Emergency Issues in Pediatric
Rheumatology
Elivette Zambrana-Flores
Assistant Professor
Pediatric Rheumatology Division
Objectives
• Discuss criteria most common rheumatic
diseases
– Systemic vasculitis
• Kawasaki Disease
• Henoch-Schonlein Purpura
– Systemic lupus erythematosus
– Juvenile dermatomyositis
– Juvenile rheumatoid arthritis
• Discuss clinical emergencies in pediatric
rheumatology
Systemic Vasculitis
Systemic Vasculitis
Heterogeneous group of
uncommon conditions
Inflammation and necrosis of blood
vessels
aberrant immune response
Systemic Vasculitis
Clinical features are determined to a
large extent by the size and distribution
of the vessels involved
Vasculitis: Classification by vessel size
•Small vessels (venules,
arterioles)
–Drug-induced and serum
sickness
–Cryoglobulinemia
–Vasculitis associated with
systemic rheumatic diseases
– Henoch-Schönlein purpura
–Vasculitis associated with
malignancy
–Hypocomplementemic
urticarial vasculitis
–Vasculitis associated with
infections
• Small and medium
muscular arteries
– Classic PAN
– Microscopic polyangiitis
– Wegener’s
granulomatosis
– Churg-Strauss vasculitis
– Kawasaki syndrome
– Rheumatoid vasculitis
– SLE
• Large arteries
– Giant cell or temporal
arteritis
– Takayasu arteritis
EPIDEMIOLOGY
Canada*
(225)
KD
HSP
Wegener’s granulomatosis
Polyarteritis Nodosa
Behcet’s disease
Takayasu’s arteritis
Unclassified
Malleson PN, et al, J Rheum 1996*
Bowyer S, et al J of Rheum 1996 ŧ
65.3%
16.9%
2.2%
1.8%
0.9%
0.9%
12.0%
USŧ
(434)
22.4%
49.1%
1.4%
3.2%
0
1.8%
22.1%
Kawasaki disease
Kawasaki disease;
criteria
•
•
•
•
•
•
Fever
Conjunctivitis
Lymphandenopathy
Rash
Changes in lips or oral mucosa
Changes in extremities
Kawasaki disease
ACUTE PHASE
1-2 WEEKS
SUBACUTE PHASE
2-4 WEEKS
CONVALESCENT PHASE
4-8 WEEKS
Kawasaki’s Disease
Criteria
ACUTE PHASE (1 - 2 weeks)
CHANGES OF MOUTH AND LIPS
•Changes in lips and oral
cavity: cracked red lips,
strawberry tongue,
erythema of oral and
pharyngeal mucosa.
Kawasaki’s Disease
Criteria
ACUTE PHASE (1-2 weeks)
CHANGES OF HANDS AND FEET
Changes in extremities:
Erythema and edema of hands and
feet
Kawasaki’s Disease
Criteria
Polymorphous exanthem
Kawasaki’s Disease
Kawasaki’s Disease
Criteria
ACUTE PHASE (1 - 2 weeks)
CERVICAL LYMPHADENOPATHY (50%-75%)
• At
least 1.5 cm
• +/- Erythema
• Nonfluctuant
• Nonpurulent
• Unresponsive to antibiotics
SUBACUTE PHASE (WEEKS 3-4)
• Resolution:
fever, lymphadenopathy and rash
• Desquamation of hands and feet (may begin)
• Coronary artery aneurysms
• Thrombocytosis
• Arthritis, irritability, anorexia, conjunctival
injection may persist in subacute phase
CONVALESCENT PHASE
( 4-8 WEEKS AFTER ONSET)
•Desquamation of fingertips and toes
•Resolution of clinical signs
•Normalizing platelet count
•Normalizing sedimentation rate
Henoch Schonlein Purpura
ACR classification criteria: HenochSchönlein purpura
• Must have at least 2 of the 4 criteria present.
