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Often we have to look beyond to see what we are supposed to…. Pediatric Board Review Course. L. Nandini Moorthy, MD MS Pediatric Rheumatology 2009. Case 1: One swollen joint. A three year old Caucasian girl presents with two-month history of swollen left knee No history of fevers No rash
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L. Nandini Moorthy, MD MS
A three year old Caucasian girl presents with two-month history of swollen left knee
Juvenile Rheumatoid Arthritis (JRA)Now Juvenile Idiopathic Arthritis (JIA)JIA Definition
Clinical recognition of each phenotype is critical
Different courses, complications, treatments and prognosis
In older children
Leg Calve Perthes disease
Slipped capital femoral epiphysis
Exclude structural abnormalities
Other causes of oligoarthritis:
Dactylitis (< 4 ?psoriatic arthritis)
Usually do well with NSAIDS
Intra-articular steroid injections
Rarely require systemic steroids or immunosupressives
Consider the case of a 6 year old girl who presents with multiple swollen joints, morning stiffness and mild anemia.
You must think of Polyarticular JIA.
first 6 months
Atlanto-axial subluxation & C2-C3 fusion
Early carpal fusion (more often in JIA)
Symmetrically affecting the large joints of knees, elbows, ankles and wrists
Hallmark of JIA posterior cervical fusion
Labs may be normal or suggest an inflammatory state
Usually require aggressive management (immunosupressives/ biologics)
May need systemic steroids
Formerly called Still’s disease
~ 10-20% of JIA diagnoses
Affects boys & girls equally
Half of them have onset in childhood
Differential for FUO
Infectious and oncologic etiologies
Inflammatory bowel disease
Vasculitides such as polyarteritis nodosa
Usually require immunosuppressives
Commonly complain of:
Arthritis & /or enthesitis with any 2 of the following:
Bamboo spine in Ankylosing spondylitis
Acute anterior iritis
Treatment: NSAIDS, Sulfasalazine,
Or arthritis with at least two of the following:
Cytokine dysequilibrium -- increased IL-1, TNF
Increased inflammation, angiogenesis, cartilage degradation and joint destruction
EARLY INTERVENTION IS CRITICAL
If a JRA patient has an autoantibody, it confers a worse prognosis
Pauci-JRA + ANA = uveitis risk
Poly-JRA + rheumatoid factor (RF) =erosive adult RA
(REF: Mead Johnson Board Tutor)
Case 3: 10 year old Caucasian boy presented with new-onset right swollen knee of three days duration. He has been afebrile and without rash.
LN Moorthy ‘09
Case 4: 7 year old Caucasian girl presented with left swollen knee. 10 days ago she developed a sore throat. She has been afebrile and without rash.
LN Moorthy ‘09
Case 5: 14 year old Hispanic boy had bloody diarrhea a month ago and for the last 2 weeks is complaining of swelling of his left and right wrists, his left ankles, and left and right 3rd and 4th toes. He has been afebrile and without rash.
All became asymptomatic in three months.
8-41% of Peds Rheum Clinic patients (UK, US & Canada)
Hallmark: Peripheral arthritis, asymmetric arthritis & history preceding infection
May be seen in Post--Parvo viral arthritis
With Parvol-viral rash, you can also see a reticular rash
With arthritis, and history of sexual encounters- think GC!!!!!
Remember Mneumonic: Can’t See. Can’t Pee. Can’t Climb a Tree
Remember HLA B 27 association
She also presented with:
Myalgias and arthralgias
ANA titre of 1:320
(1. Other Lupus serology (anti-Sm, anti-ds-DNA, anticardiolipin antibody, lupus anticoagulant, false positve RPR)
Arthritis (non erosive polyarthritis)
Systemic lupus erythematosus: 1982 classification criteria
Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982;25:1271-1277.
Incidence: 0.36 -0.9 per 100,000 per year
Prevalence: <1- 4.5% of patients in pediatric rheumatology
Age: Childhood onset occurs in 15-17 %, Rare (but still occurs) below 5 years
Sex: More in females (Male:Female ratio 1:4.5)
In younger age group, the ratio is almost equal
Race: African-American, Hispanic, Asian, Native Higher incidence/severity of renal/CNS lupus in pediatric onset
Episodic, Chronic, Fluctuating
PRESENTATION - fever, fatigue, weight loss, irritability
Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation
Oral ulcers -Oral or nasopharyngeal ulceration, usually painless, observed by physician
Joints and Muscles: joint pain and swelling (arthralgias and arthritis), muscle pain and inflammation (myalgias and myositis) Arthritis-Nonerosive arthritis involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion
Cardiac - inflammation of different parts of the heart (pericarditis, myocarditis,valuvilitis), Myocardial infarction (Libman Sacks endocarditis)
20-40% of affected children, depression, difficulty in concentrating, cognitive impairment (~52% of children), lupus headache, seizures etc.
a) Seizures--in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance
b) Psychosis--in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance
PUNCTATE AREAS OF INFARCTS CAN BE SEEN
Rim (peripheral) SLE
Homogeneous (diffuse) Drug-induced LE; SLE
Speckled SLE Sjogren’s; MCTD; Scleroderma
Antimalarial drugs (Plaquenil)- Retinal damage
Steroids-Cushingoid appearance, thinning of skin, acne, short stature, suppresses immunity, osteoporosis, avascular necrosis (may need joint replacement), cataracts, etc.
Very important to not miss any doses/ or drastically change without speaking to your physician!
Other immunosuppressive agents -risk of infection, liver and kidney function, blood counts, risk for cancer, risk for infertility, affects bladder
Remember: Most infants have a self-resolving illness and DO NOT DEVELOP SYSTEMIC LUPUS ERYTHEMATOSUS (SLE).
Main morbidity/mortality is due to heart block which is often 3rd degree requiring the placement of a pacemaker
A 5 year-old Caucasian girl had sudden onset of muscle pains followed by weakness, symmetric, scaly, erythematous papules over the metacarpophalangeal and proximal interphalangeal joints of the hands, and reddish purple facial rash.
Uncommon idiopathic multi-system vasculitis affecting primarily the muscle, skin and gastrointestinal tract.
Diagnosis requires pathognomonic rash and two of the other criteria:
1. Symmetric weakness of proximal muscles
2. Characteristic cutaneous changes consisting of heliotrope discoloration of the eyelids with periorbital edema, and Gottron’s papules
3. Elevation of one or more skeletal muscle enzymes
4. EMG characteristic of myopathy and denervation
5. Muscle biopsy documenting histologic evidence of necrosis and inflammation
Clinical picture varies with size of vessel involved
2nd most common type of vasculitides
At least two of the following 4 should be present (ACR criteria 1990):
One-third of the children
Life threatening/Serious is <10%
Within one month of rash
Spectrum: mild proteinuria/microscopic hematuria to NS, HTN, renal failure, acute nephritic syndrome
Risk factors for renal disease:>7 years of age, persistent pupuric lesions, severe GI complaints, decreased factor XIII activity
Focal or segmental mesangial proliferation to crescentric
Opposite: IgA deposition in skin
(Wang et al)
and mucous membrane changes
An acute febrile illness of childhood with medium and small sized necrotizing vasculitis first described by Kawasaki in 1967
Fever persisting for at least 5 days (mandatory criterion) plus 4 of the following 5 should be present:
1) Changes in peripheral extremities or perineal area (usually in the subacute phase 80%)
2) Polymorphous exanthema-non vesicular (>90%)
3) Bilateral non-purulent conjunctival injection (80-90%)
4) Changes of lips and oral cavity: injection of oral and pharyngeal mucosa, strawberry tongue (80-90%)
5) Cervical Lymphadenopathy; one node>1.5 cm (50%)
In the presence of coronary artery involvement detected on echocardiography and fever, fewer
than 4 of the remaining 5 criteria are sufficient (exact number of criteria required to be defined in the
pANCA -Microscopic polyarteritis, Churg-Strauss syndrome, rapidly progressive glomerulonephritis, inflammatory bowel disease, and a variety of other conditions.
cANCA -Wegener's granulomatosis and closely related vasculitides
Lymphadenopathy -most common
Chronic cough (2nd most common)
PT and OT
En Coupe De Sabre (stroke of sword linear scleroderma on the head)
Parry Romberg (hemi-facial)
USUALLY NO SYSTEMIC
Case 12: Mask like-face and digital necrosis, Contractures of hands and thickening of skin
SYSTEMIC SCLEROSIS- DIFFUSE AND LIMITED (CREST)
Major criterion - sclerodermatous skin changes affecting areas proximal to the metacarpophalangeal or metatarsophalangeal joints
Minor criteria are as follows:
Diffuse: Interstitial pulmonary fibrosis; Anti Scl 70 ab
Limited (CREST): Pulmonary hypertension; Anticentromere ab
Primary (Common in adolescent girls)
Secondary to autoimmune disease (eg. Scleroderma,SLE)