Juvenile Idiopathic Arthritis (JIA)

Prescribing Notes:

For children at high risk of gastro-intestinal adverse events:

• First choice would be to avoid a NSAID. Second choice, prescribe a NSAID + omeprazole.
• Children at ‘high risk’ of developing serious gastro-intestinal adverse events include:
  • Children with previous peptic ulcer
  • Children with previous GI bleed
  • Children receiving systemic corticosteroids
  • Children receiving anticoagulants
  • Children requiring very high dose NSAIDs [greater than 120% average daily dose]

• Consider whether an NSAID is required; regular dosing of paracetamol is often adequate for pain.
• All long-term use of NSAIDs in children should be under the guidance of a specialist.
• Relative contra-indications to NSAIDs include heart failure, hypertension, renal impairment, history of gastro-intestinal bleeding, coagulation defects; absolute contra-indications include proven hypersensitivity to aspirin or any NSAID.
• NSAIDs may worsen asthma; they are contra-indicated if aspirin or any other NSAID has precipitated attacks of asthma, although this rarely occurs in children.
• Naproxen is an alternative NSAID which combines good efficacy with a low incidence of side-effects.
• Naproxen is associated with a 10% risk of blistering skin rash in blonde, blue eyed, non-tanning children.
• Diclofenac e/c tablets are only suitable for children who are able to swallow tablets whole. Naproxen oral suspension may be a suitable alternative preparation for patients who cannot swallow tablets. For those patients in whom naproxen is not a suitable choice the rheumatology team will advise.
• If other therapy is successful in controlling arthritis, then consider withdrawing NSAIDs especially in children receiving conventional DMARDs and drugs affecting the immune system (see DMARD and biologic pathways).

Prescribing Notes:

• Corticosteroids should ideally only be commenced after liaison with a rheumatologist, and a steroid card given in every case.
• Patient/parent/carer should be provided with written information and given the opportunity to discuss the benefits and risks of long-term corticosteroids before treatment is commenced.
• Further weekly doses of intravenous methylprednisolone sodium succinate may be required for some patients on the advice of a specialist.
• The requirement for prophylactic bone protection in children is uncertain. However, this should be considered for children receiving long-term corticosteroids.
• Long-term steroids should be withdrawn gradually.
• In patients receiving high doses of corticosteroids consider assessment for adrenal suppression using a short Synacthen (tetracosactide) test.
• For patients with swallowing difficulties prednisolone oral solution or soluble tablets may be considered. Prednisolone tablets may be dispersed in water as an alternative to soluble tablets or oral solution preparations. This is an off-label use but more cost-effective option. Refer to local board policies on the use of unlicensed (and off-label) medicines for further guidance
• The British Society for Rheumatology and Scottish Paediatric and Adolescent Rheumatology Network (SPARN) websites include useful supporting information. Locally, specialists refer to the current edition of the Paediatric Rheumatology (Oxford Specialist Handbook in Paediatrics). Consult up to date BNFc and product literature for full information.

Prescribing Notes:

• Children requiring joint injections may require general anaesthesia or sedation. Factors to consider include: patients age, the number and the type of joints affected. In children> 5 years Entonox may be considered for some patients. Older children can be administered intra-articular injections with a topical local anaesthetic, e.g. Emla cream.
• Triamcinolone acetonide and triamcinolone hexacetonide are discontinued. For further information see Medicine Supply Alert Notice MSAN (2025) 16 Triamcinolone acetonide 10mg/ml (Adcortyl® ampoules) and 40mg/ml (Kenalog® vials) suspension for injection.

Prescribing Notes:

• The medicines in this pathway are for use within specialist rheumatology services including those working within the paediatric rheumatology network.
• This pathway refers mainly to the management of JIA. DMARDs are used for other paediatric rheumatology conditions at the discretion of a specialist experienced in the management of the condition, the order of choices in this pathway is therefore not intended to guide treatment choice.
• Methotrexate is the first choice in the majority of rheumatic diseases but in certain conditions other agents may be preferred first e.g. lupus.
• Risks/benefits of DMARDS and drugs affecting the immune response should be discussed with the patient/parent/carer before commencing using a written information sheet available from the Versus Arthritis website or RHSC.
• DMARDs are appropriate for a shared care arrangements to facilitate the seamless transfer of individual patient care from secondary care to general practice.
• The MHRA has received reports of prescription and dispensing errors for methotrexate that have resulted in serious and fatal adverse reactions. Methotrexate tablets should be prescribed in 2.5mg strength only. The 10mg strength should not be used since they may be confused with the 2.5mg tablets.
• The use of oral methotrexate for non-malignant conditions such as rheumatoid arthritis has been highlighted nationally as a potential risk for fatal medication errors. New measures have been implemented to prompt healthcare professionals to record the day of the week for intake and to remind patients of the dosing schedule and the risks of overdose due to continued reports of inadvertent overdose. For further advice see MHRA Drug Safety Update September 2020.
• Patients who have a partial response to or intolerance of oral methotrexate may be switched to subcutaneous methotrexate under the guidance of a rheumatologist.
• Subcutaneous methotrexate should only be given by individuals trained to administer methotrexate competently.
• Providing monitoring procedure is followed, NSAIDs may be prescribed with methotrexate. However, the need for NSAID therapy should be reviewed once methotrexate becomes effective.
• Calcium folinate (folinic acid) 15mg orally or by injection may be an alternative for children who are unwilling to take folic acid due to gastro-intestinal intolerance or its unpalatability.
• All children must be adequately monitored refer to SPARN guidelines/BNFc/SPC for full details for the medication in question.
• Azathioprine is sometimes used as an adjunct to infliximab.
• Concurrent use of ciclosporin and corticosteroids is common and advantageous but be alert for any evidence of increased ciclosporin and corticosteroid adverse effects.

