030. Immunoglobulin therapy in the treatment of cerebral consequences of Neonatal Lupus Erythematosus

Abstract

Background: The neurological consequences of neonatal lupus erythematosus (NLE) are increasingly recognised. As far as we are aware, there are no reports of immunomodulatory treatment in this situation. Aims: To report the case of a 3 month old infant with NLE with hydrocephalus associated with bilateral subdural collections and to describe the use of immunoglobulin (IVIG) in her management. Methods: We produced a retrospective case review. Results: Antenatally, complete heart block (CHB) was identified on 20 week scan. Foetal cardiac anatomy was normal. Further investigation revealed maternal anti-Ro and anti-La antibodies. The infant was delivered in good condition at 34 +4 weeks gestation at the regional cardiac centre, following antenatal steroids. Epicardial pacemaker was inserted on day 7 and she was discharged on day 19. Subsequently, her mother reported her own fatigue, dry eyes and intermittent photosensitive vasculitic rash, and was diagnosed with systemic lupus erythematous. At 12 weeks of age, it was noted during routine follow-up that her occipitofrontal circumference had increased from 75th centile at 4 weeks to > 99.6th. There was evidence of increased intracranial pressure with tense fontanelle, cranial suture separation, though no papilloedema. Cranial ultrasound scan showed lateral and third ventriculomegaly, bilateral subdural collections and enlarged extra-axial space. CT scan showed generalised reduced brain volume with increase in fluid space. MRI scan was contraindicated due to pacemaker. She developed a rash; palpable erythematous lesions with fine scale on her forehead and back; consistent with NLE and topical tacrolimus and SPF50 therapy were initiated. She had mild transaminitis and platelets were normal. She was referred to Rheumatology. Investigations showed positive ENA titres with anti-Ro60 and anti-La antibodies present. Hydrocephalus was rapidly increasing; neurosurgical intervention was considered. Following parental discussion, she was treated with a single dose of 2g/kg IVIG. Following this, there was clinical improvement with her fontanelle becoming less tense and her OFC stabilised. She did not require neurosurgical intervention. Her rash and transaminitis completely resolved. Cranial ultrasound 4 months post-IVIG showed improvement in the depth of extra-axial fluid; ventricular-cranial ratio had improved from 45% to 35%. ANA and ENA remained positive at 6 months. Aged 2 years, she continues to reach neuro-developmental milestones. Conclusions: To our knowledge, this is the first case report of IVIG being used to ameliorate the neurological consequences of anti-Ro antibodies. IVIG has been shown not to prevent CHB when administered antenatally to mothers of infants at risk of NLE. However, in this case, IVIG as a treatment of chronic subdural collections associated with NLE appeared to offer benefit. This case raises awareness of the under-recognised neurological sequelae of NLE. Infants with NLE should be monitored for abnormal head growth. The use of IVIG in this infant appeared to ameliorate the rapidly progressive hydrocephalus associated with subdural collections.