Rheumatology Flashcards
rheumatic dz’s are characterized by what/
autoimmunity & inflammation
rheumatic dz’s DDx
active infxns
post-infectious phenomena
malignancies
rheumatic dz is characterized by what arthritis (synovitis)….
enthesitis serositis myositis vasculitis activation of reticuloendothelial system
activation of the reticuloendothelial system causes what?
lymphadenopathy
in systemic-onset JRA & SLE- HSM
chronic inflammation in rheumatic dz’s can lead to what?
growth delay & disability
what is the hallmark of rheumatic dz?
chronicity
radiographic joint findings in rheumatic dz’s
lag far behind symptoms
what is one of the MC vasculitides of CH?
Kawasaki dz
theorized pathology of Kawaski dz
abnormal immune response to some super-antigen
epidemiology of Kawasaki dz
peak age 2-3 yr rare over age 7 peaks bet. Feb & May highest incidence in Japan MCC of acquired heart dz in CH
Kawasaki dz diagnostic criteria
fever for 5+ days!!!!
presence of 4 of the following:
1. bilateral nonpurulent conjunctivitis w/ perilimbic sparing (MC)
2. changes in the oropharyngeal mucous membranes
3. extremity changes (most often swelling & redness of the hands & ft)
4. rash (>80% of CH)
5. cervical adenopathy (>1.5 cm node, 70% of CH)
illness unexplained by other dz
some of the changes in oropharyngeal membranes you might see in Kawasaki dz
injected pharynx
injected lips
dry or cracked lips
strawberry tongue
some changes in the perpheral extremities you might see in Kawasaki dz
edema or erythema
desquamation
periungual desquamation
what type of rash might you see in Kawasaki dz?
mostly truncal
polymorphic but not vesicular
acute phase of Kawasaki dz
affected CH are generally irritable
abdominal pain is common
hydrops of the GB, CSF pleocytosis, arthritis & carditis can manifest in acute phase
subacute phase of Kawasaki dz
thrombocytosis
desquamation of groin, hands & feet
coronary aneurysms (up to 20% of untreated CH)- higher risk in boys, CH w/ prolonged fever or inflammatory markers, age under 1 yr
lab work-up in Kawasaki dz
increased WBC increased ESR, CRP anemia mild increase in transaminases decreased albumin sterile pyuria CSF pleocytosis increased platelets by day 10-14
you can use an echocardiography to search for what cardiovascular complications in Kawsaki dz
myocarditis
pericarditis
evidence of coronary vasculitis
viral exanthems
measles rubella roseola adenovirus EBV
staphylococcal or streptococcal toxin-mediated syndrome
staphy scalded skin syndrome
scarlet fever
streptococcal toxic shock
difficulties w/ dx KD
no single test is diagnostic
Kawasaki dz remains a dx of exclusion
atypical Kawasaki dz- when illness does not fulfill all criteria; more common 8 yo
KD tx
- admit to monitor cardiac function
- r/o other causes of similar dz
- complete cardiac eval (EKG & echocardiogram)
- IVIG (2mg/kg)- reduces coronary aneurysm rate to s persist or recur
- aspirin
- f/u echocardiography in 6-8 wks after IVIG
aspirin tx in KD
high-dose for anti-inflammatory effect in first 24-96 hrs
low dose for anti-thrombotic activity pending f/u echocardiography for late-onset coronary aneurysms
SLE
deposition of immune complexes in tissue which activates lymphocytes, neutrophils, & complement
SLE is a multisystem inflammatory dz & may include what?
joints serous linings skin kidneys CNS
epidemiology of SLE
female predominant
median age for peds ~12 yo (uncommon before age 4 yo)
Asians>blacks>hispanics>caucasians
health disparity-Caucasians have better survival rates
peds presenting sx’s may be vague
MC findings in SLE
fever abdominal complaints malar rash &/or oral ulcers MS arthritis or arthralgias hematologic anemia, leukopenia, thrombocytopenia
crash & burn pneumonic for KD
Conjunctivitis Rash Adenopathy Strawberry tongue Hands & feet
burn (fever)
renal findings in CH w/ SLE
hematuria
proteinuria
mild hypertension
diffuse proliferative glomerulonephritis
neurologic findings in CH w/ SLE
HA deterioration of academic performance withdrawal social isolation depression *full hx & PE important in teens, screening CBC, ESR reasonable in vague complaints
more dramatic neurologic presentations in SLE
stroke
seizures
chorea
coma
hematologic findings in CH w/ SLE
thrombocytopenia, hemolytic anemia, leukopenia (together will lead to sending an ANA)
f/u studies when suspecting SLE: antiphospholipid Ab’s, lupus anticoagulant activity
cardiopulmonary findings in CH w/ SLE
increased risk of atherosclerosis & CAD
increased risk of pericarditis, endocarditis
pleural effusions
diagnostic criteria of SLE
malar rash discoid rash photosensitivity oral ulcers arthritis serositis renal d/o neruologic d/o hematologic d/o immunologic d/o anti-nuclear Ab
malar rash in SLE
fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
discoid rash in SLE
erythematous, raised patches w/ adherent keratotic scaling & follicular plugging
photosensitivity in SLE
skin rash as a result of unusual rxn to sunlight
oral ulcers in SLE
usually painless
arthritis in SLE
nonerosive arthritis involving 2+ peripheral joints, characterized by tenderness, swelling, or effusion
serositis in SLE
pleuritis or pericarditis
renal d/o in SLE
persistent proteinuria, cellular casts
neurologic d/o in SLE
seizures or psychosis
hematologic d/o SLE
hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia
immunologic d/o in SLE
anti-phospholipid Ab, anti-DNA, anti-Sm, false positive syphilis serology
anti-nuclear Ab in SLE
abnormal titer of ANA by immunofluorescence or an equivalent assay
labs for dx in SLE
ANA
CBC w/ leukopenia, anemia
complement level
ESR
checking for dz severity in SLE
anti-dsDNA Ab more specific (also Abs to Sm nuclear antigen)
antiphospholibid Abs as anti-cardiolipin Ab, lupus anticoagulant or false + RPR or VDRL over 6 months
UA & renal function in SLE
BUN
Cr
renal dz is a major component of SLE
SLE therapy
corticosteroids
cyclophosphamide-early therapy improves survival
steroid-sparing agents
non-steroidal anti-inflammatory drugs
steroid sparing agents for SLE
methotrexate
azathiprine
mycophenolate
mofetil
non-steroidal anti-inflammatory drugs for SLE
hydroxychloroquine
dapsone
challenges in CH w/ SLE
poor compliance
renal dz- 30% will go on to renal failure
neurologic complications
intercurrent infxns
what is the most prevalent chronic rheumatologic dz of CH
juvenile rheumatoid arthritis (JIA- juvenile idiopathic arthritis?)
