid
illness in children
Arthritis
It is a clinical diagnosis made in a child less than 16
Juvenile years of age with arthritis
Chronic
The incidence of JIA ranges from 1 to
Arthritis
22 per 100,000.

Pathophysiology:

➔ The pathogenesis of JIA is not understood well

➔ Substantial evidence suggests that JIA is an autoimmune process.
➔ Can be triggered by infections or environmental factors
➔ They usually have other Autoimmune diseases or family history of the disease

Classification:

International League of Associations for Rheumatology (ILAR)

1- Oligoarticular JIA.
2- Polyarticular rheumatoid factor positive JIA.
3- Polyarticular rheumatoid factor negative JIA.
4- Systemic JIA.
5- Psoriatic JIA.
6- Enthesitis related arthritis(ERA).
7- Undifferentiated.

Oligoarticular (few) JIA:

◦ <5 joints during the first 6 months of disease. Large joint : ankle or knees
◦ At high risk for developing uveitis especially ANA-positive girls “<7yrs at the onset of illness”
◦ Persistent & extended oligoarticular JIA.

Q: child presented w/ 3 joint involvement

in the first 6 months , then after 6 months
he came w/ more joint involvement (6-7)
what’s the type?
Answer: Extended Oligoarticular JIA
 Juvenile Idiopathic Arthritis

Oligoarticular (few) JIA:

● Every single child with oligo JIA should do eye examination. We don’t expect them to
have symptoms, they may present with asymptomatic uveitis (anterior uveitis)
Ocular Manifestations

Irregular pupil due to

the eye inflammation
“uveitis”.
High risk: female &
ANA +ve
Need to be evaluated
by ophtha every 10
months
Normal rounded pupil This is permanent change
called synechia

Polyarticular JIA:

● RF-negative disease (20% to 30% of JIA patients)

● RF-positive disease (5% to 10% of JIA patients).
● Both types affect girls more frequently than boys.
● RF-negative patients often develop polyarthritis in early childhood
● RF-positive disease is like RA in adults “erosion and destruction of joints” . The child may
cotinue to have Sx even as an adult

Systemic onset juvenile idiopathic arthritis:

● Undergo extensive investigations to role out other ddx like: infection or cancer like leukemia ,
some may need bone marrow aspiration to r/o malignancies. They may present like leukemia!
● No specific age and gender.
● At onset, extra-articular manifestations including rash, fever, lymphadenopathy,
hepatosplenomegaly, and serositis predominate.

- The classic rash - Or salmon like rash that

is evanescent . disappears within few hours
comes and go - If you are on call and a
- Stress/ fever or a nurse calls you for a pt w/
warm bath may possible systemic JIA you
exacerbate the may not see it but it can
rash. come again few hours later

- Quotidian fever ( 1 or 2
spikes of fever )
- This chart has 1 spike in
day 1 then it’s back to
normal / subnormal .In
the next day there is a
2nd spike

[
[Quotidian fever, skin rash, and arthritis this is in favor of systemic JIA
From a paper: In patients with non-typical JIA and monoarthritis accompanying with intense
night pain, highly elevated acute phase reactants, and bone edema in MRI, we believe that
bone marrow aspiration should be performed before treatment even if ANA positivity is
present.
 Juvenile Idiopathic Arthritis

Enthesitis-related arthritis:

● It is the inflammation of the insertion site of a tendon ,ligament, fascia into bone
● Most common in boys older than 8 years of age.
● It has a strong genetic predisposition.
● The hallmarks of the disease are:

And eventual loss of

Pain Stiffness
mobility of the back

● Like spondyloarthropathy in adults sacroiliac joint involvement, may end up w/ bamboo sign
● Some may present w/ pain in ASIS (anterior superior iliac spine) or below the knee.
● They may have acute eye inflammation not chronic like oligoarticular JIA
● They have genetic predisposition: HLA-B27 positivity
● Strong family history of inflammatory back pain

Bamboo sign!

