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Flashcards in Rheumatology Deck (26):
1

Name the cardinal features of Henoch-Schonlein Purpura.

- Palpable purpura on the extensor surfaces of the bilateral lower limbs
- Abdominal pain
- Arthralgia with periarticular edema
- Hematuria
- Fever

>> Usually have a history of URTI 1-3 weeks before onset

2

What is the diagnostic criteria of Kawasaki Disease?

Fever of >38.5 degrees for at least 5 days AND any 4 of the following 5:

- Conjunctivitis (Bilateral nonpurulent conjunctivitis)
- Rash (polymorphic)
- Adenopathy (cervical lymphadenopathy >1.5cm)
- Strawberry tongue (and other mucosal changes like cracked lips)
- Hand skin-peeling (as well as perianal skin peeling)

>> CRASH and burn

3

What are the causes of limb pain in children?

BY AGE

<3 years of age
- Infections
   >> Osteomyelitis
   >> Septic arthritis
- Trauma
- Inflammatory causes
   >> Transient synovitis
   >> Juvenile idiopathic arthritis
- Neoplastic
   >>.Leukemia
   >> Neuroblastoma
- Hematological
   >> Hemophilia
   >> Sickle-cell disease


3-10 years
- Infection
- Trauma
- Inflammatory
   >> Transient synovitis
   >> Juvenile idiopathic arthritis
   >> Systemic lupus erythematosus
   >> Henoch Schonlein purpura
   >> Spondyloarthritis
   >> Dermatomyositis
- Neoplastic
   >> Bone tumours: osteosarcoma, Ewing sarcoma
   >> Leukemia
   >> Neuroblastoma
- Hematological
   >> Hemophilia
   >> Sickle cell disease
- Orthopedic
   >> Perthes disease
   >> Growing pains


>10 years
- Infection
- Trauma
- Inflammatory
   >> Juvenile idiopathic arthritis
   >> Systemic lupus erythematosus
   >> Henoch-Schonlein purpura
   >> Spondyloarthritis
   >> Dermatomyositis
- Neoplastic
   >> Bone tumours
   >> Leukemia
- Hematological
   >> Hemophilia
   >> Sickle-cell disease
- Orthopedic
   >> Perthes disease
   >> Slipped capital femoral epiphysis
   >> Osgoode-Schlatter
- Psychosocial: pain syndromes

4

What do we need to rule out in a child presenting with limb pain?

- Infection
- Acute orthopedic conditions: e.g. fractures
- Malignancy

5

What investigations are useful for working up a child with limb pain/limp?

Blood
- CBC with differential count
- Blood smear: sickle cell
- ESR/CRP: for inflammation
- Clotting profile: for hematological causes
- Blood culture
- CPK: for muscular causes
- Autoimmune screening
   >> ANA
   >> RF
   >> Immunoglobulins
   >> C3/C4 levels

Synovial fluid
- Cell count
- Gram stain and culture
- Protein (>4.4mg/dL)
- Glucose (Less than blood glucose in septic arthritis)

Urinalysis

Others
- Radiograph of the limb for orthopedic disorders
- Ultrasound of the joint: especially for effusion
- TB skin test if TB arthritis is suspected
- BM aspiration if leukemia is suspected
- Slit-lamp examination of the eye if JIA suspected: for any anterior uveitis


>> Especially in joint pain: listen for heart murmurs to rule out rheumatic fever and infectious endocarditis -- if the arthritis does not improve within 48 hours of therapeutic aspirin dose, it is probably not rheumatic fever.

6

What are the presenting features of transient synovitis of the hip?

Age: 2- 10 years; M>F

- Afebrile pain over hip and knee (referred from hip)
- Painful limp
- ROM full although painful
   >> Slight limitation at internal rotation and adduction
   >> No pain at rest
- Preceded by an upper respiratory tract infection
- Benign and self-limiting within 7-10 days

7

What is the management for transient synovitis of the hip?

- Symptomatic treatment
- NSAIDs

8

What are the most common organisms of septic arthritis in children?

Neonates
- GBS
- E. Coli
- Staphylococcus aureus

Infants (1-3 months)
- Streptococci
- Staphylococci
- Hemophilus influenzae

Children
- Staphylococcus aureus
- Streptococcus pneumoniae
- Group A streptococci + Neisseria gonorrhea in adolescents

9

What is the criteria for JIA diagnosis?

- Arthritis of more than 1 joint
- Duration of at least 6 weeks
- Onset less than 16 years of age

>> Classification defined by features/number of joints affected
in the FIRST 6 MONTHS OF ONSET

>> After exclusion of other causes of arthritis

10

What are the types of JIA (juvenile idiopathic arthritis)?

Oligoarticular arthritis
- Defined as arthritis of, at most, 4 joints (1-4 joints)
- Onset: 1-6 years
- Typically affects large joints
- ANA POSITIVE 60-80%
- Commonly associated with anterior uveitis
- Two types: persistent and extended
- PERSISTENT
   >> No more than 4 joints after first six months
- EXTENDED
   >> More than 4 joints after first six months
   >> Often asymmetrical

Polyarticular arthritis
- Defined as arthritis of 5 or more joints
- Two types: RF positive and RF negative
- RF negative
   >> Age of onset: 1-6 years; F>M
   >> Symmetrical involvement
- RF positive
   >> Age of onset: 11-16 years; F>M
   >> More severe than RF negative -- similar to adult RA
   >> Symmetrical involvement
   >> Rheumatoid nodules at pressure points
   >> Unremitting -- persists in adulthood

Enthesitis-Related Arthritis - HLA-B27 positive
- Age of onset: 6-16 years; M>F
- Risk of developing ankylosing sponylitis in childhood