– Palpable purpura
– Age < 20 years at disease onset
– Bowel angina (abnormal pain after meals or bowel
ischemia usually with bloody diarrhea)
– Granulocytes in walls of arterioles or venules on
biopsy
• Sensitivity 87.1% and specificity 87.7%
HENOCH-SCHOENLEIN PURPURA;
dermatologic disease
HENOCH-SCHOENLEIN PURPURA;
dermatologic disease
HENOCH-SCHOENLEIN PURPURA;
dermatologic disease
HENOCH-SCHOENLEIN PURPURA; joint
disease
Systemic Lupus Erythematosus
Definition: SLE
• Episodic, multisystemic, autoimmune
disease characterized by widespread
inflammation of blood vessels and
connective tissues and by the presence of
antinuclear antibodies
Systemic lupus erythematosus: 1982
classification criteria definitions
• Malar rash
Fixed erythema, flat or raised,
sparing the nasolabial folds
• Discoid rash
Raised patches, adherent
keratotic scaling,
follicular plugging; older
lesions may cause scarring
• Photosensitivity
Skin rash from sunlight
Systemic lupus erythematosus: 1982
classification criteria definitions
• Oral or nasopharyngeal
ulcers
Usually painless
• Arthritis
Nonerosive,
inflammatory in
two or more
peripheral joints
• Serositis
Pleuritis or
pericarditis
Systemic lupus erythematosus: 1982
classification criteria definitions, cont’d
• Renal disorder
Persistent proteinuria or
cellular casts
• Neurologic disorder
Seizures or psychosis
• Hermatologic
Hemolytic anemia,
leukopenia
(<4,000/mm3),
lymphopenia
(<1,500/mm3), or
thrombocytopenia
(<100,00/mm3)
Systemic lupus erythematosus: 1982
classification criteria definitions, cont’d
• Immunologic disorder
Antibodies to dsDNA or
Sm or positive
antiphospholipid
antibodies (IgG or IgM
antibodies, lupus
anticoagulant, or falsepositive serologic test
positive serologic test for
syphilis)
• Antinuclear antibody test Positive
Systemic lupus erythematosus: malar
rash, face
Discoid lupus: skin lesions, face
Systemic lupus erythematosus: oral ulcer
Systemic lupus erythematosus:
photosensitivity
Systemic lupus erythematosus:
photosensitivity
Juvenile Dermatomyositis
Definition
• JDMS is a multisystem occlusive
vasculopathy predominantly affecting
vessels in skin(typical rash),
muscle(muscle weakness), and
gastrointestinal tract
DIAGNOSTIC CRITERIA
• 4/5 criteria
– Symmetric weakness of the proximal
musculature
– Cutaneous findings
• heliotrope discoloration of the eyelids with
periorbital edema
• erythematous, scaly rash over the dorsal aspect of
the metacarpophalangeal and proximal
interphalangeal joints (Gottron’s papules)
DIAGNOSTIC CRITERIA cont.
– Elevation of skeletal muscle enzymes:
• CPK, AST, ALT, lactic dehydrogenase, and aldolase.
– Electromyographic evidence of myopathy
and denervation characterized
• brief polyphasic motor units of decreased amplitude
and high frequency discharges.
– Muscle biopsy findings
• evidence of necrosis of fibers with small blood
vessel vasculitis.
