RHEUMATOLOGY

Question 1

A 7-year-old with morning stiffness, knee and
ankle swelling for 2 months, ESR is normal,
antinuclear antibody (ANA) 1:160

Answer 1

Oligoarticular juvenile idiopathic arthritis (JIA)

Question 2

A 2-year-old female with recently diagnosed oligo JIA and positive ANA requires frequent screening for this comorbidity

Answer 2

Chronic anterior uveitis

Question 3

Fatigue, weight loss, arthritis in multiple joints for > 6 weeks, positive RF, anti-cyclic citrullinated peptide antibodies present and ANA is negative

Answer 3

Polyarticular JIA

Question 4

Fatigue, weight loss, no fever, arthritis in multiple joints, negative RF, ANA is positive

Answer 4

Polyarticular JIA with an increased risk of
uveitis

Question 5

A 5-year-old girl recently diagnosed with JIA, her
ANA is positive; how frequently does she need
screening for uveitis?

Answer 5

Every 3–4 months (JIA, < 7 years and positive
ANA is the highest risk of uveitis)

Question 6

A 9-year-old girl recently diagnosed with JIA, her
ANA is positive, how frequently does she need
screening for uveitis?

Answer 6

Every 6 months (JIA, > 7 years and positive
ANA)

Question 7

An 8-year-old with knee pain for 6 weeks, noted to have pain, swelling, decreased range of motion, difficulty bearing weight, synovial fluid shows decreased viscosity and WBC 15,000

Answer 7

Inflammatory arthritis

Question 8

Fever, salmon-colored rash with fever and hot
showers, arthritis in major joints, hepatosplenomegaly, leukocytosis, thrombocytosis, anemia of chronic disease, elevated ESR, negative RF, and negative ANA

Answer 8

Systemic JIA

Question 9

Side effects include immunosuppression, adrenal suppression, weight gain, cushingoid facies, diabetes and acne

Answer 9

Corticosteroids

Question 10

This test is very sensitive but not specific for SLE

Answer 10

Antinuclear antibody (ANA)

Question 11

Child with systemic JIA presents with elevated
liver enzymes, prolonged PTT, positive D-dimer,
thrombocytopenia, hyperferritinemia, and low ESR

Answer 11

Macrophage activation syndrome

Question 12

An African-American girl with pericarditis,
pleurisy, recurrent oral ulcers, hemolytic anemia, and RBC casts in urine

Answer 12

SLE

Question 13

Malar rash, arthritis, proteinuria, leucopenia,
thrombocytopenia, positive ANA, and anti-dsDNA

Answer 13

Systemic lupus erythematosus (SLE)

Question 14

Autoantibodies associated with arterial/venous
thrombosis or recurrent miscarriage (in patients
with or without SLE)

Answer 14

Antiphospholipid antibodies

Question 15

The most severe type of lupus nephritis resulting in hematuria, proteinuria, elevated blood pressure and can lead to end-stage renal disease

Answer 15

Diffuse lupus nephritis (membranoproliferative,
class IV)

Question 16

Neonate born with heart block, annular erythematous plaques, anemia, thrombocytopenia, and elevated liver enzymes, positive SSA (Ro) and SSB (La) antibodies

Answer 16

Neonatal lupus

Question 17

Recurrent parotitis, xerophthalmia, conjunctivitis, xerostomia, positive ANA, RF, and anti-Ro

Answer 17

Sjögren syndrome

Question 18

A 7-year-old female, with proximal muscle
weakness in both sides, arthralgia, heliotrope rash, elevated creatine phosphokinase (CPK) and LDH

Answer 18

Juvenile dermatomyositis

Question 19

A 6-year-old female with difficulty climbing stairs and voice change, Gottron papules and abnormal nailfolds, and telangiectasias

Answer 19

Juvenile dermatomyositis

Question 20

A 15-year-old had diarrhea positive for Yersinia
2 weeks ago, now is having conjunctivitis,
urethritis, arthritis of the hip and knee

Answer 20

Reactive arthritis

Question 21

Adolescent with inflammatory bowel disease
(IBD) has arthritis

Answer 21

Arthritis-related to IBD

Question 22

An 8-year-old, pain in the sacroiliac joint,
tenderness, stiffness and joint pain in the morning that improves with activity, and positive HLA-B27

Answer 22

Enthesitis-related arthropathies

Question 23

Child with nail pitting, psoriasis, arthritis, positive ANA

Answer 23

Juvenile psoriatic arthritis

Question 24

Adolescent with recurrent oral and genital ulcers, positive pathergy test

Answer 24

Behçet disease

Question 25

Adolescent girl with chronic left foot pain, minimal touch aggravates the pain, foot is swollen, warm to touch, and mottled skin

Answer 25

Complex regional pain syndrome or reflex sympathetic dystrophy (RSD)

Question 26

Adolescent, 1 year with fatigue, multiple areas of pain, tenderness, no signs of inflammation, and labs are normal

Answer 26

Fibromyalgia

Question 26

A 7-year-old boy with pain in both legs, worse in the evening, sometimes awakens him from sleep, no fever, no limping, joints are normal on exam, pain responds to ibuprofen and heat massage

Answer 26

Growing pain

Question 27

A 5-year-old boy with acute onset palpable purpura on lower extremities, joint swelling and abdominal pain, labs reveal normal CBC, PT and PTT, positive proteinuria

Answer 27

Henoch-Schönlein purpura (HSP)

Question 28

An 18-month-old with fever for 6 days, rash, conjunctivitis, strawberry tongue, and erythema of the palms and soles

Answer 28

Kawasaki disease

Question 29

The primary treatment for Kawasaki disease, ideally given between day 5 and 10 of illness

Answer 29

IVIG, aspirin

Question 30

A 15-year-old female with fatigue, weight loss, recurrent sinusitis, and joint pain. Labs reveal hematuria, proteinuria, and positive cANCA

Answer 30

Granulomatous polyangiitis (GPA, formerly Wegener granulomatosis)

Question 31

Pain, numbness, and discoloration of fingers (white then blue then red) that is triggered by cold weather

Answer 31

Raynaud’s disease

Question 32

A 14-year-old female with severe Raynaud disease with ulceration on her fingertip, tightening of her skin, sclerodactyly, positive ANA, positive anti-Scl-70

Answer 32

Systemic sclerosis

Question 32

A 12-year-old female with a linear hyperpigmented lesion on her leg that appears white and sclerotic in the center and has an erythematous border

Answer 32

Localized scleroderma

Question 33

A chest radiograph with pulmonary infiltrates and hilar adenopathy, biopsy with non-caseating granulomas, elevated ACE

Answer 33

Sarcoidosis

Question 34

Recurrent knee arthritis in an otherwise well child with a prior history of camping in the woods

Answer 34

Lyme arthritis

Question 35

Newborn infant presents a few hours after birth with hypotonia, facial muscle weakness, ptosis, weak cry, respiratory distress, the mother has a history of muscle weakness

Answer 35

Neonatal myasthenia gravis

Question 36

Adolescent with muscle weakness, worsens with repetitive movement and at the end of the day, difficulty breathing, abnormal ocular movement

Answer 36

Juvenile myasthenia gravis

Question 37

A 3-year-old child from the Middle East, recurrent fever and abdominal pain, during the episode the ESR and CRP are elevated, WBC 25,000

Answer 37

Familial Mediterranean fever

Question 38

Child with fever for 3–5 days every month associated with mouth ulcers, throat pain, cervical lymphadenitis. The patient is well in- between episodes

Answer 38

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis)