Paeds: The Limping Child (2)

Question 1

Features of Henoch-Schonlein-Purpura

Answer 1

• palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs
• abdominal pain
• polyarthritis
• features of IgA nephropathy may occur e.g. haematuria, renal failure

Question 2

Management of Henoch-Schonlein -Purpura

Answer 2

• analgesia for arthralgia
• treatment of nephropathy is generally supportive
• there is inconsistent evidence for the use of steroids and immunosuppressants

Question 3

Prognosis of Henoch-Schonlein purpura

Answer 3

• usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
• around 1/3rd of patients have a relapse

Question 4

How common malignancy is in paediatric rheumatology?

Answer 4

• 1% of patients referred to paediatric rheumatology have underlying malignancy
• Haematological disease, e.g. leukaemia and solid organ, e.g. neuroblastoma

Question 5

What are primary bone tumours?

Answer 5

Primary bone tumour

• Osteosarcoma
• Osteoid osteoma (benign)

Question 6

Osteoid osteoma

• epidemiology
• symptoms
• location
• x - ray findings

Answer 6

Osteoid osteoma → benign, bone-forming neoplasm

• M>F = 2:1
• Teens and 20s

Symptoms:

• Severe nocturnal pain relieved by aspirin
• Hot on bone scan

Location: lower limb, diaphyseal cortex

X-ray: lytic lesion with central nidus and sclerotic rim

Question 7

Osteosarcoma

• epidemiology
• symptoms
• location
• management

Answer 7

Osteosacroma

• 20% of all primary bone tumours
• Incidence of 5 per 1,000,000
• Peak age 15-30, commoner in males
• Commonest primary bone tumour
• May arise secondary to Paget’s or irradiation

Symptoms: pain, warm, bruit

Location: knee metaphysis

Management: limb preserving surgery may be possible and many patients will receive chemotherapy

Location

Question 8

Callular changes in osteosarcoma

Answer 8

Mesenchymal cells with osteoblastic differentiation

Question 9

X-ray changes in osteosarcoma

Answer 9

Periosteal Elevation:

• Sunburst appearance
• Codman’s triangle

Question 10

Ewing’s sarcoma

• epidemiology
• presentation
• location
• management

Answer 10

Ewing’s sarcoma

Epidemiology

• commoner in males
• Incidence of 0.3 / 1, 000, 000
• onset typically between 10 and 20 years of age
• Histologically it is a small round tumour

Signs and Symptoms:

painful, warm, enlarging mass, fever, ↑ESR, anaemia, ↑WCC

Location: femoral diaphysis is commonest site

Management: Blood borne metastasis is common and chemotherapy is often combined with surgery

Question 11

X-ray changes seen with Ewing’s sarcoma

Answer 11

• Lytic tumour
• Onion-skin periosteal reaction

Question 12

(3) types of Benign Cartilaginous Neoplasms

Answer 12

Question 13

Association of ALL and MSK

Answer 13

• Bone pain and arthralgia in 20 - 40%
• Suspect from history, exam, or blood count
• Diagnosis by bone marrow aspirate

Question 14

Neuroblastoma

• epidemiology

Answer 14

Neuroblastoma

• one of the top five causes of cancer in children
• accounting for around 7-8% of childhood malignancies
• Median age of onset is around 20 months

Question 15

Pathophysiology of neruroblastoma

Answer 15

The tumour arises from neural crest tissue of the adrenal medulla (the most common site) and sympathetic nervous system

Question 16

Features of neuroblastoma

Answer 16

• abdominal mass
• pallor, weight loss
• bone pain, limp from secondary spread
• hepatomegaly
• paraplegia
• proptosis

Question 17

Ix in neuroblastoma

Answer 17

• raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels
• calcification may be seen on abdominal x-ray
• biopsy

Question 18

Treatment and prognosis in neuroblastoma

Answer 18

Treatment surgery, radiation, chemotherapy, stem cell transplantation Prognosis US five-year survival ~95% (< 1 year old), 68% (1–14 years old)

Question 19

Features that raise concerns of possible malignancy (in MSK) - red flags

Answer 19

• Bone pain (night time)
• Weight loss
• Night sweats or fevers
• Abnormal bloods
• X-ray changes

Question 20

What’s Juvenile Idiopathic arthritis?

