Rheumatology 3 Flashcards
juvenile idiopathic arthritis (JIA) is an umbrella term for chronic arthritides in patients __ years old or less
16
JIA must involve at least __ joint and last for
__ weeks
1 joint
6 weeks
what are the subtypes of JIA (6)
- oligoarticular 2. polyarticular (sero pos, sero neg) 3. systemic onset 4. psoriatic 5. enthesitis-related 6. undifferentiated
JIA is likely caused by __ increase with __ cells and formation of __
synovial fluid increase with inflammatory cells and formation of panes
what is another name for oligoarticular JIA
pauciarticular JRA
oligoarticular haș __ joint involvement and must involve fewer than __ joints
asymmetric 4
în oligoarticular JIA, ESR/CRP might be slightly elevated, but __ is positive in 60% of pt’s
ANA
oligoarticular JIA increases risk for __, so regular __ exams must be done
uveitis slit-lamp
polyarticular JIA must involve at least __ joints
5
in polyarticular JIA, CRP may be normal, but __ is usually very elevated
ESR
what does seropositive polyarticular JIA mean
positive RF
seropositive polyarticular JIA resembles adult __; seronegative polyarticular JIA will not have any __ features
RA extraarticular
systemic onset JIA (SOJI) is also called
Still’s Dz
2 common clinical manifestations of SOJIA/Still’s
- quotidian fever 2. salmon-colored evanescent macular rash
SOJI/Stills mc affects
trunk, extremities
what can be a fatal complication of SOJIA/Still’s
pericarditis or MAS (macrophage activation syndrome)
what might labs show for SOJIA/Stills
leukocytosis very high ESR elevated ferritin
macrophage activation syndrome (MAS) is a complication of SOJI and can cause
fevers coma shock DIC -> immune system going crazy -> up to 50% mortality
psoriatic arthritis is associated w. what 5 clinical manifestations
DIP synovitis dactylitis nail pitting psoriatic rash acute anterior uveitis
what 3 JIA conditions increase risk for uveitis
oligoarticular psoriatic enthesitis-related
psoriatic arthritis increases risk for
uveitis
enthesitis-related JIA is characterized by __ tenderness
sacroiliac
what genetic factor is associated w. enthesitis related JIA
HLA-B27
enthesitis ininflammation of the
tendons or ligaments -> ex kid w. swollen achilles tendon
enthesitis-related JIA includes pt’s w.
juvenile-onset spondylitis reactive arthritis IBD arthritis
enthesitis-related JIA is a __ diagnosis
clinical
JIA are all __ diagnosis
clinical
labs can be supportive
first step in all JIA is
pediatric rheum referral
what are the 2 mainstays of therapy in JIA
NSAIDS
intraarticular joint injxn
SOJI pt’s need __ initially for symptom control
steroid
in addition to NSAIDs and intraarticular joint injxns, __ might also be a drug used in JIA tx
DMARDs → MTX
what JIA condition needs aggressive tx w. DMARD
seropositive polyarticular JIA
which JIA has the highest mortality rate
SOJIA
what are the two forms of systemic sclerosis (scleroderma)
limited
diffuse
systemic sclerosis/scleroderma is caused by
fibrosis of the skin and other organs
CREST syndrome is a type of __ scleroderma
limited
what does CREST stand for
calcinosis cutis
raynaud phenomenon
eesophageal dysmotility
sclerodactylyl
telangiectasia
what does CREST stand for
calcinosis cutis
raynaud phenomenon
eesophageal dysmotility
sclerodactylyl
telangiectasia
what 2 groups are esp at risk for scleroderma
aa
choctaw Native American
what does this make you think of: widespread proliferative/obliterative vasculopathy of small and medium arteries and capillary refraction
scleroderma
what is the first sign of scleroderma
Raynaud phenomenon
initial skin manifestations of scleroderma
edema
pruritus
late skin manifestations of scleroderma
thick, hide like skin
tight skin
hyperpigmentation/depigmentation
ulcers
telangiectasis
what is watermelon stomach
GI manifestation of scleroderma
pulmonary manifestation of scleroderma
fibrosis → pHTN
cardiac manifestations of scleroderma
pericarditis
diastolic hf
what do calcinosis cutis and siccca syndrome make you think of
scleroderma
what is scleroderma renal crisis
sudden onset of malignant HTN → renal failure → death
up to 80% of scleroderma pt’s have
pericardial involvement
can scleroderma also cause thromboembolic dz and affect MSK, neuromuscular, and GU systems?
yes
2 GU manifestations of scleroderma
ED
dysperiunia in women → fibrotic vaginal opening
over 95% of scleroderma pt’s have (+) ANA
what (+) lab value is related to poor prognosis w. scleroderma
anti-SCL-70
imaging for lung involvement in scleroderma
high resolution CT
baseline PFTs
what test to order for cardiac involvement in scleroderma
ECHO
why would you order EGD in scleroderma
Barrett’s esophagus
score of __ or above is diagnostic for criteria for scleroderma
9
can scleroderma be dx’ed w.o the criteria
yes
tx for scleroderma targets
affected organs
tx for skin sx in scleroderma
glucocorticoids, MTX, cyclophosphamide, IVIG
tx for raunaud phenomenon in scleroderma
CCB → nifedipine
__ agents are used for GI symptoms in scleroderma
pro kinetic agents → reglan or erythromycin
tx for lung sx in scleroderma
diuretics
+/- digoxin
tx for renal sx in scleroderma
ACEI
tx for ED in scleroderma
long acting Cialis (tadalafil)
leading cause of mortality in scleroderma
pulmonary fibrosis
what do you think when you see inflammatory dz w. autoabs affecting every organ
systemic lupus erythematosis (SLE)
lupus incidence is highest in
child bearing females
rf for SLE
estrogen!
