rheumaped

Question 1

possible triggers of juvenile RA

Answer 1

rubella; parvovirus; EBV; host hyperactivity to specific self-antigens; enhanced T-cell reactivity to bacterial/mycobacterial heat proteins

Question 2

HLA type implicated in polyarticular Juvenile RA

Answer 2

HLA-DR4

Question 3

HLA type implicated in pauciarticular Juvenile RA

Answer 3

HLA-DR8; HLA:DR5

Question 4

3 principal types of onset of JRA

Answer 4

oligoarthritis; polyarthritis; systemic-onse dse

Question 5

idiopathic synovitis of the peripheral joints with soft tissue swelling and effusion

Answer 5

Juvenile Rheumatoid Arthritis

Question 6

Criteria for JRA

Answer 6

age at onset6wks; polyarthtritis:>5 or more joints; pauci: <5(usually knees and ankles); exclusion of other forms of juvenile arthritis

Question 7

clinical manifestations of JRA morning stiffness

Answer 7

ease of fatigue esp early afternoon; joint pain later in the day; joint swelling; chronic uveitis in pauciarticular; quotidian fever with daily temp spikes of 39C for 2weeks

Question 8

Lab findings in JRA

Answer 8

Inc ESR/CRP; leukocytosisl thrombocytosis; anemia; (+) ANA in 40-85%; (+) RF (poor prognosis); Xray: soft tissue swelling; osteoporosis; periostitis; narrow cartilage space

Question 9

Tx for JRA

Answer 9

combination; depends on severity; subtype; start with NSAID proceeding to Methothrexate and immunosuppresive tx; occupational therapy

Question 10

inflammation of joints of the axial skeleton and limb; presence of enthesitis; most commonly in older boys; adolescents; and young adults; (+) HLA-B27 in 90%

Answer 10

Ankylosing Spondylitis

Question 11

Reiter Syndrome arthritis

Answer 11

conjunctivitis;urethritis

Question 12

Tx of Juvenile AS

Answer 12

anti-inflammatory; PT; psychosocial support; NSAID; Sulfasalazine(50mkd)

Question 13

autoantibody production against self-antigens resulting in inflammatory damage to target organs

Answer 13

SLE

Question 14

Female to Male ratio of SLE

Answer 14

4:1

Question 15

Criteria for Diagnosis

Answer 15

4 of 11 criteria(SOAPBRAINMD) serially or simultaneously; ANA not required; anti-dsdna-more specific and reflects disease activity

Question 16

antibody only found in px with SLE

Answer 16

anti-Smith antibody

Question 17

TX of SLE

Answer 17

prednisone (1-2mkd); severely ill: pulse steroids(30mkd; 1gm max over 60mins OD x3days); severe dse:pulse IV cyclophosphamide

Question 18

most common of the pediatric inflammatory myopathies

Answer 18

dermatomyositis

Question 19

implicated organisms in dermatomyositis

Answer 19

Coxsackie B; GABHS

Question 20

race at increased risk of developing dermatomyositis

Answer 20

Blacks and Asians

Question 21

Systemic vasculopathy with cutaneous findings and focal areas of myositis resulting in progressive muscular weaknes that is responsive to imunosuppressive tx

Answer 21

dermatomyositis

Question 22

Gottron papules; periorbital violaceous eryhthema may cross the nasal bridge; rash on upper torso and extensors; neck flexor/abdominal muscle weakness; dysphagia is a severe prognostic sign; dilated cardiomyopathy

Answer 22

dermatomyositis

Question 23

Tx of Dermatomyositis

Answer 23

sunscreen(PABA free); vitamin D; if muscle damage mild -oral steroids; severe:high dose intermittent IV methylprednisolone

Question 24

chronic dse characterized by fibrosis affecting dermis and arteries of the lungs kidneys and GIT; F:M is 3:1

Answer 24

Scleroderma

Question 25

earliest manifestation of scleroderma

Answer 25

Raynaud phenomenon

Question 26

CREST syndrome

Answer 26

calcinosis;Raynaud phenomenon; esophageal involvement; sclerosis of the skin; telangiectasia

Question 27

Dx criteria for Systemic Sclerosis

Answer 27

Major Criterion: proximal scleroderma: typical skin changes(tightness; nonpitting induration) involving areas proximal to the MCP/MTP joints; Minor Criteria: 1.sclerodactyly; 2.digital pitting scars; 3. bibasilar pulmonary fibrosis not attributable to primary lung dse; diagnosis made by major criterion OR 2 of the 3 minor criteria

Question 28

Lab finding in systemic sclerosis

Answer 28

(+) anti-SCL70 specific for TopiIsomerase I

Question 29

Tx for systemic sclerosis

Answer 29

immunosuppressive agents(methotrexate and steroids); CCB/ACE inhibitor may prevent finger tip ulceration

Question 30

severe vasculitis of ALL blood vessels esp MEDIUM-sized arteries with predilection to coronary arteries

Answer 30

Kawasaki dse

Question 31

Diagnostic Criteria for Kawasaki dse

Answer 31

Fever lasting at least 5 days; presence of at least 4/5 (1. bilateral bulbar conjunctival injection; 2. change in the mucosa of the oropharynx(eg. strawberry tongue); 3. changes in the extermities(edema/ erythema of the hands and feet; periungual desquamation); 4. rash; primarily truncal; polymorphous; 5. CLAD(1.5cm usually unilateral)

Question 32

Tx for Kawasaki dse

Answer 32

Acute phase: IVIG; Convalescent stage: Aspirin

Question 33

IgA mediated vasculitis of small vessels

Answer 33

Henoch-Schonlein purpura

Question 34

most common cause of nonthrombocytopenic purpura in children

Answer 34

Henoch-Schonlein purpura

Question 35

Hallmark of Henoch-Schonlein purpura

Answer 35

palpable petechiae or purpura that evolve from red to brown that last from 3-10days

Question 36

IgA mesangial deposition on renal biopsy

Answer 36

Henoch-Schonlein purpura

Question 37

Tx of Henoch-Schonlein purpura

Answer 37

symptomatic treatment; self-limiting; steroids for severe abdominal pain; urinalysis every 3 months regardless of severity(high recurrent rate)