rheumaped Flashcards
possible triggers of juvenile RA
rubella; parvovirus; EBV; host hyperactivity to specific self-antigens; enhanced T-cell reactivity to bacterial/mycobacterial heat proteins
HLA type implicated in polyarticular Juvenile RA
HLA-DR4
HLA type implicated in pauciarticular Juvenile RA
HLA-DR8; HLA:DR5
3 principal types of onset of JRA
oligoarthritis; polyarthritis; systemic-onse dse
idiopathic synovitis of the peripheral joints with soft tissue swelling and effusion
Juvenile Rheumatoid Arthritis
Criteria for JRA
age at onset6wks; polyarthtritis:>5 or more joints; pauci: <5(usually knees and ankles); exclusion of other forms of juvenile arthritis
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
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
Tx for JRA
combination; depends on severity; subtype; start with NSAID proceeding to Methothrexate and immunosuppresive tx; occupational therapy
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
Reiter Syndrome arthritis
conjunctivitis;urethritis
Tx of Juvenile AS
anti-inflammatory; PT; psychosocial support; NSAID; Sulfasalazine(50mkd)
autoantibody production against self-antigens resulting in inflammatory damage to target organs
SLE
Female to Male ratio of SLE
4:1
Criteria for Diagnosis
4 of 11 criteria(SOAPBRAINMD) serially or simultaneously; ANA not required; anti-dsdna-more specific and reflects disease activity
antibody only found in px with SLE
anti-Smith antibody
TX of SLE
prednisone (1-2mkd); severely ill: pulse steroids(30mkd; 1gm max over 60mins OD x3days); severe dse:pulse IV cyclophosphamide
most common of the pediatric inflammatory myopathies
dermatomyositis
implicated organisms in dermatomyositis
Coxsackie B; GABHS
race at increased risk of developing dermatomyositis
Blacks and Asians
Systemic vasculopathy with cutaneous findings and focal areas of myositis resulting in progressive muscular weaknes that is responsive to imunosuppressive tx
dermatomyositis
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
Tx of Dermatomyositis
sunscreen(PABA free); vitamin D; if muscle damage mild -oral steroids; severe:high dose intermittent IV methylprednisolone
chronic dse characterized by fibrosis affecting dermis and arteries of the lungs kidneys and GIT; F:M is 3:1
Scleroderma
earliest manifestation of scleroderma
Raynaud phenomenon
CREST syndrome
calcinosis;Raynaud phenomenon; esophageal involvement; sclerosis of the skin; telangiectasia
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
Lab finding in systemic sclerosis
(+) anti-SCL70 specific for TopiIsomerase I
Tx for systemic sclerosis
immunosuppressive agents(methotrexate and steroids); CCB/ACE inhibitor may prevent finger tip ulceration
severe vasculitis of ALL blood vessels esp MEDIUM-sized arteries with predilection to coronary arteries
Kawasaki dse
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)
Tx for Kawasaki dse
Acute phase: IVIG; Convalescent stage: Aspirin
IgA mediated vasculitis of small vessels
Henoch-Schonlein purpura
most common cause of nonthrombocytopenic purpura in children
Henoch-Schonlein purpura
Hallmark of Henoch-Schonlein purpura
palpable petechiae or purpura that evolve from red to brown that last from 3-10days
IgA mesangial deposition on renal biopsy
Henoch-Schonlein purpura
Tx of Henoch-Schonlein purpura
symptomatic treatment; self-limiting; steroids for severe abdominal pain; urinalysis every 3 months regardless of severity(high recurrent rate)
