P35 Dermatomyositis with retinal involvement: a case series

Abstract Case report - Introduction Dermatomyositis (DM) is a multisystem, autoimmune condition. It belongs to a spectrum of idiopathic inflammatory myopathies (IIMs) which can present with proximal muscle weakness or cutaneous lesions, but can also cause systemic disease including interstitial pneumonia, myocarditis, and inflammatory arthritis. Retinal involvement is not widely described. However, we present three cases with visual disturbance caused by retinal pathology – two of retinal vein occlusion, and one of retinal vasculitis. Two cases have anti-melanoma differentiation-associated gene 5 (anti-MDA-5), which may be a pathophysiological link to retinal microvascular damage. Case report - Case description Patient 1: A 43-year-old female with Anti-NXP-2 juvenile DM presented with bilateral visual loss. She was diagnosed with DM aged 6 when she presented with calcinosis, weakness, inflammatory arthritis and rash. Though disease was most active during adolescence, she has ongoing problematic calcinosis. ESR remains 26mm/hr. Fundoscopy showed scattered haemorrhages and macular oedema with no features of retinal vasculitis. Thrombophilia screens including antiphospholipid were negative. Bilateral branch retinal vein occlusions were treated with anti-VEGF injections. Post-treatment visual acuity is 6/6. Patient 2: A 46-year-old female with Anti-MDA-5 DM presented with monocular reduced visual acuity. She was diagnosed with a hemi-retinal vein occlusion. Thrombophilia screen showed she was antiphospholipid negative, but a heterozygote carrier of Factor V Leiden. She had moderately active disease at the time with evidence of organising pneumonia, and active Gottron’s papules. