Pediatric Examination and Board Review (245 page)

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Rheumatic Disease Clinics of North America
. 2002;28:531-60.

Hashkes PJ, Laxer RM. Update on the medical treatment of juvenile idiopathic arthritis.
Current Rheum Reports.
2006;8:450-458.

Petty RE, Cassidy JT. Chronic arthritis. In: Cassidy JT, Petty RE, Laxer RM, Lindsley CB, eds.
Textbook of Pediatric Rheumatology.
5th ed. Philadelphia, PA: Elsevier Saunders; 2005:206-323.

Petty RE, Southwood TR, Baum J, et al. Revision of the proposed classification criteria for juvenile idiopathic arthritis: Durban 1997.
J Rheumatol.
1998;25:1991-1994.

Ravelli A, Martini A. Juvenile idiopathic arthritis.
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2007;369:767-778.

CASE 141: A 15-YEAR-OLD GIRL WITH FATIGUE, FEVER, AND ARTHRITIS

A previously healthy 15-year-old girl has had fatigue, low-grade fevers, sore throat, joint pain and stiffness for the past few weeks. On physical examination, she has cervical adenopathy and mild swelling of her finger joints. Her throat culture is negative for group A streptococcus. CBC: leukocyte count 3.9/mm
³
with a normal differential; the platelets are 165,000/mm
³
and the hemoglobin is 10.5 g/dL.

SELECT THE ONE BEST ANSWER

1.
The differential diagnosis for this patient includes

(A) EBV infection

(B) leukemia

(C) SLE

(D) A and C

(E) A, B, and C

2.
Her ANA titer is 1:320. In addition to SLE, ANAs can be found in which of the following?

(A) patients taking phenytoin

(B) healthy individuals

(C) patients with an EBV infection

(D) A and C

(E) A, B, and C

3.
Autoantibodies that are specific for SLE include

(A) anti-double-stranded DNA (anti-dsDNA) and antiribonuclear protein (anti-RNP)

(B) anti-dsDNA and anti-Smith (anti-Sm)

(C) anti-SS-A and anti-Sm

(D) antihistone antibodies and anti-dsDNA

(E) anti-SS-A and anti-RNP

4.
Which of the following skin findings is not included in the classification criteria used for the diagnosis of SLE?

(A) erythema marginatum

(B) photosensitivity

(C) malar rash

(D) discoid lesions

(E) mucocutaneous ulcerations (oral and/or nasal)

5.
Which of the following complications does not occur in SLE?

(A) platelet counts less than 5000/mm
³
(B) psychosis, seizures, or cognitive dysfunction

(C) interstitial pneumonitis, interstitial fibrosis, or pleuritis

(D) progressive erosive arthritis in most patients who have joint swelling

(E) pericarditis, myocarditis, or valvular dysfunction

6.
Which statement regarding renal disease in pediatric SLE is true?

(A) a normal urinalysis rules out lupus nephritis

(B) lupus nephritis usually recurs in a transplanted kidney

(C) lupus nephritis occurs in approximately twothirds of children with SLE

(D) patients with mesangial disease on biopsy are at increased risk for progression to renal failure compared with those with proliferative lesions

(E) the presence of anti-Sm antibodies increases the risk of lupus nephritis

7.
All of the following are true regarding laboratory evaluation in SLE except

(A) the erythrocyte sedimentation rate and CRP are good markers for subsequent SLE disease activity

(B) an elevated leukocyte count is associated with the use of high-dose CS or may be secondary to infection

(C) C3 and C4 complement levels may be helpful in following disease activity

(D) increases in anti-dsDNA levels may indicate impending exacerbation of disease

(E) patients with SLE may have a combination of a Coombs positive hemolytic anemia and thrombocytopenia, known as Evans syndrome

8.
Which statement is false regarding antiphospholipid antibodies (APLA)?

(A) the risk of both venous and arterial thrombosis is increased in patients with APLA

(B) all patients with SLE should be checked for the presence of APLA

(C) examples of APLA include lupus anticoagulant, anticardiolipin antibodies, anti-beta
₂
-glycoprotein

