Pediatric Board Review - Rheumatology Flashcards

Terms Definitions
Which of the following can cause arthritis in kids?
Genetic syndromes
Viral (Toxid synovitis)
Evasive infection (ie septic arthritis)
All of them
Which of the following are normal and which are positive in Ankylosing Spondylitis?
HLA B27 is positive
ANA normal
RF normal
ESR elevated
A 16 yo boy has night pain in his knee which is releived with exercise. On x-ray you see a bamboo spine. What is the diagnosis?
Ankylosing spondylitis
A 15yo boy has back and leg pain along with weight loss and low grade fever. His x-ray looks like this. What is his diagnosis and treatment?
Bamboo spine. Ankylosing spondylitis, ossification fo annulus fibrosis. HLA-B27.

Rx: exercise, NSAIDS, Methotrexate, etanercept, infliximab
A child has apthous ulcers, genital, GI ulcers, uveitis and arthritis. His ANA and RH are normal but ESR and CRP are high. What is the diagnosis and treatment?
Behcet's syndrome

Think oral, genital and stomach ulcers while eating a baguette
A child younger than ten has this rash as well as shiny skin on extensor surfaces of the extremities (Grotton Papules). She is clumsy and has been having difficulty climbing steps and getting dressed. What is the diagnosis and first step in evaluation?
Dermatomyositis and D) CK level.
A 4yo child presents with poor wound healing and multiple bruising. She has hyper-elastic skin and joints. What is her diagnosis?
Ehlers-Danlos Syndrome
A child has hyper elastic joints, blindness, mental retardation, and hypotonia. (ie. low tone, low cognition, low vision) What is the diagnosis?
Lowe's syndrome
A 5yo boy has had palpable blanching purpura on lower exptremities and buttocks. He also has colicky abdominal pain with hematuria and proteinuria. His labs show an elevated BuN, creatinine and heme positive stools. He is diagnosed with HSP. What is the p
Vasculitis of skin, GI tract, joints, kidneys.
True or false.
Intussusception is a potential complication of HSP and it can be easily detected by barium or air enema.
False. Intussusception is a complication of HSP, and it occurs in an ileoileal location making it difficult to detect by air or barium enema.
True or False.
HSP is associated with joint involvement.
False. It is associated with periarticular involvement typically around the soft tissues around the knees and ankles.
True or False
HSP typically has normal platelet levels.
What is the treatment for HSP?
Supportive care
How long must there be symptoms to diagnose JRA?
At least 6 weeks
True or False.
Large joint involvement is more common than small joint involvement in JRA.
True are False.
ANA and RF are usually positive in JRA.
False. ANA is positive but RF is not.
Which of the following is not typical of JRA?
A) Morning stiffness
B) Gradual loss of motion
C) Rash
D) RF positive
E) ANA positive
How many joints are affected in polyarthritis?
5 or more
A teenage girl has JRA. Which type is most consistent with the following characteristics. 1=Oligoarthritis, 2=Polyarthritis and 3= Systemic JRA
A) Males and Females equal
B) Extraarticular involvement
C) HLA-B27
D) Chronic uveitis
E) ANA positive in young
A) Males and Females equal -- 3
B) Extraarticular involvement -- 1
C) HLA-B27 -- 2
D) Chronic uveitis -- 2
E) ANA positive in young females -- 1 and 2
F) 4 or fewer joints involved -- 2
G) 5 or more joints involved -- 1
Which of the following is a harbinger of systemic JRA?
A) High fever
B) WBC 30000-50000
C) Rash
D) Hepatosplenomegaly
E) Lymphadenopathy
F) Pleuritis/Pericarditis
G) All of the above
Are ANA and RF positive or negative in systemic JRA?
How do you distinguish JRA and acute leukemia?
JRA has morning stiffness. Acute leukemia is likely to awaken the child at night and bone pain does not involve a joint.
True or False
LAD and HSM are present in JRA and in acute leukemia.
What are the 1st and 2nd line treatments for JRA?
NSAIDS then steroids and immunosuppressants.
What is the 1st line treatment for JRA with cardiac involvement?
A 2yo presents with 5 days of fever. She also has conjunctivitis, desquamation of hands and feet, cervical LAD, a polymorphous exanthem and these are her lips. What is this and what is the treatment?