Dermatomyositis: periorbital edema and
heliotrope, eyes
Dermatomyositis: rash, face and elbows
Dermatomyositis: heliotrope rash, face
Dermatomyositis: heliotrope rash, eyelid
Dermatomyositis: Gottron’s papules and
periungual erythema, hands
Dermatomysotis: Gottron’s papules, hands
JUVENILE RHEUMATOID
ARTHRITIS
JUVENILE RHEUMATOID ARTHRITIS
• Group of diseases
• Chronic joint inflammation
• These diseases are very different in their
symptoms, treatments and outcomes
• One of the most common rheumatic
diseases of childhood
• Immunogenetic predisposition
JUVENILE RHEUMATOID ARTHRITIS
• Classification criteria and terminology of
American College of Rheumatology (ACR)
• New criteria has been recently proposed
• Almost all published data based on the
European League Against Rheumatism
(EULAR) or ACR criteria
Classification criteria for juvenile
rheumatoid arthritis
• Age at onset <16 years
• Arthritis defined as articular
swelling/effusion or the presence of two or
more of the following signs:
•
Limitation of range of movement
•
Joint tenderness on palpation
•
Pain on joint movement
•
Increased heat over joint
Classification criteria for juvenile
rheumatoid arthritis
• Duration of arthritis > 6 weeks
• Exclusion of other causes of arthritis
Juvenile rheumatoid arthritis:
synovitis, hands
Systemic-onset juvenile
rheumatoid arthritis: fever
Systemic-onset juvenile
rheumatoid arthritis: rash
Systemic-onset juvenile
rheumatoid arthritis: rash
Systemic-onset juvenile
rheumatoid arthritis: rash
Systemic-onset JRA:
hepatosplenomegaly
Systemic-onset JRA: enlarged
axillary lymph node
Medical Emergencies
Medical Emergencies
• Cardiovascular system
• Central nervous system
• Gastrointestinal system
• Pulmonary involvement
• Renal disease
Cardiovascular System
Cardiovascular System
•
•
•
•
•
Pericardium
Coronary arteries
Myocardium
Valves
Conductive system
Cardiovascular System
• Pericarditis
–Most often
• Systemic lupus erythematosus
• Juvenile rheumatoid arthritis
• Kawasaki Disease
• Acute rheumatic fever
Cardiovascular System
• Pericarditis
–SLE
• Most common cardiac complication-30%
• Rare as presenting manifestation
• < 4% will experience tamponade
• Rarely causes death
Cardiovascular System
• Pericarditis
–JRA
• 3%-9%
• Most common in systemic onset
Cardiovascular System
• Pericarditis
–Rarely
• Juvenile dermatomyositis
• Spondyloarthropathies
• Polyarteritis nodosa
• Mixed connective tissue disease
• Familial Mediterranean fever
• Scleroderma
Cardiovascular System
• Pericarditis
–Few or no symptoms
–If symptoms
• “brassy cough”
• mild shortness of breath
• substernal chest pain (positional or
irritability)
Cardiovascular System
• Pericarditis
–Examination
• Unexpected resting tachycardia
• Friction rubs
• Diminished heart sounds
Cardiovascular System
• Tamponade
–Examination
• Pulsus paradoxus
• Jugular venous distention
• Hepatomegaly
• Peripheral edema
Pericarditis
• Evaluation
–EKG
–CXR
–Echocardiogram
Pericardial effusion
Pericarditis
• Treatment
–Steroids
• Oral
• IV
–NSAIDs
–Pericardial window
Cardiovascular System
• Coronary syndromes
–Coronary ischemia
–Arteritis
–Infarcts
–Aneurysms
Coronary syndromes
•
•
•
•
•
Kawasaki Disease
SLE
Churg-Strauss granulomatosis
Polyarteritis nodosa
Wegener’s granulomatosis
Coronary syndromes
• Kawasaki Disease
–20% develop coronary artery lesions
–Death in 0.5%-2%
Coronary syndromes
Coronary syndromes
• Kawasaki Disease
– Convalescent phase
• Myocardial infarction
–Aneurysms
»thrombosis,
»Stenosis
»rupture
Coronary syndromes
• Kawasaki Disease
–IVIG
–ASA
–Steroids
Central Nervous System
CNS
• SLE
• Kawasaki disease
• Neurosarcoidosis (10%)
CNS
• SLE
–20%-95%
• 50% at onset
• 40% 1st year
–Seizures
–Psychoses
CNS
•SLE
–Headaches-10%
•Infection
•Vasculitis
•Pseudotumor cerebri
•Hemorrhage
•Visual disturbances