what are the 3 types of JIA/JRA
pauciarticular (~50%) /= 5 joints
systemic onset
what is JIA/JRA
non-migratory arthropathy w/ a tendency to involve large joints or proximal interphalangeal joints lasting over chronic period of months
when is pain usually worse in JIA/JRA
morning
joint swelling w/ diminished mobility
JIA/JRA complicated by uveitis
MC treatable cause of blindness in CH
ANA-positive CH are more likely to have chronic uveitis
pauciarticular onset JIA/JRA is more common in girls or boys?
girls
what are the clinical manifestations of pauciarticular onset JRA
- arthritis of large joints (knee>ankle>wrist)
- uveitis is only systemic manifestation
- involves < 5 joints
- ANA often +
- rheumatoid factor often neg
- may have no fever/abnormalities of inflammatory markers (WBC, ESR, CRP)
- 90% have complete remission
complications of pauciarticular onset JRA
uveitis untreated may progress toward blindness
inflammation may cause bony changes that distort growth
what mild systemic complaints may exist w/ JRA
low grade fever
anemia
malaise
JRA can result in________growth
asymmetric
polyarticular onset JRA is more common in girls or boys?
girls
*> 50% have complete remission
manifestations of polyarticular onset JRA between ages 2-5 yo
- progressive involvement of joints
- symmetrical joint involvement
- uveitis
- can present w/ systemic sx’s
symmetrical joint involvement in polyarticular onset JRA
small joints of the hands & feet
ankles, wrists, knees
may cause fusion of the cervical spine
some systemic sx’s that may present w/ polyarticular onset JRA
malaise fever growth retardation anemia elevated inflammatory markers
manifestations of polyarticular onset JRA ages 10-14 yo
arthritis of rapid onset
small joints of hands & feet typically involved
adolescents that are rheumatoid-factor + probably have true adult rheumatoid arthritis
systemic onset JRA affects boy or girls more?
affects both sexes equally
manifestations of systemic onset JRA
typically present originally without arthritis systemic sx’s
dx often made after elimination of other dz’s, as sx’s become evidently chronic
what are some of the systemic sx’s of arthritis
fever- often high, regular (daily/twice daily)
rashes- salmon pink, macular rash
HA, HSM, lyphadenopathy
pleural & pericardial effusions
arthritis in systemic onset JRA may affect what joints?
may be of any joint
wrists, knees, ankles most typical
may occur in hands, hips, cervical spine & temporomandibular joints
lab findings in systemic onset JRA
leukocytosis thrombocytosis anemia elevated ESR AST/ALT elevation hypoalbuminemia elevated globulin level
complications of systemic onset JRA
macrophage activation syndrome- uncontrolled proliferation of lymphocytes & macrophages
prone to sequelae of chronic systemic inflammation- growth retardation, osteoporosis
what is dermatomyositis?
inflammatory dz of muscle & skin
what are the clinical manifestations of dermatomyositis
weakness in proximal m. distribution tenderness of muscles absent DTRs flexion contractures & m. deformities rash
describe the rash found in dermatomyositis
heliotrope rash of upper eyelids (red-purple color), sun sensitive
Gottron’s papules
what are Gottron’s papules
papules found on the extensor surfaces of knuckles, elbow, knees
start as erythema, progressing to scales
lab findings in dermatomyositis
elevated muscle-source enzymes
-CK
-aldolase
muscle bx
tools for diagnosing dermatomyositis
labs
electromyography
MRI
tx of dermatomyositis
PT
meds- steroid anti-inflammatories, methotrexate, refractory cases: IVIG, cyclosporine
what can you use meds wise in tx of refractory dermatomyositis?
IVIG
cyclosporine
what meds can you use to tx dermatomyositis?
steroid anti-inflammatories
methotrexate