Psoriatic arthritis:

● Psoriasis + arthritis , but kids may not present w/ typical features of psoriatic arthritis
● A peak age of onset in mid childhood
● Extra-articular manifestations include: rash, nail changes (including pitting, onycholysis) and
uveitis.+ family history of psoriasis

Pitting nail Onycholysis

Inflammation under the
nail bed

Dactylitis
Dactylitis inflammation of
the whole digit
 Juvenile Idiopathic Arthritis

Investigations: Dx is mainly clinical

Laboratory Radiology

- No specific lab. can confirm the diagnosis Plain x-ray

- Lab. can be used to:
1. – Provide evidence of inflammation. Early radiological changes:
2. – Support the clinical diagnosis. ● Periosteal soft tissue
3. – Monitor treatment toxicity. Monitor liver enzymes and CBC
swelling
Limited joint disease:
● – Mild anemia.
● –Widening of the joint
Moderate –extensive arthritis: space
● – Normocystic hypochromic anemia or severe anemia. ● –Juxta articular
● – Iron deficiency anemia. osteoporosis
● – High WBC count. ● Usually normal X-ray in
● – High platelets. initial presentation

ESR (erythrocyte sedimentation rate): Later changes:

● –Useful but not totally reliable measure of active disease. Oligoarticular particularly may ● – Joint space narrowing
be normal ● – Erosions.
● Helpful in monitoring the therapeutic efficacy of the medications. ● – Subluxation.
CRP (c –reactive protein ):
● – Ankylosis.
● –More reliable monitor of inflammation response.
● – Fracture. Specially
vertebra
Rheumatoid factor: help in differentiating RF +ve from -ve
● – IgM anti IgG.
● – RF positive in:
○ – Later childhood polyarthritis.
○ – Subcutaneous nodules.
○ – Articular erosions.
ANA (antinuclear antibody):
● –More frequent in young girls with oligo JIA
● – Less frequent in older boys with systemic arthritis.

E: When the chronic inflammation is not treated ,there

will be Area of erosion (bone eaten up by chronic )
osteopenia and osteoporosis and fracture

A: Cervical vertebra you expect cervical spine be

separated , but it will be ankylosed due to aggressive
or chronic inflammation

- Joint aspiration not done routinely for JIA

unless we want to r/o other ddx(send for
culture and cell count). But if the pt has oligo
JIA w/ knee swelling we may aspirate the joint
(therapeutic aspiration) and then inject it w/
steroids (intra-articular).
- Inflammatory is what we expect in JIA.

➔ In oligo JIA you might have completely normal labs

➔ In systemic JIA (high WBC , low Hgb, High plts
initially but when MAS developes (pancytopenia)
 Juvenile Idiopathic Arthritis

Management:

Multidisciplinary approach Medications

NSAIDS Naproxen or ibuprofen

The aims of management of JIA
Controlling pain Methotre- Specially, for polyarticular
Controlling inflammation xate JIA
Preserving function
Promoting normal growth -Systemic (systemic JIA)
Steroids -Intraarticular
Promoting overall
development
Manage systemic Relatively new (we add it when it is
complication Biologics NOT responding to treatment. TNF antagonist
like humera)

You don’t have to know which medication

is for which class.

For oligo JIA we start w/ NSAIDs ; if failed → steroids ( intra-articular)

Complication:

M A S EULAR/ACR CLASSIFICATION CRITERIA FOR MAS

A Serious complication of systemic JIA is macrophages activation syndrome

(MAS) =Hemophagocytic lymphohistiocytosis (HLH)
Classification of macrophage activation syndrome in systemic juvenile
idiopathic arthritis.

A febrile patient with known or suspected systemic juvenile idiopathic arthritis

is classified as having macrophage activation syndrome if the following
criteria are met:

Ferritin >684 ng/ml and any 2 of the following:

Platelet count ≤ 181 x 109/liter

Aspartate aminotransferase >48
unitsiter

Triglycerides >156 mg/dl

Fibrinogen ≤ 360 mg/dl

Laboratory abnormalities should not be otherwise explained by the patient's condition, such as
concomitant immune-mediated thrombocytopenia, infectious hepatitis, visceral leishmaniasis, or
familial hyperlipidemia.
Persistent fever (not quotidian), pancytopenia , high liver enzymes and they are very sick.
It can be fatal in 40% of pt specially if not detected early
 Systemic Lupus Erythematosus

