Rheumatology Flashcards
What is the most common chronic rheumatological disease in children?
JIA
- Fixed flexion deformities
- NOT all swollen joints are JIA
Although it is not completely understood what is suspected to be the cause of JIA?
Genetics susceptibility plays part.
Immune response
Pro-inflammatory markers i.e TNF, interleukin 1 & 2.
Presence of antibodies.
How does JIA present?
Arthritis for at least 6 weeks
Morning stiffness or gelling (joint fluid getting thicker after periods of inactivity)
Irritability or refusal to walk in toddlers
School absence or limited ability to participate in physical activity
Rash /fever
Fatigue
Poor appetite/wt loss
Delayed puberty
What is the difference between transient synovitis and reactive arthritis?
Transient synovitis-limping, not unwell, Hx of runny nose/cough, settles quickly
Reactive arthritis-similar as above but takes longer to settle-Strep throat infection
What are the differential diagnosis of JIA?
Septic arthritis
Osteomyelitis
Transient synovitis
Malignancies i.e lymphoma, neuroblastoma, bone tumours
Recurrent haemarthrosis
Vascular abnormalities
Trauma
What are the signs of JIA?
- Swelling:periarticular soft tissue edema/intraarticular effusion/hypertrophy of synovial membrane
- Tenosynovitis (swollen tendons)
- Pain
- Joint held in position of maximum comfort
- Range of motion limited at extremes.
The ILAR classification shows the mutual exclusivity of the subtypes: What are these categories?
a) Psoriasis or a history of psoriasis in the patient or first-degree relative.
b) Arthritis in an HLA-B27-positive male beginning after the sixth birthday.
c) Ankylosing spondylitis, enthesitis-related arhtiris, sacroiliitis with inflammatory bowel disease or acute anterior uveitis or a history of one of these disorders in a first-degree relative.
d) The presence of IgM rheumatoid factor and at least two occasions at least 3 months a part.
e) The presence of systemic JIA in the patient
RF, Rheumatoid factor
How does Oligoarthiric/Pauciarticular JIA present?
<5 joint
Large joints, no symmetry
Early childhood onset is more common (Mostly girls between 1-5 years old)
Late childhood onset (Mostly boys over 8 years old)
Oligoarthiric/Pauciarticular JIA: What is the difference between presentation of children with early onset compared to late onset (think joint involved, tests etc)?
Early onset:
- Can develop irdocyclitis (uvititis)
- Test +ve for ANA
- Knees, ankles, hands & wrists
- NO hip involvement
Late onset:
- Test -ve for ANA
- No extra articular manifestation
- Hip involvement
How does polyarticular JIA present?
- 5 or more joints
- Few or no systemic manifestations of disease
-Bothe seropositive and seronegative types common in girls > boys - Onset can be acute but mostly insidious
- Large fast growing joints are mostly affected
In what age group is seropositive (RF positive) more common compared to seronegative?
Seropositive=More common in children>8
Seronegative=More common in children <5
In what type of JIA is TMJ injury common?
Polyarticular
- Leads to limited bite & micrognathia
What systemic symptoms can present in polyarticular JIA even though they are rare?
Fever, slight hepatosplenomegaly, lymphadenopathy, pericarditis & chronic uveitis
Enethesitis related arthritis (ERA) JIA is inflammation of the enthesis along with arthritis: How does it present?
Enthesitis related JIA should have 2 of:
- Onset of polyarthritis/oligoarthritis in a boy >8 years old
- HLA B27 positivity
- Acute anterior uveitis
- Inflam spinal pain
- Sacroiliac joint tenderness
- FH of enthesitis related JIA
Psoriatic JIA (chronic arthritis & definite psoriasis is evident) should have 2 of what characteristics?
- FH of psoriasis
- Dactylitis (finger or toe inflam)
- Onycholysis (nail pitting)
Is Psoriatic JIA HLA B27 +ve?
All are HLA B27 +ve
How does Systemic JIA present?
Accounts for 5-15% of JIA
Unwell
Arthritis
Intermittent fever>2 weeks
Salmon pink erythematous rash
Generalized lymphadenopathy
Serositis
Hepatomegaly/splenomegaly
High inflammatory markers
What musco-skeletal screen can be used in suspected JIA?
pGALS musco-skeletal screen
What investigations can be done in suspected JIA?
