Paediatric Case Presentation

1. Paediatric Case Presentation Natasha Quader ST1 September 2008

2. Patient CD • 8 year old girl • Normally fit and well • PC – Pyrexia - Lethargy - Groin and hip pain

3. History of Presenting Complaint • 7/52 ago had pyrexia for 1 week followed by a cough → viral URTI • Intermittent pyrexia - mainly nocturnal spikes • C/o hip and groin pain. - Present on waking up in the morning - Pain waking her up during sleeping - Developed difficulty in walking, pain on weight bearing • Increasingly lethargic – sleeping during the day and missing school • Generally unwell, ↓ appetite, irritable and crying

4. PMH • PMH - Laryngomalacia during infancy - RTA in Feb ’07 – back injury resolved • SH – no recent travel abroad • Immunisations up-to-date • DxH – nil, NKDA • FH – Maternal grandmother with RA

5. Alert, communicating PR – 80 regular Temp – 38 °C RR-18 Bilateral cervical lymphadenopathy Throat – NAD Ears – wax bilaterally No rashes HS I + II + O Chest: Clear Examination 1 • Abdomen x x x x x Lower abdominal tenderness Inguinal lymphadenopathy

6. Examination 2 MSK • No hip/knee joint swelling • Tenderness over AIS and crest bilaterally • Hips - ↓ active ROM CNS • CN – NAD. PEARL • Lower limbs bilaterally - tone normal - power 4/5 - reflexes symmetrical • Co-ordination – intact • Romberg’s negative • Antalgic gait • Slight waddling and holding R. inguinal region

7. Positive Examination Findings • Pyrexic • Lower abdominal tenderness • Bilateral inguinal lymphadenopathy • Bilateral hip tenderness and restricted ROM • Antalgic gait

8. Differential Diagnosis • Septic hip • Infection – viral, bacterial, Lyme disease • Juvenile arthritis • Malignancy – leukaemia, neuroblastoma • Perthes • Transient irritable hip

9. Infection Screen • MSU – NAD • Blood cultures – no growth • EBV Igm – negative • Toxopl Igm – negative • ASOT – normal • Rubella Igm – negative • Parovirus Igm – negative

10. Bilateral Hip X-ray

11. Imaging • USS abdo and hips – no fluid in hip joints • MRI abdo/pelvis - No evidence of avascular necrosis

12. Blood Test Results

13. Follow Up • Follow up 10/7 after discharged from Hospital • Symptoms much improved - no complaints of hip pain on weight bearing - no pain during sleep - no pyrexia for 5/7 - analgesia reduced from QDS to ON - generally more mobile, active and cheerful

14. Follow up Results • On examination - discomfort on extreme range of full abduction of R. hip - Tearful on jumping on 2 feet • Repeat bloods: ESR remains high – 104 Anti nuclear antibody positive • Referral → Rheumatologist → Ophthalmologist

15. Juvenile Arthritis Is a group of conditions in which there is chronic arthritis lasting more than 6 weeks, presenting before 16 years of age

16. Classifications • Systemic illness (Still’s disease) - 20% • Rh factor negative polyarticular - 25% • Rh factor positive polyarticular - 5% • Pauciarticular arthritis assoc with ANA & chronic uveitis - 30-35% • Pauciarticular arthritis assoc with spondylitis and HLA B27 - 10-15%

17. Aetiology • Autoimmune disease in which the cause of arthritis is largely unknown • A possible viral aetiology has been considered for a variety of arthritis conditions and clustering of patient’s following viral epidemics • Viral infections that have noted include mumps, rubella and Parvovirus B19

18. Pauciarticular Arthritis • Female > Male • Involvement of up to 4 or fewer joints – typically larger joints such as the knee • 3 subtypes: 1) The early onset form (< 5 years of age), involves most frequently knee joints 2) The late onset form ( > 9 years of age) most frequently involves hips with/without sacroilitis 3) Develops at any age with an asymmetrical oligoarthritis, dactylitis and psoriatic manifestation

19. Pauciarticular Arthritis 2 • Is a large association with Anti Nuclear Antibodies between 40-75% • Frequently associated with chronic anterior uveitis (can be asymptomatic or asso pain, light sensitivity and redness) • Regular opthalmological screening is indicated

20. Complications • Chronic anterior uveitis – if poorly controlled may result in permanent eye damage, including blindness • Flexion contactures of the joint – joint held in the most comfortable position, thereby minimising intra-articular pressure. Chronic disease can lead to joint destruction and need for joint replacement • Growth failure – anorexia, chronic disease and steroid therapy • Amyloidosis – rare complication causing proteinuria and renal failure

21. Management 1 • Multidisciplinary approach is required for optimal Rx: - relieve pain - preserve joint function - maintain normal growth and psycho – social development • Physiotherapist & Occupational Therapists - strengthen muscles & keep joints flexible - encourage normal limb development - maintain function and prevent deformities - develop exercise programs

22. Management 2 • Paediatricians, Rheumatologist and Nurses - educating child and family - medical management • Counsellor – for the child and their family to reduce anxiety and share management of disease

23. Medical Management • NSAIDS – aspirin, ibuprofen, diclofenac • DMARDS (Disease modifying anti rheumatic drugs) - under rheumatologist’s supervision - hydroxychloroquine, penicillamine, methotrexate • Intra-articular corticosteroid injections • Corticosteroids – severe systemic involvement &/or for eyes Rx • TNF alpha blockers – block the immune protein TNF (inflammatory agent in arthritis)

24. Prognosis • Symptoms usually go away after a few years • There are usually no further recurrences • Some children may have longer lasting involvement

25. References • Laura Quarte et al. Juvenile idiopathic arthritis: An update on clinical and therapeutic approaches. Ann Ital Med Int 2005; 20: 211-217 • Juvenile Rheumatoid Arthritis http://www.emedicine.com/ped/topic1749.htm • Juvenile Rheumatoid Arthritis http://www.emedicinehealth.com/juvenile_rheumatoid_arthritis/article_em.htm • Arthritis in children http://www.medicinenet.com/juvenile_arthritis/article.htm