● SLE is a multisystem autoimmune disease with a great variability in disease

presentation and course. Same as adult, but more complication due to the duration
of disease and medication S/E
● The diagnosis of SLE is based on the clinical and laboratory features consistent
with this illness.
● The etiology of systemic lupus erythematosus (SLE) remains unknown and it is
multifactorial:

Genetics factors Hormonal factors Immune abnormalities Environmental factors

There is a high
More in females (4:1) Viruses
concordance rate (14 to
57 percent) of SLE in SLE is primarily a disease
The use of Ultraviolet (UV) light
monozygotic twins. with abnormalities in
estrogen-containing should avoid exposure
immune regulation.
contraceptive agents is
Children of mothers with Immune deficiency is risk
associated with a 50 Allergies to medications
lupus may have a factor
percent increase in risk of anti-TB (isoniazid),
positive test for
developing SLE anti-HTN ( hydralazine)
antinuclear antibodies

Criteria:

Old Criteria
SLICC (Systemic Lupus International Collaborating Clinics) Classification Criteria for Systemic Lupus
Erythematosus requirements:

➔ ≥ 4 criteria (at least 1 clinical and 1 laboratory criteria)

➔ OR biopsy-proven lupus nephritis with positive ANA or Anti-DNA

Clinical Criteria Immunological Criteria

1.Acute Cutaneous Lupus: malar rash. Sparing nasolabial folds

2.Subacute cutaneous lupus
3.Chronic Cutaneous Lupus: discoid rash. 1.ANA level above laboratory reference
4.Oral Ulcers (painless) OR Nasal Ulcers. range. Has to be +ve for Dx of SLE
5.Non-scarring alopecia. Ask about hair loss - can be early presentation of SLE 2.Anti-dsDNA antibody level above
6.Arthritis involving 2 or more joints (non-erosive) laboratory reference range. specific, renal
7.Serositis (major): pleural effusions, pericardial effusion, and pericarditis by involvement
electrocardiography in the absence of other causes, such as infection, uremia. 3.Anti-Smith. Also specific
Peritonitis (abdominal pain) 4.Antiphospholipid antibody. They are
8.Renal: urine protein–to-creatinine ratio (or 24-hour urine protein) prone to secondary (APS) . ask for
representing 500 mg protein/24 hours OR red blood cell casts. Imp poor anticardiolipin, beta-2 glycoprotein I
prognostic factor. Even with mild renal involvement sometimes we need (β2GPI), and lupus anticoagulant. Don’t
renal bx ; why? We expect worse finding in the biopsy compared to the renal bother yourself about names just have an
symptoms. idea
9.Neurologic: seizures, psychosis, mononeuritis multiplex (in the absence of 5.Low complement (C3, C4, or CH50). If
other known causes), myelitis, peripheral or cranial neuropathy (in the absence low indicates flare-up. Renal involvement
of other known causes) and acute confusional state (in the absence of other (low C3)
causes). Ask about school performance, renal and CNS involvement affect prognosis 6.Direct Coombs’ test (in the absence of
10.Hemolytic anemia (sudden drop of Hb, jaundice and dark urine) hemolytic anemia)
11.Leukopenia (<4000/mm3) OR Lymphopenia (<1000/mm3).
12.Thrombocytopenia ( <100,000/mm3) at least once in the absence of other Antihistone Ab → drug- induced lupus
known causes. Pt w/ isolated thrombocytopenia should be followed-up as they
may develop lupus in the future.
 Systemic Lupus Erythematosus

Criteria:

Old Criteria

Subacute Non-scarring
Malar rash Cutaneous Lupus
Discoid rash Oral ulcer alopecia

Updated Criteria

It must reach ≥ 10 points

Rules:
-1 from each category
- If you have 2 from the same
category , pick the highest
weight
- Last one in renal = 10 If
present alone w/ +ve ANA we
can diagnose the pt w/ SLE

It’s a must to have ANA

Don’t remember all the numbers

but I want you to have an idea
 Systemic Lupus Erythematosus

Lupus Nephritis (Notes):

