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Central nervous system involvement in systemic lupus erythematosus (SLE), termed neuropsychiatric SLE (NPSLE), is a relatively common manifestation of the disease. A previous study showed that patients with NPSLE with eye involvement are less common, with a prevalence of 0.66%.1 However, retinopathy occasionally presents as the primary manifestation of NPSLE, making diagnosis and management more challenge.2 Limited data exist regarding the optimisation of NPSLE treatment.1 3 Patients with NPSLE are usually treated with high doses of corticosteroids, cyclophosphamide (CTX), mycophenolate mofetil and azathioprine. In the case of refractory NPSLE, rituximab might be an alternative option.2 3 A few case reports on the use of immunoadsorption as a rescue treatment for patients with life-threatening NPSLE; however, none reported on refractory NPSLE patients with eye involvement.
We report on a patient with refractory NPSLE and progressive retinopathy who was successfully treated with immunoadsorption.
An 18-year-old female patient was first diagnosed with SLE in July 2016 had been treated with oral methylprednisolone (MP) (4 mg) and hydroxychloroquine (200 mg) daily, more recently photophobia in both eyes had developed. Laboratory tests revealed the presence of anti-nuclear antibody positive (1:1280), with antibodies to Smith (+), double-stranded DNA (dsDNA) (47 IU/L), Anti-Ro-52/ sjögren syndrome A antibody (RO-52) (+++), Anti-Ro-60/ sjögren syndrome A antibody (SSA) (+++), anti-UI-ribonucleoprotein antibody (U1-RNP) (+++), Anti–ribosomal-P antibodies (rib-P) (+) and white blood cell count was 2.62×109/L. Despite the prompt withdrawal of hydroxychloroquine which was suspected to be the cause of visual loss initially, the patient complained of worsened vision loss a week later. Retinal vasculitis was confirmed by ophthalmological examinations and multiple haemorrhages, multiple arteriolar occlusions, extensive avascular areas and macular oedema in …
Handling editor Josef S Smolen
Contributors GZ, XZ and HW contributed equally to this work. GZ examinedthe patient as the attending physician. SZ performed ophthalmological examinations. GZ and HW wrote the manuscript. HW and XZ supervised the study. All authors contributed to discussion and approved the final version.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.