smoking
constitutional SLE sx (5)
fever
fatigue
anorexia
malaise
wt loss
skin manifestations of SLE
acute cutaneous lupus erythematosis (ACLE)
ACLE looks like
butterfly rash
ACLE spares __
and is triggered by __
nasolabial folds
sun
what do you think when you see maculopapular lesions in a photosensitive distribution on any part of the body
ACLE
what is SCLE
subacute cutaneous lupus erythematosis (SCLE)
what do you think when you see scaly erythematous papule
SCLE
most photosensitive SLE rash
SCLE
what is CCLE
chronic cutaneous lupus erythematosis
mc subtype of CCLE
discoid lupus
what do you think when you see raise, erythematous plaques w. a scale or ring around the lesion
CCLE
what MSK sx is common in SLE
arthralgia
what is renal involvement of SLE called
lupus glomerulonephritis
vascular sx of SLE
raynaud
vasculitis
what does palpable purple petechiae make you think of
vasculitis in SLE
mc cardiac manifestation of SLE
pericarditis
what does “wart-like” pericardial lesions make you think of
cardiac symptom of SLE
what ocular sx is very common in SLE
dry eye (keratoconjunctivitis sica) → secondary Sjorgen’s
what lab value is always positive in SLE
ANA
besides ANA, what other lab values are associated w. SLE
anti-dsDNA
anti-SM
antiphospholipid abs in SLE can cause false positive for
syphilis
what organ involvement do you not see in drug induced lupus
renal
CNS
T/F drug induced lupus is just in common in women as men
T
name 3 drugs that are associated w drug induced lupus
procainamide
hydralazine
isoniazid
→ lots more
do lab abnormalities and sx go away when drug is removed in drug-induced lupus
yes
tx considerations for SLE
exercise
smoking cessation
sun protection
healthy diet
prevention of glucocorticoid-induced osteoporosis
utd on vaccines
+/- antimalarials (hydroxychloroquine)
tx for cutaneous lupus
topical corticoids
+/- systemic meds → hydroxychloriquine
tx for MSK SLE
NSAIDs/Tylenol
+/- hydroxychloriquine
tx for serositis SLE
NSAIDs
+/-steroids and hydroxychloriquine
test to order before tx of renal manifestation of SLE
renal bx
4 important drugs in SLE
glucocorticoids
hydroxychloriquine
MTX
4 important drugs in SLE
glucocorticoids
hydroxychloriquine
MTX
what do you think of when you see ai myopathy characterized by proximal muscle weakness
polymyositis
in SLE, pt’s w. __ involvement have the poorest prognosis
renal
polymyositis pt’s have __ abs
anti muscle:
MSAs
MAAs
muscle weakness in polymyositis is __,
__,
and __
gradual
progressive
symmetric
weakness in polymyositis affects __ first
legs
most pt’s w. polymyositis will have elevated __;
__ may also be (+)
CK
ANA
gold standard for polymyositis dx
muscle bx
what do you think of when you see inflammatory infiltrate on muscle bx
polymyositis
how can EMG be helpful in polymyositis
shows early muscle involvement → show you where to bx
how can MRI be helpful in polymyositis
areas of muscle inflammation → help decide where to bx
can you dx polymyositis w.o muscle bx
yes
what clinical symptoms can be diagnostic of polymyositis
proximal muscle weakness → can be more helpful/used more than enzymes
abnormal muscle enzyme labs
no better explanation
initial tx for polymyositis
steroids
→ usually combined w. another immunosuppressant
do pt’s w. polymyositis need to avoid exercise
no! it helps → PT recommended
5 clinical domains of psoriatic arthritis
peripheral
axial
enthesitis
dactylitis
skin and nail
psoriatic arthritis usually develops
after or at same time as psoriasis
genetic component of psoriasis
HLA
psoriatic arthritis pt’s will have morning pain/stiffness __ min
more than 30
polyarhtritis can be __
or __, but
__ is mc
symmetric or asymmetric mc: symmetric
4 clinical presentations of psoriatic arthritis
symmetric vs asymmetric
distal → DIP
arthritis mutilans
spondyloarthritis → C-spine involvement
3 periarticular manifestations of psoriatic arthritis
enthesitis
tenosynovitis
dactylics
what is dactylics
sausage digit
in psoriatic arthritis, nails can show
pitting
ridging
oncholysis
psoriasis can be __, so it is important to do through exam in pt’s you suspect psoriatic arthritis
hidden
what do you think of when you see “pencil-in-cup”deformity
psoriatic arthritis → damage to DIP and PIP
what criteria is used for psoriatic arthritis
CASPAR
tx for mild psoriatic arthritis
NSAIDs → naproxen, Celebrex
tx for severe/unresponseive to NSAIDs psoriatic arthritis
DMARDs → MTX, sulfasalazine
TNF inhibitors → Etanercept
what do you have to screen for before prescribing immunosuppressing drugs in psoriatic arthritis (or any rheum condition)
latent TB
what drug to avoid in psoriatic arthritis
PO steroids → can cause flare of pustular psoriasis