Lupus nephritis

- It has 6 classes
- Class 3,4 - significant renal
involvement ( should go for
aggressive Tx like
cyclophosphamide,MMF or
rituximab )
- Class 5 membranous nephritis (
proteinuria )
- Class 6 sclerosed kidney ( should
go for dialysis)

Treatment :

General

Team approach:
○ – Counseling
○ – Education
○ – Appropriate nutrition
○ – Use of sun protection
○ – Immunization
○ – Prompt management of infection

Nonsteroidal Anti-inflammatory

Hydroxychloroquine Specially in skin and hematology

problems
Glucocorticoids The Main treatment specially in the
beginning IV in cns, renal and active

Immunosuppressives In bad cns or renal

“Red riding hood will help you to remember SLE. Lupus means wolf and
erythematosus means red, and in the story the villain is the wolf and the prey is the
little girl wearing red hood. SLE = commonly affects woman!”
 Henoch-Schonlein Purpura

● HSP is the most common pediatric vasculitis. Involves small arteries

● Classically presents with the triad of:

Non-thrombocytopenic Colicky abdominal Arthritis

palpable purpura pain

Differential diagnoses of Vasculitis (according to vessel size):

Pathophysiology:

Immunoglobulin A (IgA) immune complexes deposition. This is what you expect to see in skin biopsy

The major cause of morbidity is renal involvement.

3-15 years.if no renal involvement = benign disease
A wide variety of infections may trigger HSP. It’s not a must but it could happen

- Skin involvement (100%) in HSP may begin as urticaria, but in most cases it progresses to
dramatic purple, non-blanching lesions . Mainly lower limb vasculitis.
- Gastrointestinal involvement (75% )ranges from colicky abdominal pain to profuse bleeding,
intussusception.
- The arthritis of HSP (50% ) is usually transient, and it does not cause chronic joint changes.
- Renal Disease: the most serious sequelae of Henoch- Schonlein purpura is renal involvement. This
complication occurs in about 25 percent of children

- HSP in ER don’t forget the possibility

of intussusception so we do radiological
investigation to rule it out. You may end
up with gut gangrene .
- Most common site of intussusception =
ileoileal . why? GI vasculitis
 Henoch-Schonlein Purpura

Diagnosis: Based on the criteria

Purpura (mandatory criterion):

1 Abdominal pain
3 Arthritis or arthralgia

Histopathology; vasculitis with

2 predominant IgA deposit. 4 Renal involvement

You can’t diagnose Skin biopsy? If not

HSP w/o skin rash! sure about the Dx

Picture is a histopathology of HSP (EXTRA)

Treatment:

- Therapy of HSP is primarily supportive, aiming for symptomatic relief of arthritis and abdominal pain.
- Use of steroids in children who do not respond to NSAIDs (we usually don’t start w/ it b.c of GI upset
and renal problems - used if needed ) or in those thought to be at highest risk of developing renal
compromise continues to be controversial.
- Indications for steroids from the beginning :

1- renal involvement 2- CNS involvement 3- sever GI bleeding

Adult HSP considered as

paraneoplastic syndrome (consider
the possibility of malignancy or
hepatitis)!!
 Juvenile Dermatomyositis
Triad of : childhood- skin - muscle involvements

Idiopathic inflammatory myopathies (IIMs), collectively known as myositis, are heterogeneous disorders
characterized by muscle weakness and muscle inflammation.
The most common subgroups in children, juvenile DM (JDM).
Incidence:
- In population-based studies, JDM has a reported annual incidence that ranges from two to four cases per
one million children. Not common
- The peak incidence is from 5 to 10 years of age.

Etiology & Pathogenesis:

● Cause unknown.
● Likely autoimmune angiopathy.
● Environmental and genetic factors implicated.
○ A history of infection prior to onset is common, 65- 70% of patients have a history of a significant
infection during the three months prior to first onset of symptoms.
○ Proposed triggers include various infectious agents, vaccines, medications, UV light.
● Cellular and humoral immunity implicated.
● Complement-mediated injury important.
● Innate immune response: type l interferons and dendritic cells.
Dermatomyositis (other organ involvement):

1. Gastrointestinal vasculitis: gut wall perforation.

2. Arthritis: common but usually early and mild, non- erosive.
3. Cardiac: inflammation, fibrosis, conduction defects.
4. Renal: glomerular hypercellularity.
5. Pulmonary: fibrosis, pneumothorax. sometimes due to the weakness they might face respiratory problems
and need ICU and ventilation .Rash + lung involvement (ILD) → anti-MDA5
6. Central nervous system: behavior changes, seizures.
7. Alopecia.
8. Eyes: exudative vasculitis of retina.
9. Derm: calcinosis, subcutaneous nodules, ulcerations.
10. Lipodystrophy. One of the major involvement -> loss of subcutaneous fat

Don’t remember the details but I want to tell you that muscle weakness
Differential Diagnosis: can present in other disease not only JDM
 Criteria:

Bonanza and Peter diagnostic criteria: Old criteria but the popular

Proximal and symmetrical muscle weakness of the pelvic and scapular girdle, anterior flexors of the neck, progressing for
A weeks to months, with or without dysphagia or involvement of respiratory muscles.

Elevation of the serum levels of skeletal muscle enzymes: creatine phosphokinase, aspartate aminotransferase, lactate
B dehydrogenase, and aldolase. AST

Electromyography characteristic of myopathy (short and small motor units, fibrillations, positive pointy waves, insertional
C irritability and repetitive high-frequency firing).

Muscle biopsy showing necrosis, phagocytosis, regeneration, perifascicular atrophy, perivascular inflammatory exudate.
D Muscle biopsy : we don’t usually do it. Replaced by MRI

Typical cutaneous changes:

E ● Heliotrope with periorbital edema and violaceous erythema;
● Gottron's sign: vasculitis in the elbow, metacarpophalangeal, and proximal interphalangeal joints.

Criteria for DM

Definitive Three criteria (A, B, C or D) + E

Probable Two criteria (A, B, C or D) + E

Possible One criteria (A, B, C or D) + E

Updated criteria

- Patients with pathognomonic skin rashes (heliotrope rash,

Gottron’s papules, and/or Gottron’s sign) of JDM or DM are
accurately classified with the EULAR/ACR classification criteria
without including muscle biopsy data.
- For patients without these skin manifestations, muscle biopsy is
recommended.
- For DM patients without muscle involvement, a skin biopsy is
recommended.
- The EULAR/ACR classification criteria provide a score and a
corresponding probability of having IIM.
- A probable IIM:
● For a total score of ≥5.5 and ≤5.7) for the criteria not
including muscle biopsy data, and a score ≥6.7 and
≤7.6 when including muscle biopsies.
- Definite IIM :
● For a total aggregate score of 7.5 or more without
muscle biopsy and 8.7 with muscle biopsy.
Skipped!

Don’t bother yourself w/ this criteria ; pt w/ typical skin

manifestation of gottron's papule or heliotrope is in favor of
dermatomyositis
 Juvenile Dermatomyositis

Clinical Presentations (&Notes):

F: calcinosis of
the skin . hard
material or
they ooze
calcium like
pus
Kids who are
diagnosed late
or treated late
are prone to
have it
Gower’s sign : proximal
Calcification of muscle weakness ( can’t
subcutaneous stand w/o hand/chair
can cause support )
disfiguring
oozing

Heliotrope
Nail bed changes : capillary
rash:
loop changes a part of
pathognomic .
Vasculitis ( hemorrhage due
Part of
to the inflammation of the
vasculitis .
vessels and the area w/o
(ask the child
blood vessels is called drop
to close his
out area)
eyes )

Malar rash ( note: it

Shawl sign : skin involvement in doesn't spare the
Gottron’s papule:
exposed area (more lung nasolabial fold)
Pathognomic
involvement?) Ddx of malar rash :
lupus or JDM
 Juvenile Dermatomyositis

Investigations:

● Muscle enzymes—including creatinine phosphokinase (CPK), LDH, AST (SGOT), ALT

(SGPT), adolase (if available). Elevated
● Full blood count and blood film.
● ESR and CRP.
● Myositis-specific and myositis-associated antibodies. Helpful to know prognosis
-anti-MDA5 bad prognosis
● Renal function and liver function tests.
● Infection screen (for differential diagnosis).
● Investigations for alternative systemic causes of myopathy including endocrine disorders
(especially thyroid function), electrolyte disturbances, vitamin D deficiency.
● Further tests for metabolic/mitochondrial myopathies (especially in the absence of
rash/atypical presentation)
● Urine dipstick (with further evaluation if positive for protein)
● Nailfold capillaroscopy
● Echocardiogram and ECG
● Pulmonary function tests (chest X-ray and HRCT if concern)
● MRI of muscles.
● EMG (particularly if suspicion of neuropathy/disorder of neuromuscular junction)
● Muscle biopsy (especially in the absence of rash/atypical presentation)
● MRI brain if neurological involvement suspected.

MRI shows Bright area which is an inflammation of the

muscle(hint for proximal muscle weakness) we
normally see muscle dark. It involves patchy muscles
and bilateral

Treatment: Combine both

● Steroid. Orally
● Methotrexate (subcutaneous). Once per week , small dose
● Biologics for non responsive cases
 Kawasaki Disease

Overview:

● The other name is mucocutaneous lymph node syndrome

● Kawasaki disease mainly affects children of 6 months to 4 or 5 years of age, with a peak at the
end of the first year of life.
● It is the commonest cause of acquired heart disease in children ( in north america) ( in SA is
Acute Rheumatic Fever):

Cardiac involvement
Dilation and aneurysm formation, thrombus formation, fibrosis and stenosis, myocardial
infarction and it may cause myocarditis and endocarditis

● Systemic inflammatory process (Vasculitis: medium size , mainly coronary arteries) with no
known etiology
● Maybe infectious etiology
● More common in children of Japanese and, to a lesser extent, Black-Caribbean ethnicity, than in
Caucasians

Diagnosis:

There is no diagnostic test; instead, the diagnosis is made based on clinical findings alone:

Fever > 5 days

At least 4 of the following:

1 Changes in the extremities:

● Erythema and edema of hands and feet (acute phase)
● Subsequent peeling of distal ends of digits (subacute phase)

2 Polymorphous rash. Any type of skin rash

except vesicles and bullae. Check the diaper area

3 Non-purulent bilateral conjunctivitis

4 Mucosal changes:
● Strawberry tongue
● Red, cracked lips and/or erythema of oral and pharyngeal mucosa

5 Cervical lymph node (>= 1.5 cm in diameter)

-It is NOT a must to have all the clinical features in the

same time
-If you don’t ask about red eyes the parents may forget
about it (it disappears quickly)
-Other Ddx of strawberry tongue : scarlet fever
-Peeling of the skin usually not in the beginning of
disease
-Other medium size arteries: axillary, femoral, iliac and
renal arteries
 Kawasaki Disease

Diagnosis & DDX:

Differential diagnoses
● Scarlet fever
● EBV infection
● Adenovirus infection
Desquamation ● Staphylococcal scalded skin
syndrome
● Drug reactions
● Stevens-Johnson syndrome

Young infants may have ‘incomplete’ symptoms or diseases, in which not all the cardinal
features are present:

For incomplete symptoms, there

should remain a high clinical
suspicion, particularly for
children less than 6 months of
age with prolonged fever and
these children are more likely to
develop coronary artery
aneurysms which affected
children within the first 6 weeks.
It should be treated as complete
KD

Investigations:
● Affected children have high inflammatory markers (C-reactive protein, erythrocyte
sedimentation rate, white cell count), with a platelet count that rises typically in
the second week of the illness.
● CBC: Neutropenia, leukocytosis (50%) and nonspecific anemia
● Elevated liver transaminases (40%), low serum albumin level
● Sterile pyuria (33%) , aseptic meningitis (up to 50%)
● Echocardiography should be performed when the diagnosis is first suspected, and
at 4–6 weeks to identify coronary artery aneurysms; and it may show a pericardial
effusion, myocardial disease (poor contractility), endocardial disease (valve
regurgitation), or coronary disease with aneurysm formation, which can be giant
(>/= 10 or >8 mm in diameter).
○ If the coronary arteries are abnormal, angiography or magnetic resonance
imaging (MRI) will be required.
 Kawasaki Disease

