Medstudy Rheumatology

Front 1

Initiated by presentation of antigens to T lymphocytes by antigen presenting cells, activation causes T and B lymphocyte production and cytokine release (TNFa, IL-1, IL-6)

Back 1

JIA

Front 2

Morning stiffness, changes in walking/running/climbing, leg length discrepancies, assistance with bathing/dressing, enuresis, loss of developmental milestones, widening of mid-portion of affected phalanges on xray

Back 2

JIA

Front 3

Female predominance, uveitis, +ANA, need ophtho exam Q3months x 4 yrs, absent fever/rash/night pain

Back 3

Oligoarticular JIA

Front 4

Female predominance, RF+ pts are older, affects large and small joints (cervical spine, hips, shoulders, temperomandibular joints) uveitis uncommon

Back 4

Polyarticular JIA

Front 5

Daily fever spikes associated with myalgia/arthralgia, rash on trunk, thighs, and axilla precipitated by Koebner phenomenon, synovitis, normal CPK but elevated aldolase, rare uveitis, RF negative, rare ANA positivity

Back 5

Systemic onset JIA

Front 6

Elevated transaminases, coagulopathy with positive D-dimer and prolonged PTT, thrombocytopenia, low ESR, hemophagocytosis in BM

Back 6

Macrophage Activation Syndrome

Front 7

Older children, male predominance, HLA-B27 association, peripheral, asymmetric arthritis

Back 7

Enthesitis Related Arthropathies

Front 8

Diagnostic Criteria for ERA

Back 8

arthritis and/or enthesitis plus SI joint tenderness, inflammatory spinal pain, HLA-B27 present, anterior uveitis, onset in boys older than 8

Front 9

Peripheral Joint - girls = boys, not associated with HLA-B27, flares with disease, Axial Joints - boys > girls, associated with HLA-B27, not dependent on disease flares

Back 9

Arthritis asociated with IBD

Front 10

Arthritis (common in DIP joints) plus 2 of: dactylitis, nail findings, family history in one 1st degree relative, acute/chronic anterior uveitis is common

Back 10

Psoriatic Arthritis

Front 11

Caused by immune complexes, presents with purpura and includes drug rxns, serum sickness, and HSP

Back 11

Small-vessel Vasculitis

Front 12

Causes organ system damage, includes PAN and Kawasaki

Back 12

Medium-vessel Vasculitis

Front 13

Causes claudication symptoms, includes Takayasu's

Back 13

Large-vessel Vasculitis

Front 14

Most common vasculitide in kids, IgA mediated, boys>girls, leukocytoclastic vasculitis with neutrophil infiltration in vessel walls, URI often precedes disease, rash, orchitis, abd pain, pulmonary hemorrhage

Back 14

HSP

Front 15

U/S reveals echogenicity and thickening of wall of 2nd portion of duodenum, hydrops of gallbladder, intussusception occurs

Back 15

HSP

Front 16

Fever plus: rash, bilateral conjunctival infection without exudate, changes in lips/oral cavity, changes in extremities, cervical LAD, CAD

Back 16

Kawasaki Disease

Front 17

Acute phase: irritability, elevated transaminases, gallbladder hydrops, sterile pyuria, uveitis, aseptic meningitis, diarrhea, obstructive jaundice, arthritis

Back 17

Kawasaki Disease

Front 18

Subacute phase: resolution of inflammatory signs/activity, thrombocytosis, coronary artery aneurysms, oligoarticular arthritis

Back 18

Kawasaki Disease

Front 19

Aneurysms in medium-sized vessels causing focal segmental necrotizing vasculitis, sx: fever, anorexia, fatigue, rashes, painful skin nodules, livedo reticularis, cutaneous ulcers, renal involvement, orchitis (esp in those with hep B infection), mononeuritis multiplex, HTN

Back 19

Polyarteridis Nodosa

Front 20

pANCA associated small vessel vasculitis causing rapidly progressive glomerulonephritis and pulmonary-renal syndrome