(D) the presence of APLA increases the risk of miscarriage

(E) more than 90% of patients with APLA have an underlying rheumatic disease

9.
Which statement regarding the treatment of SLE in children is not true?

(A) hydroxychloroquine is often used to treat skin, joint, and muscle inflammation

(B) complications of CS treatment include avascular necrosis, osteoporosis, hypertension, and diabetes

(C) long-term prednisone doses of less than 8 mg/day in children will not cause growth suppression

(D) cyclophosphamide treatment may cause gonadal injury and may increase the risk of oncogenesis

(E) stress doses of CS may be necessary 6-12 months after cessation of chronic CS treatment

10.
All of the following statements are true about childhood SLE except

(A) infection is a major cause of early mortality in children with SLE

(B) cardiovascular disease, including myocardial infarction and stroke, is a major long-term cause of morbidity and mortality

(C) most children are able to discontinue all medications after 5 years of treatment

(D) 10-year survival rates have increased since the availability of CS from less than 50% to more than 90%

(E) 20% of adults with SLE were either diagnosed with SLE or developed initial SLE symptoms during childhood

11.
All are true about neonatal lupus except

(A) the risk of having a baby with neonatal lupus increases if the mother has active lupus during pregnancy

(B) most children with congenital atrioventricular heart block require a pacemaker

(C) neonatal lupus rash, thrombocytopenia, and hepatitis usually resolve spontaneously by 6 months of age

(D) neonatal lupus is strongly associated with the presence of anti-Ro (SS-A) and/or anti-La (SS-B)

(E) a mother may have a baby with neonatal lupus even if she has never had a diagnosis or symptoms of a rheumatic illness

12.
Which statement is correct regarding drug-induced lupus?

(A) all patients require treatment with CS to control symptoms

(B) renal involvement is common

(C) anti-dsDNA is usually positive

(D) minocycline is one of several drugs known to cause drug-induced lupus

(E) complement levels are usually depressed in drug-induced lupus

13.
Which of the following statements is true about Raynaud?

(A) the order of the triphasic color change is red → white → blue

(B) primary Raynaud, which is not associated with any underlying rheumatic disease, is more common in children than adults

(C) Raynaud phenomenon occurs in approximately a third of children with SLE and in more than 90% of children with systemic sclerosis (diffuse scleroderma)

(D) Raynaud is seen more often in males than females

(E) the order of the triphasic color change is red → blue → white

14.
Approximately 4 weeks ago, a mother noticed that her 7-year-old son had a rash on his face and knuckles. Two weeks later, she noted he had decreased endurance and was having difficulty running and climbing at the playground. His teacher reported to his mother that he was having problems ascending 2 flights of stairs to go to music class. Which of the following would you not expect to find on initial physical examination of this child?

(A) dystrophic calcification

(B) violaceous discoloration of the upper eyelids

(C) Gower sign

(D) muscle tenderness

(E) diffuse cutaneous vasculitis

15.
Which of the following is not helpful in diagnosing juvenile dermatomyositis (JDM)?

(A) presence of Gottron papules and a heliotrope rash over the eyelids

(B) presence of ANA and an elevated sedimentation rate

(C) elevated serum muscle enzymes

(D) proximal muscle weakness

(E) muscle biopsy showing inflammatory cell infiltrate, perifascicular atrophy, and necrosis

16.
Which statement is not true regarding the complications and outcome of JDM?

(A) calcinosis may cause limitation in joint range and cosmetic problems

(B) more than two-thirds of patients have good to excellent functional outcomes

(C) mortality is usually secondary to respiratory failure or gastrointestinal (GI) vasculitis

(D) lower esophageal dysfunction often leads to dysphagia

(E) in severe cases, dysphonia may be present

17.
For the past few days, a 5-year-old child has been complaining of intermittent abdominal pain. No emesis or diarrhea has been reported. Then her mother noted a red rash on her legs and brought her to your office. Your examination reveals a nontoxic child who is afebrile. She has palpable purpuric lesions, coalescing by the ankles, and ascending up to the buttocks. Her abdominal examination reveals mild diffuse tenderness but no rebound. You suspect that she has Henoch-Schönlein purpura (HSP). Which of the following statements regarding HSP is not true?

(A) the rash may recur during the first 6 weeks after presentation, often increasing after physical activity