Rheum and MSK Flashcards
Diagnosis of JIA
Onset before age 16
Presence of inflammatory arthritis in at least one joint for >= 6 weeks
Risk factor for uveitis
Female
oligo articular
age < 7
ANA +ve
Systemic onset JIA presenting symptoms
Fevers (often predictable timing)
Rashes
Serositis
Lymphadenopathy
HSM
joints
Common complications JIA
Uveitis
Tmj complications
Macrophage activation syndrome
How to assess uveitis
Refer to optho for slit lamp
Screen every 3 months in high risk children
Symptoms of acute uveitis
Conjunctival erythema
Photophobia
Eye pain
Macrophage activation syndrome signs and symptoms
Rapid development
Constant fevers
HSM
lymphadenopathy
Hepatic dysfunction
Encephalopathy
Easy bruising/ bleeding
Macrophage activation syndrome lab findings
Hyperferritinemia (thousands)
Cytopenias
Hypertriglycerides
Coagulopathy
Elevated LDH and liver enzymes
Sharp drop in ESR with higher CRP
Bone marrow: hemophagocytosis
Macrophage activation syndrome treatment
Hugh dose steroids
Interleukin inhibitor (anakinra)
genu varum vs. valgus
valgum/valgus - goes in - knees in
varum/vargus - air - knees out
varus <2 can be normal
Blount Disease
tibia rotates
+ lateral thrust sign (sharp angulation outwards at the knee)
on differential for bowing of the legs before age 2
think about this if sharp bowing at the knee
usually asymmetric
widening of growth plates on XR
leg bowing referral to ortho
age over 2
sharp angle
lateral thrust
methaphyseal/diaphyseal angle >11
Elbow ossification centers
CRITOE (2,4,6,8,10,12)
- capitellum
- radial
- internal
- trochlea
- olecranon
- external
Nursemaid’s elbow (radial head subluxation)
radial head normally anchored to ulna by annular ligament
in nursemaid’s, the proximal annular ligament subluxes off of the radial head
exam: cannot supinate (hand up)
reduction: supination with pushing onto radial head
posterior elbow dislocation
mechanism: falling backwards onto arm with elbow extended
look for olecranon fractures
treatment: immediate reduction with longitudinal traction to forearm with upward pressure on distal humerus
olecranon fracture
mechanism: falling ont o flexed ot extended elbow
treatment
- non-displaced = non-operative in long arm splint or cast
- displaced = ORIF
condylar fracture
need AP, lateral and oblique views of elbow
high risk for nonunion and AVN
treatment
- non-operative if <2mm displaced
- operative if >2mm
avascular necrosis
Management
- bisphosphonates (unless associated with LCP)
12 yo F with pain in her right leg with running and squatting. X ray shows soft tissue swelling over right tibia tubercle, and the tubercle appears fragmented. What is this? Management?
Osgood Schlatter Disease (OSD)
- microavulsion fracture of patellar tendon onto tibial tuberosity
- M>F, bilateral in 30%
- localized pain with running, jumping, kneeling and focal tenderness at tibial turberosity
Treatment:
- activity modification
- stretching
- PT to strengthen surround muscles
- DONT CAST
Sinding-Larsen-Johansson Disease
Sinding-Larsen-Johansson Disease (SLJD) is like osgood sclatter but patellar tendon insertion to inferior patellar pole
Complex regional pain syndrome diagnostic criteria
Need 2/4:
1. Presence of initiating noxious event or cause of immobilization
2. Continuing pain, allodynia, or hyperalgesia with which pain is disproportionate to inciting event
3. Evidence of edema, changes in skin blood flow abnormal motor activity in the region of the pain.
4. Exclusion of alternative diagnosis
more common in teen females
do CBC, CRP, ESR - should be normal
treatment: pain goals, CBT, PT/OT
CPS NAT Fracture pearls
high risk fractures
- rib
- metaphyseal
- humerus <18 mo
- femur non ambulatory
- spiral/oblique
Imaging
- skeletal survey in all children < 2yo with fracture. If initial negative, repeat in 2 weeks
Labs
- add ca, po4, alp, urine
- consider pth, vit d, serum copper, ceruloplasmin
Calcaneal apophysitis
AKA Sever disease
- common in kids (more common than Achilles tendonitis)
- common in active kids
- often bilateral
presentation
- heel pain with running and jumping
- tenderness over posterior heel
- pain with squeeze test
Tx: NSAIDs, ice, PT, activity modification
Lupus common antibodies
ANA + (sensitive)
dsDNA (Specific)
anti-smith (specific)
anti-Histone (drug induced lupus)
multi system involvement in lupus (MD SOAP BRAIN)
Malar rash
Discoid rash
Serositis
oral ulcers
arthritis
photosensitivity
blood (hemolysis, low WBC, low plt)
renal (proteniuria, casts, hematuria)
ANA +
immunologic (dsDNA, smith, lupus anticoagulant)
neurologic (psychosis, seizures)
Labs to follow in lupus
- low C3/C4, complement (lower in worse disease)
- anti ds DNA
- anti phospholipid ab
Classic rash in lupus
cheeks
sparing nasolabial folds
photosensitive
what is an important test to do in new lupus?
urinalysis (if urine involvement, admit them)
Investigations in lupus
- CBCd
- CRP/ESR
- ferritin
- ANA (must be positive to be lupus)
- Anti dsDNA
- extractable nuclear ab (anti-sm, anti-rnp, anti-ro, anti-la)
- antiphospholipid antibodies (increased risk for clotting)
- C3/C4 (low)
- urinalysis
once diagnosied:
- echo
- PFT
- bone density
- TSH, anti TPO
-consider macrophage activation syndrome labs
(ferritin, trigs, fibrinogen, d-dimer)
Meds for lupus treatment
plaquinil for everyone
- aka hydroxychloroquine
- check eye exam annual
steroids when unwell
lupus emergency complications
DIC/clotthing
- pulm hemorrhage, stroke, PE
- due to antiphospholipid antibody syndrome
MAS
- type of HLH
- excessive inflammation and cytokine storm
- fever, rash, HSM
- high CRP, ESR (may be lower than baseline)
Catastrophic Antiphospholipid Syndrome
- Evidence of involvement of three or more organs, systems, or tissues
- Development of manifestations simultaneously or within 1 week
- Confirmation by histopathology of small-vessel occlusion in at least 1 tissue
- Lab confirmation of presence of APLs
Systemic JIA (aka Still’s disease)
- daily spiking fever (quotidian) x 2 weeks
- erythematous, salmon colored rash (during fever)
- lymphadenopathy
- HSM
- serositis
- arthritis (may appear months later)
Vacterl features
Diagnostic: > 3 ___ anomalies
Vertebral defects (hemivertebrae, butterfly, rib anomalies, sacral agenesis, scoliosis, tethered cord)
Anal atresia (others: imperforate anus, fistula)
Cardiac anomalies (VSD, ASD, TOF)
TEF
Esophageal atresia
Renal anomalies (renal agenesis/dysplasia, horseshoe kidney, ureteral anomalies)
Limb (radial defects): underdevelopment/agenesis of thumb, radial bone
Legg Calves Perthes
- idiopathic AVN of femoral head in young kids age 3-10 yr
Presentation
- Trendelenburg gait
- Pain, if present, is usually activity related and may be localized to the groin or referred to thigh/knee region
- Decreased internal rotation
- Decreased abduction
- Thigh and buttock atrophy
Imaging
- Pelvic X-ray (AP + frog-leg): morphologic changes (“crumbled”) of the proximal femoral epiphysis
- MRI is the best test for early Perthes
- Bone scan can show decreased perfusion to femoral head early on
MR is most sensitive
XR is first line
Anti TNF side effects (ex. infliximab)
- Injection or infusion side effects
- Infections
- Reactivation of TB (disseminated)
- Pancytopenia
- Neurological (MS like syndrome)
- Autoimmunity
- Malignancy
- Don’t give live vaccines to kids on immunosuppressants !!!