Back 20

Microscopic polyangitis

Front 21

Necrotizing granulomatous vasculitis of small vessels of upper and lower resp tracts adn kidney, fever, weight loss, arthralgias/myalgias, epistaxis, sinusitis, nasal deformities (saddle nose), scleritis, dacrocystitis, proptosis, cANCA positive

Back 21

Wegener Granulomatosis

Front 22

Granulomatous vasculitis of large vessels, causes arteritis in aorta and its branges leading to weak or absent pulses in upper extremities, onset: fever, weight loss, fatigue, arthritis, myalgias

Back 22

Takayasu Arteritis

Front 23

Painful recurrent oral and genital ulcers, inflammatory eye disease (uveitis, retinal vasculitis, papilledema), skin lesions (erythema nodosum) are common, positive pathergy test

Back 23

Behcet Disease

Front 24

Rash, cytopenias, hepatitis, complete heart block, SS-A or SS-B antibodies

Back 24

Neonatal SLE

Front 25

+ANA, +dsDNA, low C3/C4, antiphospholipid antibodies - associated with miscarriages, low plts, livedo reticularis, and clots

Back 25

SLE

Front 26

Most common cause of chorea

Back 26

SLE

Front 27

Minimal mesangial nephritis, mesangial proliferative nephritis, focal nephritis, diffuse segmental or global nephritis (most severe, leads to ESRD), membranous nephritis, advanced sclerosing nephritis

Back 27

6 forms of Lupus Nephritis

Front 28

Vesicular or bullous rash associated with SS-A or SS-B

Back 28

Subacute Cutaneous LE

Front 29

Libman-Sacks endocarditis associated with these antibodies

Back 29

Antiphospholipid Antibodies

Front 30

Treatment mainstay for SLE

Back 30

Anti-malarial drugs

Front 31

Heliotrope or Gottron papules, periungual changes, proximal muscle weakness, photosensitivity, dystrophic calcification of skin, subcutaneous tissue, and fascia, lipodystrophy (late), tx with steroids, elevated CPK, aldolase, or LDH

Back 31

Juvenile Dermatomyositis

Front 32

Lab dx: nucleolar or speckled ANA, SCL-70 antibodies, anticentromere antibodies

Back 32

Systemic Scleroderma

Front 33

Most common localized scleroderma in children, linear streaks in UE and LE that are dermatomal, become indurated and extend down to muscle, associated seizures, uveitis, dental defects, and facial abnormalities

Back 33

Linear Scleroderma

Front 34

Bilateral pain, occurs in late day or evening, pain awakens pt from sleep, not associated with limping/mobility problems, located in non-articular areas

Back 34

Growing Pains

Front 35

Major concern/complication of Familial Mediterranean Fever

Back 35

Amyloidosis

Front 36

Recurrent fever, responds well to steroids but decreases recurrence interval, fever with pharyngitis/adenitis, normal growth, resolves by teenage years

Back 36

Periodic Fever, Aphthous-stomatitis, Pharyngitis, cervical Adenitis (PFAPA)

Front 37

AR inheritance, gene localized to chromosome 16 produces pyrin, monthly fevers with severe abd pain, pleuritis, pericarditis, and scrotal swelling, rash, arthritis, and arthralgias can also occur, tx with colchicine

Back 37

Familial Mediterranean Fever (FMF)

Front 38

AD inheritance, fevers with abd pain, focal migratory myalgias, single or multiple erythematous patches on extremities, does not respond to colchicine, tx with NSAIDs, prednisone, etanercept, anakinra

Back 38

TNF Receptor-1 associated Periodic Syndome (TRAPS)

Front 39

Mutation in MVK gene, elevated IgD and IgA, tx with colchicine, prednisone, IVIG, NSAIDs, etanercept, anakinra

Back 39

Hyper-IgD