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Management of Systemic Lupus Erythematosus

thrombosis and bone mass loss compared with control in SLE. 60, level I
However, the quality assessment of primary studies was not reported.

A prospective cohort study showed that the use of HCQ reduced the risk
of death (HR=0.46, 95% CI 0.29 to 0.72) and renal damage (HR=0.30,
95% CI 0.13 to 0.68) compared with non-HCQ use in patients with
SLE. 61, level II-2

In a retrospective cohort study among patients in the maintenance
phase of SLE, there were no significant difference between usual dose
HCQ (5 mg/kg) and low dose HCQ (200 mg) in SLEDAI, Cutaneous
Lupus Erythematous Disease Area and Severity Index (CLASI) and
serum levels of anti-dsDNA antibodies at 6-month. 62, level II-2

A systematic review of mixed study designs in SLE showed higher
odds of flare in patients with low HCQ levels (<1000 ng/mL) compared
with high levels (OR=5.89, 95% CI 1.38 to 25.08). The overall risk
of bias assessment of primary studies in the review showed the
eight observational studies were of fair to good quality while the
three interventional studies were of unclear to low risk. 63, level II-2 HCQ
adherence can be assessed using drug levels in the blood but it has not
been recommended in routine clinical practice at present.

Long-term use of HCQ treatment is safe. Toxicity related to HCQ is
infrequent, mild and usually reversible. 60, level I

Specifically, retinal toxicity related to HCQ is uncommon. A retrospective
cohort study on newly diagnosed SLE patients showed an incidence of
HCQ retinal toxicity at one in 1000 person-years. 64, level II-2 However, the
incidence increased in SLE patients with risk factors (e.g. long duration
65
and high dose of HCQ) for toxic retinopathy. In a case-control study
on SLE, a small proportion (5.5%) of patients developed antimalarial-
induced retinal complications over an average usage of 12.8 years. No
retinal toxicity was reported in the first five years of exposure. 66, level II-2
The American Academy of Ophthalmology recommends HCQ dose of
no more than 5 mg/kg actual body weight to reduce the occurrence of
retinopathy. 65

Recommendation 6
• All patients with systemic lupus erythematosus (SLE) should be on
hydroxychloroquine (HCQ) unless intolerant or contraindicated.
• Ophthalmologic assessment should be done for patients with SLE on
HCQ at baseline, and then:
 yearly in the presence of known retinopathy risk factors*
 after five years and yearly thereafter in the absence of retinopathy
risk factors*

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