What is the diagnosis and treatment.
Kawasaki syndrome - also called mucocutaneous lymph node syndrome. This reaction involves inflammation of mucous membranes, erythematous skin rashes followed by desquamation (particularly marked around the nails folds), high fever and lymphadenopathy. The trigger is unknown. Treatment involves high dose aspirin and immunoglobulin..
What is the treatment for Kawasaki?
High dose aspirin and IVIG.
What do you expect on CBC of a child with Kawasaki on the 5th day AND 12th day?
elevated platelets on 5th day, leukocytosis on 12th day
Normocytic anemia is present throughout
How long are CRP, ESR, Alpha-1-antitrypsin elevated?
4-6 weeks.
True or False.
ANA and RF and circulating anticoagulant will not be elevated in Kawasaki disease.
What is the major worrisome complication of Kawasaki? How do you monitor for this complication?
A serious complication is coronary arteritis and aneurysm formation.
Do a 2D ECHO at time of diagnosis, then in 2-3 weeks then 6-8 weeks from initial diagnosis.
What is the treatment regimen for KD?
IVIG 2g/kg and ASA 80mg/kg/day for 2 days THEN ASA 5mg/kg/day for 2 months.
Of the following findings. Indicate 1 for Kawasaki, 2 for JRA/Systemic type or 3 for both.
1. Acute onset fever
2. Adenopathy
3. Evanescent Reticular Rash
4. Conjunctivitis
5. Polymorphous exanthem
6. Desquamation of hands and feet.
7. Hepatomegaly
8. Gr
1 for Kawasaki, 2 for JRA/Systemic type or 3 for both.
1. Acute onset fever = 1
2. Adenopathy = 3
3. Evanescent Reticular Rash = 2
4. Conjunctivitis = 1
5. Polymorphous exanthem = 1
6. Desquamation of hands and feet. = 1
7. Hepatomegaly = 2
8. Gradual onset = 2
9. Cardiomegaly = 2
10. Pleural effusions = 2
How do you distinguish measles and kawasaki?
Measles rash goes head to toe and the conjunctivitis is exudative.
How do you distinguish rash of scarlet fever and that of kawasaki?
Scarlet fever rash is primarily in flexural areas
True or False.
Lyme disease may present with joint pain and history of fatigue and cold like symptoms.
A patient comes to ED and you suspect Lyme. What is the first test you order?
Lyme antibody titer then Western blot for confirmation.
What is the treatment for lyme disease?
30 days of doxy or erythromycin.
A child is being treated for lyme disease with appropriate antibiotics. She comes into ED hypotensive, febrile and septic. What is the reason?
The medications are lysing the bacteria which is causing toxin release.
Which of these is not within the diagnostic criteria of Marfan's syndrome?
A) Dilitation and dissection of the ascending aorta
B) Lumbosacral dural ectasia
C) Mitral valve prolapse
D) Ecotopia lentis
E) Four skeletal anomalies
What are the two preventive measures that are recommended for patient's with Marfan?
Regular echo and regular slit lamp exam
A 15yo comes in complaining of vague joint aches. She recently received an MMR booster but is otherwise healthy. What would explain the joint aches?
Post infectious arthritis from the rubella in the MMR vaccine especially in a post-pubertal girl.
Is the ANA and RF expected to be positive or negative in reiter's syndrome?
What are the three problems in Reiter's syndrome?
Can't see, pee, or climb a tree.
Conjunctivitis, Urethritis, Arthritis
True or False
Urethritis and Conjunctivitis are typically associated with chlamydia or gonorrhea.
True or False
Reactive arthritis (or Reiter's syndrome) typically occurs after a bacterial enteral or venereal disease.
What is the treatment for Reactive arthritis?
NSAIDs and Antibiotics
A young boy comes in with R knee pain and left ankle pain. He also has conjunctivitis and dysuria. What is the cause of his dysuria?
Urethritis from reactive arthritis.
A 5yo child comes in after recent pharyngitis. Today she is complaining of knee pain. Her mom comments that normally her child is very calm and relaxed, but lately she has been emotional and has these fits of flailing her arms unnecessarily. On exam you n
Rheumatic fever.
A child with all the symptoms of rheumatic fever has mild CHF. What is the likely problem?
Aortic regurgitation.
A child has a holosystolic murmur heard best at the apex. What is the most likely murmur?
Mitral regurgitation.
What are the requirements of Rheumatic Fever diagnosis?
Recent Group A Strep infection.
2 Major Criteria
1 Major or 2 Minor Criteria
What are the Major Criteria for Rheumatic Fever?
Joints = Polyarthritis
❤ = Carditis (CHF, new murmur, cardiomegaly)
N = nodules - firm and painless on extensor surfaces of wrists, elbows and knees
E = erythema marginatum
S = sydenham chorea - rapid purposeless movements of face and arms
What are the Minor Criteria for Rheumatic Fever?
H = hot - fever
E = elevated acute phase reactants
L = arthaLgia
P = prolonged PR interval
True or False.
The arthritis of rheumatic fever is migratory and the rash is erythema marginatum.
What "SHAPeD" your treatment regimen for rheumatic fever?
S = steroids
H = Haldol
A = aspirin
P = penicillin
extra support
D = digoxin for CHF
Which of the following are acceptable as proof of strep infection? (Pick 2)
A) Throat culture
B) Rapid strep test
C) ASO titer
D) Positive streptozyme.
C and D
A 12yo African American boy is brought to your office for a chronic cough and constantly being tired. He has no TB contact and a negative PPD. You order labs and note hypercalcemia and renal insufficiency. This is the CXR. What is the diagnosis?
A 14yo girl has a linear hyper-pigmented patch with a brown border that is painful and tender. What is the diagnosis and treatment?
This is scleroderma. If limited to fingers treat with topical lubricants. If widespread (Type 3), treat with steroids, immunosuppressives, methotrexate and peicillamine.
True or False.
ANA is positive in systemic scleroderma.
What is the most common pathogen in septic arthritis for kids over age 2yo-teen. What about for teens?
For 2yo-teen is S. aureus
For teens it is N. gonorrhea
How do you diagnose septic arthritis?
Aspirate the joint
A 16yo girl has FEVER, URTICARIA and ARTHRALGIA. She can not recall any recent allergic reactions and has been healthy other than a recent UTI which was treated with bactrim.
What is her diagnosis?
Serum sickness. Type 3 hypersensitivity.
What is the treatment for septic arthritis?
Long term IV Antibiotics. Do not wait.
What are the three classic features of Sjogren's syndrome?
Dry mouth (xerostomia), Dry eyes (xeropthalmia), Painless parotid swelling.
What is the Shirmer test?
It measures tear production
What is the risk of untreated Sjogren's?
What is Sjogren-Larsson Syndrome?
An autosomal recessive inborn error in lipid metabolism.
True or False.
SLE is more common in caucasians than blacks.
False. It is 4 times more common in blacks.
True or False.
SLE is more common in girls than boys.
False. This is true in adults, but it is gender equal in boys.
What is the diagnostic serology in SLE?
Anti-smith antibodies
What is the most specific serology for SLE?
Anti-smith antibody
What is the screening test for SLE?
True or False.
Lupus anticoagulant can result in a false positive serology for syphillis.
What is Raynaud's Triad?
White ischemic fingers
Red cold fingers
Blue cyanotic fingers
Which drugs cause Drug Induced Lupus (DIL)?
D = Drugs
S = Sulfonamides
L = Lithium
E = Epilepsy

