E024 Optic neuritis with neuropsychiatric lupus: a rheumatological emergency
Authors: Singh Hargurdas , Mohan Gurinder , Kaur Ranjeet , Jagga Sankalp H , Kaur Karandeep , Bansal Charles 
Published: April 2023

Background/Aims

Optic neuritis (ON) is uncommon rheumatological emergency with prevalence of around 0.6-1% in SLE although neuropsychiatric manifestations are found in 20-40%. The mechanism can be vasculitic or inflammatory or vaso-occlusive, etc.

Methods

A 24-year-old female with history of systemic lupus erythematosus for four years presented with headache, typical skin rash, alopecia, frothy urine, and pedal oedema from 2-3 months. The patient attendant also complained of mood changes, increased irrelevant talking, and different behaviour. On second day of admission, she complained blurring of vision. Ophthalmological examination revealed blurred disc margins with bilateral papilledema, 6/6 vision in both eyes, and negative Schirmer's test. MRI of brain with orbit showed distension and hyperintensities of both optic nerves on T2W images s/o optic neuritis and small areas of blooming in right parietal lobes, left insula, and thalamus on T2W images and isointense on T1w images and chronic white matter changes. Then, right vision dropped to 6/36 and left to 6/24. 24-hour urinary protein was 2040 mg/day and CT-guided renal biopsy showed coexisting lesions of membranous lupus nephritis: ISN/RPS Class V and focal lupus nephritis: ISN/RPS Class III with a modified NIH disease activity index of 6/24 and chronicity 1/12. ANA (IFA) Reflex to ENA Profile showed nuclear, speckled 3+ ANA with primary titre of 1:100 and end titre of 1:1000, strongly-positive U1RNP, and Sm. Hemoglobin 6.5 gm%, TLC 3700 cells/cumm, Platelets: 1.35 lakhs/cumm, ESR 84 mm/hr, CRP 0.1 mg/L, Serum Ferritin 626 ng/ml. Urine complete, chest-X Ray, liver and thyroid function tests were within normal limits, and viral markers, urine, blood culture, cANCA, and pANCA were negative.

Results

She was given methylprednisolone pulse therapy for 5 days and on fifth day of pulse therapy, vision was again 6/6 in both eyes with resolving papilledema, normal colour vision and resolving ON on repeat MRI. She was then started on oral steroids and mycophenolate mofetil as induction agent along with supportive care considering presence of concomitant lupus nephritis. Cyclophosphamide shows better results in resistant or worsening ON or severe NPSLE or nephritis. As patient was improving, young, and unmarried mycophenolate was chosen with strict close follow-up. Lin, Y.C. (2009) reviewed 8 patients of SLE-associated-ON reported between 1986 and 2004 and found three had very poor outcomes and only four achieved 20/25 and one 20/80. Even though it is not considered rare, data are scarce and requires more research.

Conclusion

ON is a rheumatological emergency requiring early aggressive intervention which can be life/organ saving. Suspecting and diagnosing ON in patients with neuropsychiatric lupus is challenging, We need specific guidelines and a national registry for SLE-associated-ON. Mycophenolate is a good option for neuropsychiatric lupus, SLE-associated-ON, and neuromyelitis optic spectrum disorder.

Disclosure

H. Singh: None. G. Mohan: None. R. Kaur: None. S.H. Jagga: None. K. Kaur: None. C. Bansal: None.