Bspar.org.uk

The British Society for
Paediatric and Adolescent
Rheumatology
Registered Charity # 1132967
HYDROXYCHLOROQUINE IN
PAEDIATRIC RHEUMATOLOGY

CLINICAL/DRUG INFORMATION
Hydroxychloroquine is an anti-malarial quinolone, which is used in the management of systemic
or discoid lupus. Although its mechanism of action is not fully understood, it is thought to
increase pH within intracellular vacuoles, which probably interferes with antigen processing in
macrophages and other antigen-presenting cells, thereby down-regulating the immune
response against auto-antigenic peptides 1.
There is increasing evidence- particularly from adult literature that its long term use in this
context, even in the absence of dramatic clinical improvement can help modify the long term
sequelae of Systemic Lupus Erythamatosus (SLE) such as cardiovascular disease 2.
It is rarely used in the treatment of Juvenile Idiopathic Arthritis and Juvenile Dermatomyositis. It
may also be helpful in Sarcoidosis and light sensitive dermatological disorders, where it may
only be needed at times of maximum light exposure.
PRE-TREATMENT CONSIDERATION/TESTING

 Baseline visual screening is recommended.  Baseline bloods including FBC, U+Es, LFTs.
CONTRAINDICATIONS 3

 Severe renal impairment- creatinine clearance <10ml/min/1.73m2  Pre existing maculopathy.  Use with caution mild/ moderate renal impairment - creatinine clearance 10- 50ml/min/1.73m2- reduce dose on prolonged use.  Neurological disorders (esp. history of epilepsy)  Liver disease - avoid concurrent use of hepatotoxic drugs.  May exacerbate psoriasis and aggravate myasthenia gravis. BSPAR is a Charitable Company registered in England and Wales. Company number 06978211 Registered office: 105 St Peter’s Street St Albans Herts AL1 3EJ DRUG INTERACTIONS 3

Major
 Increased risk of ventricular arrhythmias with: amiodarone, moxifloxacin.  Increased risk of seizures with: antiepileptics, mefloquine.  May increase plasma concentrations of: digoxin, ciclosporin  Avoid other antimalarials: Artemether/ luefantrine.  Inhibits the effects of: agalsidase beta (used in Fabrys disease), laronidase (used in mucopolysaccaridosis), neostigmine, pyridostigmine.  Effect reduced by: Kaolin, antacids.  Plasma concentrations increased by cimetidine.
SIDE-EFFECTS 3

 Gastrointestinal disturbance- nausea, diarrhoea, anorexia abdominal cramps.  Skin reactions- rash, pruritis. Rarely pigmentary changes, bleaching of hair and hair loss.  Visual changes – see visual screening section.  ECG changes, convulsions, ototoxicity.  Rarely: Blood disorders, psychological disturbance, myopathy, Steven Johnson syndrome, acute generalised exanthematous pustulosis, exfoliative dermatitis, photosensitivity, hepatic damage. DOSAGE /ADMINISTRATION 3
Dose should be calculated on lean body mass. For dosage calculator based on ideal body
weight:
Child age 1 month - 18 years: 5 - 6.5mg/kg once daily.
Maximum Dose 400mg od
Very toxic in overdosage- immediate advice form poisons centre essential. Parenteral diazepam
has been shown to reverse chloroquine cardiotoxicity.
FORMULATIONS 3
Plaquenil (Sanofi-Synthelabo) – Tablets – 200mg
Tablets may be halved, crushed or an extemperaneous suspension made (Formula available
from manufacturer)


MONITORING

Routine blood monitoring is not required.

Assessment and Monitoring of Vision 4:
The incidence of hydroxychloroquine induced retinopathy is very low with only 2 cases of a
series of 400 patients developing irreversible retinopathy, both after having taken maximum
BSPAR is a Charitable Company registered in England and Wales. Company number 06978211 Registered office: 105 St Peter’s Street St Albans Herts AL1 3EJ dose for 6 years. The maximum dose and duration of treatment both appear to have the most impact on the development of retinopathy. Quinolones may also precipitate in the cornea- however this is much less common with hydroxychloroquine than chloroquine. Recommendation to consult ophthalmologist at baseline for assessment of baseline visual assessment including: Enquiry about any disturbance of central vision  Central visual field, using an Amsler Chart (preferably red on black) or automated perimetry (e.g. Humphrey 10-2 protocol).  Stereoscopic slit lamp examination of the retina (e.g. with a 90D or 78 biconvex lens) Evaluation may need to be extended according to signs and symptoms to include retinal
photography, ocular coherence tomography (OCT), fundus autofluorescence (FAF) imaging and
visual electrophysiological tests.
Subsequent examinations should be at the discretion of the ophthalmologist, but indefinite
follow up is not likely to be required for most patients. For patients who have received
continuous treatment for more than 5 years, an individual arrangement should be agreed with
the local ophthalmologist.
Visual side effects are dose related and drug should be stopped if they develop.

FERTILITY/PREGNANCY/BREAST-FEEDING 3

 Pregnancy: Not necessary to withdraw anti malarial drug during pregnancy if rheumatological disease is well controlled. However manufacturer advises to avoid- possibly increased risk of cochlear damage  Breastfeeding: Avoid- risk of toxicity to infant.
REFERENCES
1) Mechanism of action of hydroxychloroquine as an antirheumatic drug. Fox RI.
Semin Arthritis Rheum 1993;23(2 Suppl 1): 82-91.
2) Effect of hydroxychloroquine on the survival of patients with systemic lupus erythematosus: data from LUMINA, a multiethnic US cohort (LUMINAet al )2007 3) BNF for children 2010-11 4) The Royal College of Ophthalmologists - Hydroxychloroquine and Ocular Toxicity Recommendations on Screening – October 2009 (contains 15 sub references) Guideline produced by Dr Tania Amin, and Dr J Walsh, Edinburgh Children’s Hospital, on behalf of the British Society for Paediatric and Adolescent Rheumatology (BSPAR) October 2010. Next review due Oct 2013 BSPAR is a Charitable Company registered in England and Wales. Company number 06978211 Registered office: 105 St Peter’s Street St Albans Herts AL1 3EJ

Source: https://www.bspar.org.uk/DocStore/FileLibrary/PDFs/BSPAR%20guidance%20for%20Hydroxychloroquine%202011.pdf