Labs
Plain x ray
US (effusions)
MRI with contrast (confirms diagnosis-inflam of synovial membranes)
How is JIA managed?
Pharmacologic management consisting of NSAIDs, disease-modifying antirheumatic drugs (DMARDs), biologic agents,and intra-articular and oral steroids
Nutrition, particularly to address anemia and generalized osteoporosis
Physical therapy to relieve pain and to address range of motion, muscle
strengthening, ADL, and conditioning exercises
Occupational therapy, including joint protection, a program to relieve pain, range of motion, and attention to ADL
What are the advantages of intraarticular steroids (IAS)?
IAS highly efficacious
Remission>6 months 84%
Greater success in oligoarticular JIA
Safe and effective & No long term side effects
What is the DMARD of choice in JIA and how and when are they used?
METHOTREXATE
- Poor response to IAS in oligoJIA
- Should be used early for good outcome
- Most given subcutaneously
- Blood monitoring needed
When should biologic agents be given?
Failure to respond to DMARD (Add on if after 3-4 months don’t go into remission)
Anti TNF agents commonly used
Good safety profile
Why is uveitis screened for in all children diagnosed with JIA?
Uveitis associated with JIA-If untreated can progress to chronic uveitis
Early detection prevents complications
All children diagnosed with JIA undergo eye screening
In what condition is uveitis more common?
Uveitis more common in ANA positive oligo JIA
How can uveitis present?
< 5 years=more at risk as often do not report any symptoms
Rarely symptomatic
Red eyes, headache, reduced vision.
Untreated=complications=Cataracts, glaucoma and blindness
Uveitis is examined via a slit lamp and all JIA patients are to be seen within 6 weeks of diagnosis as they are high risk: How is Uveitis managed?
Initially topical steroids to reduce inflammation
More severe need systemic steroids
Poor response to steroids=DMARD and biologics
Early detection and treatment-prognosis good.
What are the possible complications of JIA?
- Poor growth
- Localised growth disturbances
- Micrognathia
- Contractures
- Ocular complications
- Osteopenia
Many hip pathologies cause limp but what is also important to remember to assess?
Other joints and think of extra-articular causes (including footwear & soles of feet for minor trauma or infection)
Growing pains should never cause a ..?..
LIMP
Even if blood tests and radiographs if normal what can you still consider?
Inflam causes such as JIA
What is a useful and quick tool to assess all joints & guide further detailed examination?
pGALS
When does a limp require referral?
Persisting beyond 2 weeks
What are red flag features?
- High fever
- Weight loss
- Night pain
- Lethargy
- New cardiac murmur
A febrile child with a limp or a non weight bearing child needs same day assessment
What is important to ask about that can indicate the presence of subacute or chronic brucellosis?
Hx of recurrent fevers even if the patient as no current fever
How does septic arthritis present?
- Acute onset of fever
- Joint pain & swelling
- Irritable
- Tachycardia
- Joint effusion
- Periarticular warmth & tenderness
- Pain on movement
What investigations are done for septic arthritis?
- Elevated CRP & ESR
- Raised WBC & platelets
- Blood cultures although results may be negative
- USS helpful to identify effusions
How is septic arthritis managed?
Referral to orthopaedics
IV Abx
Joint aspiration
How does slipped upper femoral epiphysis (SUFE or SCFE) present?
Displacement of the capital femoral epiphysis from femoral neck
Most common hip disorder of adolescence
Associated with obesity
Pain and altered gait
Worse with activity
How does SUFE present on a radiograph?
Ice cream slipping off cone appearance
How does transient synovitis present?
- Pain and limited movement in hip
- Aetiology unclear
- 3-8 years
- Symptoms < 1week
- Fever absent or low grade (not unwell looking)
How is transient synovitis seen on USS and how is it managed?
USS bilateral effusion
Resolves gradually with conservative therapy
What is Perthes disease and how does it present?
- Idiopathic avascular necrosis of hip
- Presents as insidious hip pain & limp
- Pain not relieved by rest or medication
- Bilateral in 10-20%
Who does Perthes disease usually effect?
3-12 years-peak 5-7 years
M:F 3-4:1
How is Perthes disease managed?
- Minimal weight bearing
- Contain head within acetabulum with use of splints
- Occasional surgery (osteotomy)
What does the outcome of Perthes disease look like?
- Long term outcome depend on age at onset
- Degree of involvement of femoral head
- Children <6-8 years have better prognosis
