Rheumatic Diseases of Childhood

Reference
Nelson Textbook of Pediatrics
Juvenile Idiopathic Arthritis
 Systemic – onset JIA
Arthritis in >1 joints with or preceded by fever at least 2 week in duration that is
documented to be daily for at least 3 days PLUS at least 1 of the following:
1. Evanescent rash
2. Generalized lymphnode enlargement
3. Hepato or splenomegaly or both
4. Serositis
 Oligoarticular JIA
Arthritis in 1-4 joints during the 1st 6 mos of disease.
1. Persistent – affecting <5 joints throughout the disease
2. Extended – affecting >4 joints after the 1st 6 mos of disease
 Polyarthritis RF (-)
Arthritis affecting >4 joints during the 1st 6 mos. RF is negative
 Polyarticular RF (+)
Arthritis >4 joints during the 1st 6 mos of disease. At least 2 test for RF (+)at least 3
mos apart
 Psoriatic Arthritis
Arthritis and Psoriasis, or arthritis PLUS at least 2:
1. Dactylitis
2. Nail pitting and onycholysis
3. 1st degree relative with psoriasis
 Enthesitis-Related Arthritis
Arthritis and/or enthesitis PLUS at least 2:
1. Presence of or a history of SI joint tenderness
2. HLAB27 Ag (+)
3. Male >6 yo
4. Anterior uveitis
5. Hx of AS, ERA, SI with IBD, reiter syndrome, or acute anterior uveitis in first degree
relative
 JIA tests
• ANA, Rheumatoid factor
• Acute phase reactants
• Radiography
 JIA treatment goals
• Achieve disease remission
• Prevent or halt joint disease
• Foster normal growth and development
 JIA treatment
• NSAIDS
• DMARDS
• Biologics
• Physical therapy
• Counseling
 JIA complications
• Chronic anterior uveitis
• Orthopedic complications
• Psychosocial disability
 JIA prognosis
• Unpredictable
• Oligoarticular – better prognosis
Systemic Lupus Erythematosus
2019 ACR/EULAR
 Autoantibodies commonly associated
with SLE

ANTIBODY CLINICAL ASSOCIATION

Anti-ds DNA Disease activity, nephritis
Anti-Smith Ab Specific for SLE
Anti-Ribonucleoprotein Ab Raynauds and pulmonary HTN;
MCTD
Anti-Ro/SSA Sicca, sjogren, neonatal lupus,
Anti-La/SSB cutaneous
Antiphospholipid Ab Venous and arterial
thrombotic events
Antihistone Ab DLE
 SLE Treatment
• Corticosteroids
• Steroid-sparing Immunosuppressants
including Methotrexate, Azathioprine,
cyclophosphamide, MMF, Biologic therapy
• Psychosocial counseling
• Sun avoidance
 SLE Prognosis
• >90% 5 year survival rate
Neonatal lupus
 Manifestation
• 1. skin rash
• 2. hepatitis
• 3. thrombocytopenia
• 4. 3rd degree heart block - permanent
• 5. presence of autoantibodies
• from mother
• Anti-Ro, Anti-La
Juvenile Idiopathic
Dermatomyositis
 JDM Diagnostic Criteria
• Classic rash
Plus 3:
– Symmetrical proximal muscle weakness
– Muscle enzyme elevation
– Electromyographic changes
– Muscle biopsy – showing inflammation, necrosis
and regeneration
 JDM complications
• Calcinosis
• Muscle atrophy, persistent weakness
• Lipodystrophy
• Airway and swallow issues
• Steroid induced complications
Scleroderma
 Scleroderma Types
• Localized
– Linear (en coup de sabre, limbs/trunk, Parry
Romberg) plaque morphea, generalized morphea,
bullous morphea, deep morphea

