Mis: Week 3 - Seronegative Arthropathies

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3 RA related disorders

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JIA
adult onset still's disease
SS

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most common form of arthritis in children

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JIA

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JIA incidence, age of onset, and needed lenth of persistent arthritis:

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JIA incidence of 10-20/100,000/year, age of onset of <16 yo, and needed lenth of persistent arthritis of > 6 mo

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DD of JIA (there are 7 + many, many others)

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leukemia, septic joint, vira illness, metabolic storage disease, rheumatic fever, periarticular disease, juvenile SLE

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JRA is current or past way of lookin at juvenile arthritis?

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PAST (now we use JIA classificatio scheme)

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JIA - current umbrella term for all childhood arthritis - divided into 6 catagories... NAME THEM:

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systemic, polyarthritis, oliogarthritis, psoriatic, enthesitis related, undifferentiated (arthritis)

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Polyarthritis is equal to or greater than X joints; can be X negative or positive (imp due to diff tx)

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Polyarthritis is equal to or greater than 5 joints; can be RF negative or positive (imp due to diff tx)

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Oligoarthritis is egual to or less than X joints; can be classified as X or X

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Oligoarthritis is egual to or less than 4 joints; can be classified as persistent (alway less than 4) or extended (equal to or more than 5 joints after 6 mo)

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systemic JIA - affects what age of children

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younger
as young as 6-7 mo
peak of 1-6 yo

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systemic JIA - is associated w/clinical sx (5)

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*daily quotidian fevers for at least 2 weeks*
evanescent, non-fixed, pale pink, maculopapular, blanching rash usl when febrile
generalized LAD
hepatosplenomegaly
serositis

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systemic JIA - is usl seronegative for?

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RF/ANA negative

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systemic JIA usl has uveitis? T/F?

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FALSE

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typically polyarticular systemic JIA involves what sized joints?

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both large and small

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is systemic JIA self-limited

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umm... YES... frequently

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systemic JIA = powerpoint description:

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very ill, young, lotsof inflammation, may not have arthritis, no diagnostic test availbale

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JIA polyarthritis = is more common in what sex?

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female

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JIA polyarthritis - involves what sized joints?

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small and large

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is uveitis common? are systemic symptoms common?

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NO and NO

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RF+ (usl over 8 yo) prognosis?

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worst

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polyarthritis = powerpoint description

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young, systemically well, symmetric arthritis in many joints, guarded prognosis

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what is the most common JIA?

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JIA oligoarthritis

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JIA oligoarthritis - described as symmetric or asymetric?

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asymetric

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JIA oligoarthritis - more common in which sex?

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female

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JIA oligoarthritis - peak age of incidence?

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1-5 yo

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JIA oligoarthritis - seronegative?

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often ANA +

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JIA oligoarthritis - uveitis?

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30-50%

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JIA oligoarthritis - % of extended?

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50%

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JIA oligoarthritis - best articular outcome?

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persistant JIA oligoarthritis

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JIA Psoriatic Arthritis - peripheral and asymmetric?

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yes

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JIA Psoriatic Arthritis - needs at least 2 of:

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dactylitis, nail pitting or onycholysis, psoriasis in first degree relative

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JIA Psoriatic Arthritis - X% develop sacroiliitis; X% have uveitis

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JIA Psoriatic Arthritis - 40% develop sacroiliitis; 20% have uveitis

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JIA Enthisitis Related - have 2 of the following (4):

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SI tenderness, +HLA-B27, male at least 6 yo, acute anterior uveitis (uveitis in 25%)

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JIA ENthisitis Related - seronegative?

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yes

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Tell me a bit about screening eye exams in JIA...

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- every 3-12 mo depending on JIA type, age of onset, ANA positivity
- there is a high risk of blindness

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5 goals for treatment of JIA

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amelioration of dis signs/sx
suppression of inflammatory response
maintenance and/or improvement of strenght and ROM
early, aggressive tx

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Tx of JIA

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corticosteroids - prefer intra-articular injections
NSAIDS
DMARDS
Biologics

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Adult Onset Still's Dis - describe symptoms

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systemic onset JIA in adults, high fevers, arthritis, salmon pink-maculopapular-evanescent rash, koebner's phenomenon (rash on friction areas)

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Diagnostic Criteria fo Adult Onset Still's Dis