Prescribing Notes:

• Biologic therapies or or targeted synthetic DMARDs may be initiated by rheumatologists in patients who have not responded to conventional DMARDs in line with East Region Formulary Decisions i.e. locally approved health technology assessment approvals and restrictions in line with national guidance.
• The British Society for Rheumatology and Scottish Paediatric and Adolescent Rheumatology Network (SPARN) websites include useful supporting information. However, some of the treatment guidelines are due for update. Locally, specialists refer to the current edition of the Paediatric Rheumatology (Oxford Specialist Handbook in Paediatrics). Please consult up to date BNFc and product literature for full information. 
• The medicines in this pathway are for use within specialist rheumatology services including those working within the paediatric rheumatology network.
• When more than 1 technology is suitable treatment should be started with the least expensive technology, taking into account administration costs, the dose needed and the product cost per dose.
• Abatacept, adalimumab, etanercept and tocilizumab are recommended, within their marketing authorisations, as options for treating polyarticular juvenile idiopathic arthritis (JIA), including polyarticular onset, polyarticular course and extended oligoarticular JIA. That is:
  • for abatacept, people 2 years and older whose disease has responded inadequately to other disease modifying anti-rheumatic drugs (DMARDs) including at least 1 tumour necrosis factor (TNF) inhibitor
  • for adalimumab, people 2 years and older whose disease has responded inadequately to 1 or more DMARD
  • for etanercept, people 2 years and older whose disease has responded inadequately to, or who are intolerant of methotrexate
  • for tocilizumab, people 2 years and older whose disease has responded inadequately to previous therapy with methotrexate.
• Adalimumab and etanercept are recommended, within their marketing authorisations, as options for treating enthesitis related JIA, that is, for people 6 years and older (adalimumab) and 12 years and older (etanercept) whose disease has responded inadequately to, or who are intolerant of, conventional therapy. 
• Etanercept is recommended, within its marketing authorisation, as an option for treating psoriatic JIA, that is, in people aged 12 years and over whose disease has responded inadequately to, or who are intolerant of, methotrexate. 
• Adalimumab is approved for the treatment of active enthesitis-related arthritis in patients, 6 years of age and older, who have had an inadequate response to, or who are intolerant of, conventional therapy. 
• Etanercept is approved for the treatment of - polyarthritis (rheumatoid factor positive or negative) and extended oligoarthritis in children and adolescents from the age of 2 years who have had an inadequate response to, or who have proved intolerant of, methotrexate; psoriatic arthritis in adolescents from the age of 12 years who have had an inadequate response to, or who have proved intolerant of, methotrexate; enthesitis-related arthritis in adolescents from the age of 12 years who have had an inadequate response to, or who have proved intolerant of, conventional therapy. 
• Secukinumab is approved for juvenile idiopathic arthritis (JIA); Enthesitis-related arthritis (ERA) - alone or in combination with methotrexate (MTX) for the treatment of active enthesitis-related arthritis in patients 6 years and older whose disease has responded inadequately to, or who cannot tolerate, conventional therapy; Juvenile psoriatic arthritis (JPsA) - alone or in combination with methotrexate (MTX) for the treatment of active juvenile psoriatic arthritis in patients 6 years and older whose disease has responded inadequately to, or who cannot tolerate, conventional therapy. Restricted for use when the patient has not responded to adequate trials of at least 2 standard disease-modifying antirheumatic drugs (DMARDs), administered either individually or in combination; the person has had a TNF-alpha inhibitor, but their disease has not responded within the first 12 weeks or has stopped responding after 12 weeks or TNF-alpha inhibitors are contra-indicated but would otherwise be considered. 
• Tofacitinib is approved for the treatment of active polyarticular juvenile idiopathic arthritis (rheumatoid factor positive or negative polyarthritis and extended oligoarthritis), and juvenile psoriatic arthritis (PsA) in patients 2 years of age and older, who have responded inadequately to previous therapy with DMARDs. Tofacitinib can be given in combination with methotrexate (MTX) or as monotherapy in case of intolerance to MTX or where continued treatment with MTX is inappropriate.  Restricted to use in patients with severe disease that has not responded to intensive therapy with a combination of conventional DMARDs. Or severe disease inadequately controlled by a TNF-alpha inhibitor. 
• Baricitinib is approved for treatment of active juvenile idiopathic arthritis in patients 2 years of age and older who have had an inadequate response or intolerance to one or more prior conventional synthetic or biologic disease-modifying antirheumatic drugs (DMARDs). Use is restricted to severe disease that has not responded to conventional therapy with a combination of conventional DMARDs, i.e. at least two conventional DMARDs. Or severe disease inadequately controlled by a TNF-alpha inhibitor. 
• Monitoring guidance for individual agents can be found in the unit protocols and is included in patient correspondence.