Answer 20

Juvenile idiopathic arthritis (JIA)

• arthritis occurring in someone who is less than 16 years old
• lasts for more than 6 weeks

Question 21

What’s pauciarticular juvenile idiopathic arthritis?

• features

Answer 21

Pauciarticular JIA

• if 4 or less joints are affected
• it accounts for around 60% of cases of JIA

Features of pauciarticular JIA

• joint pain and swelling: usually medium sized joints e.g. knees, ankles, elbows
• limp
• ANA may be positive in JIA - associated with anterior uveitis

Question 22

What’s systemic-onset juvenile idiopathic arthritis?

Answer 22

Systemic onset JIA is a type of JIA which is also known as Still’s disease

Question 23

Features of Systemic onset idiopathic juvenile arthritis

+ investigations

Answer 23

Features of systemic onset JIA aka Still’s disease:

• pyrexia
• salmon-pink rash
• lymphadenopathy
• arthritis
• uveitis
• anorexia and weight loss

Investigations

• ANA may be positive, especially in oligoarticular JIA
• rheumatoid factor is usually negative

Question 24

Classification of Juvenile Idiopathic Arthritis (JIA)

Answer 24

Classification into sub-types by clinical onset pattern in first 6 months

Question 25

Systemic arthritis features and criteria

Answer 25

**Systemic arthritis** = arthritis and quotidian (high daily) fever **PLUS** **One of:** * evanescent, pink rash * lymphadenopathy * serositis * hepatosplenomegaly \*Unwell - exclude sepsis & malignancy

Question 26

What's ***Macrophage activation syndrome***?

Answer 26

* Life threatening complication of sJIA (systemic juvenille idiopathic arthritis) * Anaemia, leucopenia, thrombocytopaenia * Raised triglycerides * Falling ESR (low fibrinogen) * Raised liver enzymes * Raised ferritin (may be \> 100,000) * Treat any underlying trigger PLUS high dose steroids / CyA

Question 27

Features of oligoarthritis

Answer 27

Question 28

Features of polyarthritis

Answer 28

Question 29

Diagnosis of Idiopathic Juvenile Osteoarthritis

Answer 29

**Juvenile idiopathic arthritis** * a group of diseases characterised by inflammation of joints that cannot be explained by another mechanism - i.e. exclusion of conditions such as infection / trauma / malignancy and others * onset pattern of arthritis * Polyarthritis onset appears different to oligoarthritis onset and subsequent polyarticular course (“extended oligoarthritis”) \*Although predominantly a clinical diagnosis investigation may be needed to exclude other causes, but this will be driven by the clinical scenario.

Question 30

Management of idiopathic juvenile osteoarthritis

Answer 30

* MDT approach - paediatric rheumatology review via network OPTHALMOLOGY REFERRAL ! Needed * Drugs: IA steroid induction then DMARD if recurrent. Methotrexate first line then biologics anti TNF poly/extended oligo or anti IL-6 systemics.

Question 31

How to clinically differentiate Juvenile idiopathic arthritis to another alternative diagnosis

Answer 31

* other (than JIA) conditions can often be excluded by careful history e.g. fever/rashes / pallor / careful history of trauma mechanism (including NAI) Alternative diagnosis: * **Reactive arthritis** → usually viral and self-limiting over a few days * **Trauma** → would need to be significant to cause persistent swelling * **Septic arthritis** → usually associated with fever and systemic upset. Immunosuppressed patients may behave differently * **Malignancy** → look for other signs, especially constant limb / joint pain / pallor / hepatosplenomegaly / lymphadenopathy * **Consider IBD** / congenital disease / coagulopathy, and look for signs

Question 32

Investigations for Juvenile Idiopathic Arthritis

Answer 32

**Investigations:** For JIA - nil specific * Often ANA + * RF if poly disease * HLA B27 in boys (usually) * Non-specific inflammatory response * XRAY for bony change - rare early in disease course * US / MR scan for soft tissues