Queen (Quinadine) Hydalazine Procainamide
What is the most sensitive test for Drug induced Lupus?
What are the signs of active reanl disease in lupus?
Increased anti-DNA titers, Decreased C3, C4 and CH50.
True or False
Microischemia and vascular disease result in seizures in SLE
What would be the most likely explanation for death of an infant born to a mother with SLE?
Heart Block.
A neonate presents with a scaly erythematous rash, bradycardia and thrombocytopenia. What are the confirmatory tests for neonatal lupus?
Anti-Ro and Anti-La
What are the best indicators of good response to treatment in SLE?
Normalization of C3 and C4.
What are the two major side effects of hydroxychloroquine?
Ototoxicity and Retinal damage
What is the treatment for mild SLE?
then hydroxycholorquine (plaquenil)
then methotrexate
What is the treatment of severe SLE?
High dose steroids and cyclophosphamide.
True or False.
Lupus patients undergoing treatment with immunosuppressive agents are at risk for varicella.
What are the three organ systems involved in Wegener Granulomatosis?
What determines the severity of Wegener Granulomatosis?
Extent of kidney involvement.
What are the expected lab findings in Wegener?
C-ANCA with joint pain, fevers and sinusitis.
What is the treatment for Wegener?
A child has bilateral joint pains without any swelling. It is worse at night. You note that he walks with a limp. What is the diagnosis?
Growing Pains
An athelete has loose joints. What is your advice to this anymore?
The must be mindful of stretching other than that.
Synovial Fluid is yellow or clear, has increased viscosity and WBC < 200. What is it?
Normal synovial fluid
Synovial Fluid is bloody or clear, has increased viscosity and WBC < 2000. What is it?
Arthritis secondary to trauma
Synovial Fluid is yellow or clear, has normal viscosity and WBC 5000 and LE cells. What is it?
Synovial Fluid is yellow and cloudy, has decreased viscosity and WBC 15000-20000. What is it?
Synovial fluid is yellow and cloudy and has decreased viscosity and WBC 5000. What is it?
Rheumatic Fever
Synovial fluid is yellow and opaque and has decreased viscosity and WBC 20000. What is it?
Synovial fluid is yellow and has variable viscosity and WBC 50000 - 300000 and low glucose and low bacteria. What is it?
Septic arthritis.
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