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ALL OF:
fever over 102.2
arthralgia/arthritis
RF < 1:80
ANA < 1:100
ANY 2 OF:
WBC over 15,000/mm3
Still's rash
pleuritis/pericarditis
hepatomegaly or splenomegaly or generalized LAD

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Sjorgen's Syndrome - description:

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immune-mediated disorder of EXOCRINE glands

primary or secondary

sicca symptoms (dryness of eyes/mouth)

autoAb to Ro/La

focal lymphoid infiltrates (CD4s)

fatigue, skin rashes, vasculitis, ILD

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Characterisitcs of Seronegative Spondyloarthritis (6)

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SI and spinal joint invlvement

peripheral arthritis

enthesopathy

common spectrum of extra-articular features (esp mucocutaneous, ocular)

familial clustering

HLA-B27

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7 spondyloarthitis conditions:

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AS
undifferentiated
reactive
juvenile
associated w/IBS
psoriatic
acute anterior uveitis

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AS-characteristics of back pain:

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onset of discomfort before 50
insidious onset
duration more than 3 mo
associated w/ morning stiffness
improvement w/exercise

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AS-presenting featuers:

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onset at 26
inflammatory back pain
sacroiliitis most common initial feature
minority present w/oligoarthritis or enthesitis (heel)
fatigue, fever, weight loss

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AS - more common in which sex?

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males

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enthesopathy - description:

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bony insertin of ligaments/tendons

common sites w/greatest physical stress: achilles tendon, plantar fascia, quads tendon

infiltration of macs and T lymphs

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Inflammatoy enthesitis involves:

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periosteal new bone formation & subchondral bone inflammation and resorption

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enthesitis of spine -

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occurs at capsular and ligamenous attachment

involvement at bony attachments and interspinous and paravertebral ligaments

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AS - shows thoracis and lumbar vertebra "X"?

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squaring, osteopenia, and ossification

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Acute Anterior Uveitis - describe me!

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most commone extraarticular complication

unilateral, asynchronous w/flares

pain, redness, lacrimation, photophobia, blurred vision

untreated can lead to vision loss

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Tx for segoneg spondylarthropathies?

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exercise
NSAIDs
DMARDs
Biologics
short term antibiotics for Reiter's syndrome
surgery

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Psoriatic arthritis: radiographics characteristics:

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erosive arthritis (usl asymmetric)
pencil in cup deformity
arthritis mutilans
bony ankylosis
spurs/perosteal rxn
non-marginal asymmetric syndesmophytes
asymmetric sacroiliitis

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psoriatric arthritis: cutaneous and other manifestations:

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psoriasis
erythroderma
nail pitting
onycholysis
conjunctivitis/iritis
valvular heart disease

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psoratic arthritis: musculoskeletal characters:

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asymmetrical oligo or monoarthritis
lower extremities
dactylitis
tenosynovitis
enthesitis
sacroilllitis
spondylitis

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Psoriatic arthritis - is usl RF neg/pos?

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RF neg

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Arthropathy associated w/IBS - description:

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usl w/UC or Crohn's Disease

usl migratory, asym, lower extremity

10% sacroiliitis

flares w/bowel disease

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Rish factors for chronic arthritis:

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post=chlamydia ReA
HLA-B27
male gender

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extra-articular manifestations:

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osteoprosis
pulmonary (upper lobe fibrosis, rigidity of chest wall, DOE)
aortitis
cord compression w/spinal disease
altantoaxial subluxation (often C5-C6 interspace)

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reactive arthritis = after GI or GU infections with?

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chlamydia, yersinia, salmonella, shigella, campylobacter

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Reiter's is?

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urethritis, conjunctivitis, arthritis

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reactive arthritis begins how long after infection?

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2-4 weeks, but not more than 6

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what is followed by conjunctivitis in 2-3 days in reactive arthritis?

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urethritis/cervicitis/vaginitis

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reactive arthritis articular manifestations (late)

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inflammatory arthritis
asymmetrical, less than or equal to 4 joints
knees and ankles most common
dactylitis
low back pain, SI joint dis, enthesitis
usl self-limited over 3-12 mo

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what % of reactive arthritis have relapses?

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15%

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skin manifestations of reactive arthritis:

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thick, crumbling nails
circinate balanitis - moist shallow ulcers around penis
keratoderma blennorrhagicum - palms and soles - waxy, papular lesoins (psoriasis-like)

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what % of reactive arthritis have anterior uveitis

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20%