Rheumatology

Question 1

2 events considered necessary for the juvenile RA to occur

Answer 1

Immunogenetic susceptibility and external environmental trigger

Question 2

HLA of patients with juvenile RA Polyarticular disease

Answer 2

HLA DR4

Question 3

2 HLA of patients with juvenile RA pauciarticular disease

Answer 3

HLA DR8 and DR5

Question 4

Difference between pauciarticular and polyarticular types of juvenile RA in terms of number of joints involved

Answer 4

Poly - 5 or more

Pauci -

Question 5

Morning stiffness, ease of fatigue especially after school in the early afternoon, joint pain later in the day, and joint swelling

Answer 5

Juvenile RA

Question 6

Type of juvenile RA that is associated with chronic uveitis

Answer 6

Pauciarticular

Question 7

(+) RF in juvenile RA portends a poor or good prognosis

Answer 7

Poor

Question 8

(+) ANA in juvenile RA portends a poor or good prognosis

Answer 8

Good

Question 9

Most common pediatric rheumatic disease witg arthritis as the distinguishing manifestation

Answer 9

Juvenile idiopathic arthritis or juvenile RA

Question 10

Systemic juvenile RA has what pattern of fever?

Answer 10

Quotidian pattern (1-3 spikes in 24 hours with rapid return to baseline or subnormal levels)

Question 11

Duration of juvenile RA

Answer 11

6 weeks or more

Question 12

Treatment of juvenile RA

Answer 12

NSAIDS (first line)
Methotrexate
Steroids

Physical and occupational therapy
Surgery

Question 13

Rheumatoid disorder characterized by absence of RF, ANA, and other markers

Answer 13

Juvenile spondyloarthropathy

Question 14

Triad of arthritis, conjunctivitis, and urethritis caused by Chlamydia trachomatis

Answer 14

Reiter’s disease

Question 15

HLA for ankylosing spondylitis

Answer 15

B27

Question 16

Characterized by presence of enthesitis

Answer 16

Ankylosing spondylitis

Question 17

Inflammation at the sites of attachments of ligaments, tendons, fascia, and capsule to bone

Answer 17

Enthesitis

Question 18

X-ray finding in ankylosing spondylitis

Answer 18

Sacroilitis

Question 19

Serologic markers specific for SLE

Answer 19

Anti-dsDNA AND anti-Sm

Question 20

Serologic markers for SLE that may be used as marker for active disease especially lupus nephritis

Answer 20

Anti-dsDNA

Question 21

Mainstay of therapy for SLE

Answer 21

Glucocorticoids

Question 22

Drug useful for children with severe lupus nephritis and CNS lupus

Answer 22

Cyclophosphamide

Other: azathioprine, methotrexate, cyclosporine

Question 23

5-yr survival rate of SLE

Answer 23

90%

Question 24

Heliotrope discoloration of the eyelids with periorbital edema and erythematous papules over the extensor surfaces of the joints, and Gottron papules in the ankle

Answer 24

Dermatomyositis

Question 25

Most common of the pediatric inflammatory myopathies

Answer 25

Dermatomyositis

Question 26

Etiology of dermatomyositis

Answer 26

Coxsackie B and GABHS Exposure to sun is a cofactor

Question 27

HLA in dermatomyositis

Answer 27

Ag DQA1*0501

Question 28

Excessive accumulation of collagen in the skin and other organs

Answer 28

Scleroderma

Question 29

Skin thickening with loss of dermal ridges, resembling tightened skin

Answer 29

Scleroderma

Question 30

Antibodies in limited scleroderma

Answer 30

Anti centromere

Question 31

Antibodies for systemic scleroderma

Answer 31

Anti Scl 70

Question 32

Earliest manifestation of scleroderma

Answer 32

Reynauds phenomenon

Question 33

Scleroderma associated with CREST

Answer 33

Limited scleroderma

Question 34

HSP is mediated by

Answer 34

IgA

Question 35

Involved vessels in HSP

Answer 35

Small blood vessels

Question 36

Skin biopsy result of leukocytoclastic angiitis; renal biopsy result of IgA mesangial deposition

Answer 36

HSP

Question 37

Treatment of HSP

Answer 37

Symptomatic Self limiting Steroids for severe abdominal pain

Question 38

Monitoring for HSP patients

Answer 38

Urinalysis every 3 months for 1 years (high recurrence rate)

Question 39

Polyarteritis nodosa is associated with what infection in adults? In children?

Answer 39

Hep B; GABHS

Question 40

Medium vessel or small vessel vasculitis characterized by aneurysms and thrombosis (brachial, femoral, or mesenteric arteries)

Answer 40

Polyarteritis nodosa Immune complex disease

Question 41

Livedo reticularis, subcutaneous nodules with skin, GI, and kidney involvement

Answer 41

Polyarteritis nodosa With HPN, peripheral neuropathy

Question 42

Treatment of polyarteritis nodosa

Answer 42

High dose corticosteroid | Cyclophosphamide

Question 43

Characterized by necrotizing granulomas in multiple organs, most commonly the respiratory tract and kidneys

Answer 43

Wegener's granulomatosis

Question 44

Severe sinusitis, hemoptysis and glomerulonephritis are seen in

Answer 44

Wegener's granulomatosis

Question 45

Antibodies in wegener's granulomatosis

Answer 45

c-ANCA

Question 46

A vasculitis syndrome characterized by a triad of aphthous stomatitis, genital ulceration, and uveitis

Answer 46

Behcet's disease CNS and GI involvement

Question 47

Treatment of Behcet's disease

Answer 47

Corticosteroids and thalidomide

Question 48

Skin reaction to a needleprick seen in Behcet's disease

Answer 48

Pathergy

Question 49

What is the most serious cardiac complication of neonatal lupus?

Answer 49

Heart block

Question 50

Puffiness around fingers, dorsum of the hands and face, followed by skin tightening

Answer 50

Systemic sclerosis

Question 51

Sclerodactyly, digital pitting scars, and bibasal pulmonary fibrosis are minor criteria for

Answer 51

Systemic sclerosis Major criteria: proximal scleroderma

Question 52

Medications that may prevent fingertip ulcerations

Answer 52

Calcium channel blockers and ACE inhibitors