Management:

Risk scores for coronary aneurysm

1. WBC > 12 000

2. Platelet < 350 000
3. CRP > 3+
4. Hct < 3.5
5. Albumin < 3.5
6. Age </= 12 months
7. Male sex

Treatment:

● Intravenous immunoglobulin (IVIG), ideally given within the first 10 days, to lower the
risk of coronary artery aneurysms. From 25% to less than 5%
● Aspirin to reduce the risk of thrombosis. due to dilation of the coronary even if there is no dilation
start aspirin then re-evaluate after 6 wks with another echo If normal → stop aspirin “coronary changes
might develop after this period’ , decrease
aspirin once afebrile
● Children with coronary artery aneurysms require long- term low-dose aspirin and
lifelong follow-up.
● Give another anticoagulant if giant aneurysm of CA
● For resistant Kawasaki disease which presents with fever persists or recurs despite initial
treatment: give a second dose of:
○ intravenous immunoglobulin or,
○ corticosteroids or,
○ infliximab (a monoclonal antibody against tumour necrosis factor-α)

If you find 3/ 6 then treat the pt as Kawasaki

disease:
Anemia for age
Plt >450
High WBC count in peripheral blood or in the
urine
Low albumin
Elevated Na

Even w/o Tx kids w/ Kawasaki most sx will disappear (

like: fever ..). But the problem is w/ coronary
involvement:
- Stagnation of flow through the coronaries.
- Dilation of the coronaries.
- Coronary aneurysm.
Whenever you suspect KD or you are not sure, ALWAYS
TREAT AS KD!!
 Book!

Juvenile Idiopathic Arthritis (JIA)

● Long term, with uncontrolled disease activity, there may be bone expansion from
overgrowth, which in the knee may cause leg lengthening or valgus deformity; in the
hands, discrepancy in digit length; and in the wrist, advancement of bone age.
● Proteinuria + renal failure = amyloidosis
● However, untreated anterior uveitis is associated with a high risk of developing glaucoma,
cataracts, and optic nerve damage.
● Osteoporosis is one of the complications and it is multifactorial: diet, reduced weight bearing,
systemic corticosteroids and delayed menarche
● Joint injection (steroids) >> first line of treatment for oligoarticular JIA
● STEROIDS injections are used as bridging agent in polyarticular JIA when starting
methotrexate

Prolonged fever
● Most childhood infections are acute and resolve in a few days. If not, the child needs to be
reassessed for prolonged fever is also required for prompt recognition of Kawasaki disease.
Causes:

Infective Non-infective
1. Systemic onset juvenile idiopathic arthritis.
1. Localized infection: e.g. osteomyelitis.
2. Systemic lupus erythematosus.
2. Bacterial infections: e.g. typhoid, Bartonella
3. Vasculitis (including Kawasaki disease).
3. henselae (cat scratch disease), Brucella species.
4. Inflammatory bowel disease (Crohn disease and
4. Deep abscesses: e.g. intra-abdominal
ulcerative colitis).
retroperitoneal, pelvic.
5. Sarcoidosis.
5. Infective endocarditis.
6. Malignancy: e.g. leukaemia, lymphoma,
6. Tuberculosis.
7. neuroblastoma, Ewing sarcoma.
7. Non-tuberculous mycobacterial infections: e.g.
8. Macrophage activation syndromes: e.g.
Mycobacterium avium complex.
9. haemophagocytic lymphohistiocytosis.
8. Viral infections: e.g. Epstein–Barr virus,
10. Auto-inflammatory disorders: e.g. familial
cytomegalovirus, HIV (human immunodeficiency
Mediterranean fever (FMF).
virus).
11. Drug fever.
9. Parasitic infections: e.g. malaria, toxocariasis,
12. Fabricated or induced illness (including Munchausen
Entamoeba histolytica.
syndrome by proxy).
Peds Cases !