Paeds: The Limping Child (2) Flashcards
Features of Henoch-Schonlein-Purpura
- 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
Management of Henoch-Schonlein -Purpura
- analgesia for arthralgia
- treatment of nephropathy is generally supportive
- there is inconsistent evidence for the use of steroids and immunosuppressants
Prognosis of Henoch-Schonlein purpura
- usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
- around 1/3rd of patients have a relapse
How common malignancy is in paediatric rheumatology?
- 1% of patients referred to paediatric rheumatology have underlying malignancy
- Haematological disease, e.g. leukaemia and solid organ, e.g. neuroblastoma
What are primary bone tumours?
Primary bone tumour
- Osteosarcoma
- Osteoid osteoma (benign)
Osteoid osteoma
- epidemiology
- symptoms
- location
- x - ray findings
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
Osteosarcoma
- epidemiology
- symptoms
- location
- management
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
Callular changes in osteosarcoma
Mesenchymal cells with osteoblastic differentiation
X-ray changes in osteosarcoma
Periosteal Elevation:
- Sunburst appearance
- Codman’s triangle
Ewing’s sarcoma
- epidemiology
- presentation
- location
- management
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
X-ray changes seen with Ewing’s sarcoma
- Lytic tumour
- Onion-skin periosteal reaction
(3) types of Benign Cartilaginous Neoplasms
Association of ALL and MSK
- Bone pain and arthralgia in 20 - 40%
- Suspect from history, exam, or blood count
- Diagnosis by bone marrow aspirate
Neuroblastoma
- epidemiology
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
Pathophysiology of neruroblastoma
The tumour arises from neural crest tissue of the adrenal medulla (the most common site) and sympathetic nervous system
Features of neuroblastoma
- abdominal mass
- pallor, weight loss
- bone pain, limp from secondary spread
- hepatomegaly
- paraplegia
- proptosis
Ix in neuroblastoma
- raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels
- calcification may be seen on abdominal x-ray
- biopsy
Treatment and prognosis in neuroblastoma
Treatment surgery, radiation, chemotherapy, stem cell transplantation Prognosis US five-year survival ~95% (< 1 year old), 68% (1–14 years old)
Features that raise concerns of possible malignancy (in MSK) - red flags
- Bone pain (night time)
- Weight loss
- Night sweats or fevers
- Abnormal bloods
- X-ray changes
What’s Juvenile Idiopathic arthritis?
Juvenile idiopathic arthritis (JIA)
- arthritis occurring in someone who is less than 16 years old
- lasts for more than 6 weeks
What’s pauciarticular juvenile idiopathic arthritis?
- features
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
What’s systemic-onset juvenile idiopathic arthritis?
Systemic onset JIA is a type of JIA which is also known as Still’s disease
Features of Systemic onset idiopathic juvenile arthritis
+ investigations
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
Classification of Juvenile Idiopathic Arthritis (JIA)
Classification into sub-types by clinical onset pattern in first 6 months
Systemic arthritis features and criteria
Systemic arthritis
= arthritis and quotidian (high daily) fever PLUS
One of:
- evanescent, pink rash
- lymphadenopathy
- serositis
- hepatosplenomegaly
*Unwell - exclude sepsis & malignancy
What’s Macrophage activation syndrome?
- 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
Features of oligoarthritis
Features of polyarthritis
Diagnosis of Idiopathic Juvenile Osteoarthritis
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.
Management of idiopathic juvenile osteoarthritis
- 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.
How to clinically differentiate Juvenile idiopathic arthritis to another alternative diagnosis
- 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
Investigations for Juvenile Idiopathic Arthritis
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
