A11 - Pediatric Rheumatology 2026

FEVER at least 5 days + 4 of:

• Conjunctivitis

• Rash

• Adenopathy

• Strawberry tongue

• Hands and feet (peeling)

• CRASH and Burn (fever)

Criteria for Kawasaki Disease

Medium-sized vasculitis

Kawasaki Disease is considered what type of vasculitis?

1-3 weeks from onset

When does periungual desquamation occur in patients with KD?

2nd-3rd week of illness

When does coronary aneurysm occur in patients with untreated KD?

Convalescent phase

Clinical phase of KD when all clinical signs have disappeared and continues until ESR and CRP return to normal about 6-8 weeks after the onset.

Subacute phase

Clinical phase of KD when fever & other acute signs have abated and is associated with desquamation, thrombocytosis, and development of coronary aneurysms (if untreated)

Atypical Kawasaki Disease

KD suspect with 2-3 of the principal clinical features, but have compatible laboratory findings.

Incomplete Kawasaki Disease

Children with unexpected fever for 5 or more days with 3 or less of the clinical criteria for KD but with laboratory evidence of systemic inflammation

At diagnosis and repeated

after 2-3 weeks of illness

When is 2D echo done for patients with KD?

IV Ig and

Aspirin 80-100 mkDay until afebrile for at least 48 hours

Preferred treatment of Kawasaki in the acute stage of the disease?

Aspirin 3-5 mg/kg OD PO until 6-8 weeks after illness

onset

Treatment of KD in convalescent stage with normal coronary findings?

Aspirin

Clopidogrel

(consider LMWH, warfarin)

Long-term therapy for KD patients with coronary abnormalities

IV methylprednisolone

KD patients with poor response to the multiple IVIG doses. May consider giving?

MIS-C

Patient < 21 y/o with Kawasaki-like features and history of exposure to Covid, recent/past infection with Covid. Impression?

Juvenile Dermatomyositis (JD)

Most common of pediatric inflammatory myopathy

Gottron papule

In patients with JD, hypertrophic and reddish skin over the metacarpal and proximal IPJ is called?

Heliotrope rash

Periorbital violaceous erythema that may cross over the bridge of the nose in patients with JD.

Classic rash

(heliotrope rash of the eyelids, Gottron papules)

PLUS 3 of the ff:

1. Weakness (symmetric, proximal)

2. Muscle enzyme elevation (CK, AST, LDH, aldolase)

3. EMG changes (myopathy, denervation)

4. Muscle biopsy (necrosis, inflammation)

Diagnostic criteria for dermatomyositis

Antibodies to Pm/Scl

This test identifies a small, distinct group with a protracted disease course with pulmonary and cardiac involvement in dermatomyositis.

• Methylprednisolone for more severe cases

• Methotrexate decreases the length of treatment with steroids

• Folic acid reduces toxicity and S/E of folate inhibition

• IVIG for severe cases

Management of dermatomyositis

Henoch Schönlein Purpura

Most common cause of nonthrombocytopenic purpura in children

Leukocytoclastic angiitis

Skin biopsy findings in HSP?

IgA mesangial deposition

Kidney biopsy findings in HSP?

Oligoarticular JIA

An adolescent with arthritis affecting 1-4 joints during the 1st 6 months of disease.

• Naproxen

• Ibuprofen

• Meloxicam

NSAIDs of choice for polyarthritis, oligoarthritic, systemic JIA

• Methotrexate

• Sulfasalazine

DMARDs for polyarthritis, oligoarthritic, systemic JIA

Etanercept

JIA treatment that targets TNF

Canakinumab

JIA treatment that targets/inhibits IL-1

• Failure of methotrexate monotherapy

• With poor prognostic factors

• With severe disease onset

When to consider DMARDs for JIA?

Start with corticosteroids followed by IL-6 antagonist

Treatment for systemic JIA

ANA at a titer of equal or >1:80 on HEp-2

cells or an equivalent positive test

An entry criterion necessary for diagnosis of SLE

≥10 points

SLE classification requires at least ONE clinical criterion and how many points from the different domains?

• Constitutional (fever)

• Hematologic (leukopenia, thrombocytopenia, hemolysis)

• Neuropsych (delirium, psychosis, seizure)

• Mucocutaneous (non-scarring alopecia, oral ulcers, subacute cutaneous / discoid lupus, acute cutaneous lupus)

• Serosal (pleural/pericardial eff, acute pericarditis)

• MSK (joint involvement)

• Renal (proteinuria >0.5g/24h, Renal biopsy Class II – V lupus nephritis)

Clinical domains and criteria in SLE

• Antiphospholipid Ab (Anti-cardiolipin Ab or Anti- β2GP1 Ab or LAC)

• Complement CHONs (low C3, C4)

• SLE-specific Ab (anti-dsDNA, anti-Sm)

Immunologic domains and criteria in SLE

Anti-dsDNA

Antibody more specific for lupus & reflects the degree of disease activity

Anti-Smith

Antibody specific for the diagnosis of SLE