•Hypertension
CNS
• SLE
–Evaluation
• LP- infection, hemorrhage
• CT scan
• MRI
• EEG-detect occult seizures
CNS
• SLE
–Initial treatment
• Steroids
• Antimicrobials
• Blood pressure control
• Antiseizure medications
• Psychotropic medications
CNS
• Kawasaki Disease
–Irritability (aseptic meningitis)
–Focal neurologic involvement
–Acute hemiplegia
Gastrointestinal Tract
Gastrointestinal Tract
• Life threatening:
– JDMS:
• gut wall perforation
– SLE:
• pancreatitis, gut wall infarct, pneumatosis cystoides
intestinalis
– HSP• intussuception
Gastrointestinal Tract
• Bleeding:
– SLE
– HSP
– JRA with DIC
– Polyarteritis nodosa
Gastrointestinal Tract
• Findings:
– Asymptomatic anemia
– Frank hematemesis/hematochezia
Gastrointestinal Tract
•Evaluation:
–History
–Examination
–Laboratory studies
•CBC
•Coagulation parameters
–Imaging
Medications
–NSAIDS
–Anticoagulants
–Steroid use
GI
• Henoch-Schonlein Purpura
– Abdominal pain 35%-85%
– Serious complications 8%
•
•
•
•
Vasculitis-ischemia
GI bleeding
Hydrops gallbladder
Intussuception
– Nonspecific endoscopic findings
Gastrointestinal Tract
• Henoch-Schonlein Purpura
– Treatment
• Steroids indicated only in severe GI
involvement
–2mg/kg/day
GI
• Juvenile Dermatomyositis
–Small vessel vasculitis
• Any part of GI tract
–Hemorrhage
–Gut wall infarctions/ulceration/perforation
–Volvulus
–Infectious peritonitis
–Poor prognostic sign
Gastrointestinal Tract
• Juvenile Dermatomyositis
– Diagnosis
• Intramural or intra-abdominal free air
• Direct surgical visualization
Gastrointestinal Tract
• Juvenile Dermatomyositis
– Treatment
• Steroids
–IV pulses
Gastrointestinal Tract
• SLE
– Abdominal serositis/vasculitis
– Infection
• immunosuppressed
Pulmonary Involvement
Pulmonary Involvement
• Pulmonary hemorrhage
–25-75% mortality
• Pulmonary embolism
Pulmonary Involvement
• Pulmonary hemorrhage
– SLE- 5%
– Henoch-Schonlein Purpura
– Wegener’s granulomatosis
Pulmonary Involvement
• Pulmonary hemorrhage
–Findings
• Blood-tinged frothy sputum
• Frank hemoptysis
• Anemia and pulmonary infiltrates
Pulmonary Involvement
• Pulmonary hemorrhage
–Evaluation
• CXR
• CT scan
• Coagulation status
• Bronchoscopy
Pulmonary Involvement
• Pulmonary hemorrhage
–Treatment
• IV steroids
• Cyclophosphamide
• Plasmapheresis
Pulmonary Involvement
• Pulmonary embolism
– SLE 7.5%
• Antiphospholipid syndrome
• Nephrotic syndrome
Pulmonary Involvement
• Pulmonary embolism
– Treatment
• anticoagulation
Renal Disease
Renal Disease
• SLE
• Henoch-Schonlein Purpura
• Wegener’s granulomatosis
Renal Disease
• Evaluation:
– Renal function tests
– ANA, C3,C4, ANCA
– Renal biopsy
Renal Disease
• SLE
– 80%
– Glomerulonephritis
– Renal vein thrombosis
Renal Disease
• SLE
– Treatment
•
•
•
•
Steroids
Cellcept
Cytoxan
Rituximab
Renal Disease
• HSP
– 30%-70%
– Poor outcome- marked proteinuria,
hypertension, impaired renal function
– Serious complications-10%
Renal Disease
• HSP
– Treatment
• Steroids, methotrexate, cytotoxic agents
• Early vs late- efficacy poorly understood
Renal Disease
• Wegener’s granulomatosis
– May be initial manifestation
– Diagnosis may be delayed
• Confusion with other entities
Renal Disease
• Wegener’s granulomatosis
– Treatment
• Steroids
• Methotrexate
• Oral cyclophosphamide
Renal Disease
• Hypertension
–Most common
• SLE with nephritis
–Rarely
• HSP
• JRA
• PAN
• KD
• scleroderma
Hypertension
• Hypertensive emergency presentation
– Seizures
– Change in sensorium
– Encephalopathy
– CHF
– Renal insufficiency
– Fundoscopic changes
• Papilledema, retinal hemorrhages
Conclusion
• Medical emergencies infrequent in
pediatric rheumatic diseases
• Spectrum of rheumatic disease in children
ranges from limited and indolent to rapidly
progressive and life threatening
• Rapid diagnosis and treatment may
minimize morbidity and mortality
THANK YOU!