• Systemic
– Diffuse, Limited (CREST syndrome)
– Complications: Pulmonary and cardiac
Raynaud phenomenon
 RP
• Most common initial symptom in pediatric
systemic sclerosis
• Classic triphasic sequence of blanching,
cyanosis and erythema
Behcet Disease
 BD
• Recurrent oral and genital ulceration, with
uveitis, associated with cutaneous, arthritic,
neurologic, vascular, and gastrointestinal
manifestation
• More common in mediterranean and asia
• Etiology is unknown
• May have (+) HLA B51
Periodic fevers
 Periodic fever Types
• Familial Mediterranean Fever
• TNF recerptor associated periodic syndrome
• Hyperimmunoglobulinemia D syndrome
• Cryopyrin associated periodic syndromes
• Muckle wells syndrome
• Familial cold autoinflammatory syndrome
• Neonatal onset multosystemic inflammatory
disease
• Periodic fever aphthous ulcer, pharyngitis,
cervical adenitis
Sarcoidosis
 Sarcoidosis
• Multisystemic, with granulomatous lesions in
any organ of the body
• Biopsy: Noncaseating granulomatous lesion
• Treatment: no protocol yet
Growing Pains
 Growing Pains
• Benign nocturnal pains of childhood
• 10-20% of kids
• Peak age 4-8 yo
• Treatment - supportive
Kawasaki Disease
 KD criteria
Fever at least 5 days
PLUS 4 out of 5:
Extremity changes Acute: Erythema of palms and edema of hands and feet
Subacute: Periungual desquamation after 2 weeks
Rash Polymorphous exanthem
Conjunctivitis Bilateral and non exudative
Oral cavity changes Erythema, lip cracking, strawberry tongue, pharyngeal
redness
Lymphnode Cervical lymphadenopathy, unilateral >1.5 cm
 KD treatment
• IVIG – intravenous gammaglobulin
• MPPT – methylprednisolone pulse therapy
• ASA - aspirin
 KD Prognosis
• Coronary abnormality risk < 5% if treated
• Recurrence rate 1- 3%
Henoch-Schonlein Purpura
 HSP 2010 Criteria
Purpura Palpable in crops or petechiae with lower limb predominance
(mandatory)
PLUS at least 1 out
of 4:
Abdominal pain Monitor for intussusception and bleeding
Histopathology IgA deposits
Arthritis or arhtralgia
Renal involvement Proteinuria. hematuria
 HSP treatment
• Supportive
 HSP complications
• Acute: GI bleeding, perforation
• Chronic: Kidney disease
 HSP prognosis
• Excellent
• Self-limited course
• Recurrence in 30% within 6 mos
Takayasu Arteritis
 TA criteria
Angiographic abnormality (mandatory)
PLUS at least 1 out of 5:
Pulse deficit or claudication Loss/decreased/unequal peripheral
pulses
Blood pressure discrepancy >10mmHg difference in any limb
Bruits Over large arteries
Hypertension >95th percentile for height
Acute phase reactants ESR >20mm, CRP
 TA type of involvement
TYPE INVOLVED ARTERIES
I Aortic only
Aortic arch and descending thoracic aorta
Aortic arch and thoracic and abdominal aorta
Aortic arch and abdominal aorta
II Descending thoracic aorta only
Descending thoracic and abdominal aorta
III Diffuse
IV Diffuse aortic and pulmonary artery involvement
 TA complications
• Arterial stenosis, aneurysms and occlusions
• Ischemia of organ – may be fatal
 TA prognosis
• 20% Monophasic course
• Most have relapses
• Angioplasty and stent placement – with high
rate of recurrent stenosis
Polyarteritis Nodosa
 PAN 2010 criteria
Histopathology or angiography (mandatory)
PLUS at least 1 out of 5:
Skin involvement
Myalgia or muscle tenderness
Hypertension
Peripheral neuropathy
Renal involvement
 PAN tests

• Conventional arteriogram – GOLD STANDARD

• Others: Biopsy (skin, kidney),
electromyography
 PAN treatment
• Corticosteroids
• Cyclophosphamide
• Dapsone, methotrexate, azathioprine,
thalidomide, cyclosporine, anti TNF agents
 PAN prognosis
• Mild to severe
• Early treatment needed to prevent vascular
complications
ANCA associated vasculitis
 ANCA associated Vasculitis
• Small vessel involvement
• 3 distinct forms:
– Wegener granulomatosis (WG)
– Microscopic polyangiitis (MPA)
– Churg-strauss syndrome (CSS)
Feature WG CSS MPA
ANCA Ab PR3 MPO>PR3 MPO
Necrotizing + + +
vasculitis
Granulomatous + + _
inflammation
Asthma and _ + _
eosinophilia

PR3 – Proteinase 3
MPO – Myeloperoxidase
 ANCA Tests
• ANCA panel
 ANCA treatment
• Corticosteroids
• Cyclophosphamide
• Methotrexate, Azathioprine
• Prophylactic antibiotics
 ANCA prognosis
• Variable course
• Relapse in 75%
 Other vasculitis
Vasculitis syndrome Approach to diagnosis
Hypersensitivity vasculitis Skin biopsy
Hypocomplementemic Skin biopsy
vasculitis C1q Ab
Cryoglobulinemic vasculitis Tissue biopsy affected
Serum cryoglobulins
Exclude Hep B , C
Primary angiitis of CNS CT/MRA
Brain biopsy
Benign angiitis of CNS CT?MRA
Cogan syndrome Ophtha and audio test
CT/MRA
Thank you