PFAPA
Onset <5yrs
Fever (recurrent, at least 5 days, regular occurence q3-6 weeks)
Aphthous stomatitis and/or pharyngitis (with or without cervical adenitis)
High inflamm markers during episodes
Well between episodes
Resolves at 5-8yrs
Treatment: Prednisone single dose 1-2mg/kg and tonsillectomy (cureable)
No genetic associations
Familial mediterranean fever
Onset <20yrs
Short duration of fever 1-3days
Irregular occurrences
Present with erysipeloid erythema rash, monoarthritis, polyarthritis or sterile peritonitis
HIGHEST RISK OF AMYLOIDOSIS (use colchicine to help reduce amyloids and avoid this risk)
Genetic testing - familial
TRAPS - TNF associated periodic fever syndrome
recurrent long fevers (1 week to 1 month long)
salmon or evanescent rash
systemic JIA
Atypical KD criteria
Fever >5d but <4 features AND CRP >30 or ESR >40
WITH + echo findings OR 3 or more lab features
Anemia
Elevated plts
Low albumin
Elevated ALT
Sterile pyuria
Elevated WBC
risk factors for coronary artery aneurysm in KD
Prolonged fever
<1yr and >9yr
Male
Hypoalbuminemia
Thrombocytopenia
Prominent infllam markers
Child with recurrent 3-4d episodes of fever, abdominal pain, arthritis and erysipeloid rash, family history of renal disease. What is this? What do you treat with?
- FMF
- colchicine (prevent amyloidosis)
CAP - Cryopyrin-Associated Periodic Fever
periodic fever syndrome assocaited with hearing loss
Ewing sarcoma classic findings
EEWWW - moths
EEWWW - onions (periosteal rxn with new bone formation)
EEWWW - axial
osteosarcoma common findings
sunburst sign on Xray (radiating calcifications around the bone) - think O the Sun!
methotrexate side effects
Immunosuppression
Transaminitis
GI upset
Oral ulcers
Pulmonary toxicity (rare and usually only upon initiation of methotrexate)
Teratogenic
Avoid alcohol (exacerbates transaminitis)
No live vaccines
Pulmonary toxicity
Tricky lab test consistent with lupus
positive RPR suggestive of lupus
What is the most clinically significant complication of neonatal lupus?!?!
HEART BLOCK!! Can also get cardiomyopathy and valve regurg and effusions - Screen with serial echos from weeks 18-30, treat with dexamethasone from time of diagnosis to delivery
Spondylosis
Defect in the pars interarticularis, the segment of bone connecting the superior and inferior articular facets in the vertebra
Mechanism: repetitive hyper-extension stresses (think gymnast, football, wrestlers)
most commonly at L5
most common cause of back pain in adolescents
Discomfort with spinal extension
JIA criteria
JIA = chronic arthritis (single joint >/= 6 weeks) with onset before age 16
Lyme disease
Presentation:
Erythema migrans - 89%
Rash that appears at the site of the tick bite, usually within 7-14 days after bite
Can be accompanied by fever, fatigue, headache, neck pain, arthralgia, myalgia
Arthritis - 7%
Most common clinical manifestation of systemic untreated lyme
Typically a knee that is ++ swollen but not that painful
Facial nerve palsy (typically bilateral)- 3%
Aseptic meningitis - 1%
Carditis - 0.5%
Diagnosis:
Clinical - Serum antibody testing is highly sensitive AFTER 4 weeks
Treatment:
Early localized - Doxycycline x 10 days for children > 8 yo
Alternate children < 8 yo - Amoxicillin or cefuroxime x 14 days
Neuro Lyme: IV ceftriaxone, IV penicillin, or PO doxy 2-3 weeks
juvenile dermatomyositis terrible D’s
Always ask about the dangerous Ds - dysphagia, dyspnea (ILD or Resp wall weakness), dysphonia - need to be admitted and kept NPO
