rheumaped Flashcards Preview

pediatrics > rheumaped > Flashcards

Flashcards in rheumaped Deck (37):
1

possible triggers of juvenile RA

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

2

HLA type implicated in polyarticular Juvenile RA

HLA-DR4

3

HLA type implicated in pauciarticular Juvenile RA

HLA-DR8; HLA:DR5

4

3 principal types of onset of JRA

oligoarthritis; polyarthritis; systemic-onse dse

5

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

Juvenile Rheumatoid Arthritis

6

Criteria for JRA

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

7

clinical manifestations of JRA morning stiffness

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

8

Lab findings in JRA

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

9

Tx for JRA

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

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%

Ankylosing Spondylitis

11

Reiter Syndrome arthritis

conjunctivitis;urethritis

12

Tx of Juvenile AS

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

13

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

SLE

14

Female to Male ratio of SLE

4:1

15

Criteria for Diagnosis

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

16

antibody only found in px with SLE

anti-Smith antibody

17

TX of SLE

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

18

most common of the pediatric inflammatory myopathies

dermatomyositis

19

implicated organisms in dermatomyositis

Coxsackie B; GABHS

20

race at increased risk of developing dermatomyositis

Blacks and Asians

21

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

dermatomyositis

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

dermatomyositis

23

Tx of Dermatomyositis

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

24

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

Scleroderma

25

earliest manifestation of scleroderma

Raynaud phenomenon

26

CREST syndrome

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

27

Dx criteria for Systemic Sclerosis

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

28

Lab finding in systemic sclerosis

(+) anti-SCL70 specific for TopiIsomerase I

29

Tx for systemic sclerosis

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

30

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

Kawasaki dse

31

Diagnostic Criteria for Kawasaki dse

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)

32

Tx for Kawasaki dse

Acute phase: IVIG; Convalescent stage: Aspirin

33

IgA mediated vasculitis of small vessels

Henoch-Schonlein purpura

34

most common cause of nonthrombocytopenic purpura in children

Henoch-Schonlein purpura

35

Hallmark of Henoch-Schonlein purpura

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

36

IgA mesangial deposition on renal biopsy

Henoch-Schonlein purpura

37

Tx of Henoch-Schonlein purpura

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