Psoriatic arthritis
- Age of onset: 1-16 years; F>M
- Either arthritis + psoriasis; or at least two of:
   >> Dactylitis
   >> Nail abnormalities
   >> Family history of psoriasis
   >> Asymmetric/symmetric large/small joint involvement

Systemic JIA/Still's disease
- Age of onset: any age; M=F
- STILLS
    S: Spiking fever -- once or twice daily >38.5C >= 2 weeks
    T: JoinT (arthralgia)
    I: Increased size of liver/spleen
    L: Lose weight (anorexia)
    L: Looks like malignancy
    S: Salmon-pink rash
- Lymphadenopathy
- Serositis
- Paradoxical CBC: high WBC, high PLT, low Hb - excessive activation of MAC
- Other biochemical findings: high ESR and CRP

11

What is the difference between oligoarticular and polyarticular JIA by definition?

Oligoarticular: <=4 joints involved

Polyarticular: 5 or more joints involved

12

What should one ask for in the history to rule out/rule in JIA?

- Gelling: stiffness after prolonged rest
- Morning stiffness
- Pain over the joints
- Intermittent limp
- Deterioration in behaviour/mood disturbances
- Avoidance of previously enjoyed activities

13

What is the management of JIA?

Dependent on the type of JIA

Mainly articular
- NSAIDs
- Intra-articular corticosteroids
- DMARDs: methotrexate, sulfasalazine, lefunamide
- Biologics
   >> Anti-TNFa: etanercept, adalimumab
   >> Anti-CD20: rituximab

If systemic: oral/systemic corticosteroids instead

14

What are the common complications of JIA to look out for?

Joints
- Knee flexion contracture
- Leg-length discrepancy
- Osteoporosis

Systemic
- Growth disturbances
- Anemia of chronic illness
- Amyloidosis >> proteinuria/renal failure

15

What is the diagnostic criteria for SLE (systemic lupus erythematous)?

SCLICC classification criteria

Biopsy-proven lupus nephritis with ANA or anti-dsDNA
OR
4 of the listed criteria (at least 1 clinical and 1 immunological)

 

ACR revised criteria for SLE classification

Any 4 of the following criteria (MD SOAP BRAIN)"
M: Malar rash
D: Discoid rash
S: Serositis
O: Oral ulcers
A: Arthritis (>= 2 peripheral joints)
P: Photosensitivity
B: Blood
      - Hemolytic anemia
      - Leukocytopenia
      - Thrombocytopenia
R: Renal
      - Proteinuria
      - Cellular casts: RBC, granular, tubular
A: ANA positive
I: (Other) Immunological markers
      - Anti-dsDNA
      - Anti-smooth mucsle
      - Anti-phospholipid
      - Low C3/C4 levels
N: Neurological
      - Seizures
      - Psychosis
      - Mononeuritis multiplex
      - Myelitis
      - Peripheral/cranial neuropathy
      - Cerebritis

16

What is the difference between childhood-onset and adult-onset SLE?

- More active disease
- More renal diseases
- More intensive drug therapy>> more damage

17

What are some possible SLE treatment?

Dermatological
- Avoid UV lights
- Topical steroids
- Topical hydroxychloroquine

Musculoskeletal
- NSAIDs
- Hydroxychloroquine
- Bisphosphonates, calcium & vit D to prevent osteoporosis, esp. in steroid usage

Organ-threatening disease
- High-dose oral prednisolone/IV methylprednisolone
- Steroid sparing immunosuppressants
    >> Azathioprine
    >> Methotrexate
    >> Mycophenolate
    >> Mofetil
    >> IVIG
- IV cyclophosphamide for cerebritis and nephritis
- Biologics
    >> Rituximab
    >> Belimumab

18

What is the most common vasculitis in childhood?

Henoch-Schonlein Purpura

19

What is the common age of onset of HSP?

4-10 years; M>F

20

What is the management of HSP?

- Mainly supportive
   >> NSAIDs for joint pain
   >> Corticosteroids
- Monitor urine protein every 6 weeks for 4-6 months to check for any renal disease needing immunosuppressive therapy (may present late)


Usually self-limiting within 4 weeks

Recurrence in about one third of patients

21

What is atypical Kawasaki disease?

Less than 5 of the 6 diagnostic features but has coronary artery involvement

22

What is the peak age of onset for Kawasaki disease?

3 months - 5 years

23

What is the management for Kawasaki disease?

- High-dose aspirin while febrile
- Lose-dose aspirin in subacute phase until platelets normalize (anti-platelet function
- IVIG within 10 days of onset (2g/kg over 8-12 hours)
   >> Baseline ECHO and follow-up ECHO at 6 weeks
   >> 50% of coronary aneurysms regress within 2 years

24

What are the risk factors for coronary disease in Kawasaki disease?

- Male
- Age: <1 year or >9 years
- Prolonged fever >10 days
- Recurrence of fever after 48 hours of being afebrile
- Cardiomegaly
- Thrombocytopenia
- Hyponatremia

25

What are the possible laboratory findings in Kawasaki disease?

CBC
- Increased WBC
- Increased PLT
- Normocytic normochromic anemia

CRP
- Elevated for >80% from normal levels LRFT
- Hyponatremia
- Hypoalbuminemia
- Increased ALT

Urine
- Pyuria without bacteruria

Lumbar puncture/CSF
- Pleocytosis
- Normal protein
- Normal glucose

26

Name the drugs that cause drug-induced SLE.

SHIPP

S: Sulfonamides
H: Hydralazine
I: Isoniazid
P: Procainamide
P: Phenytoin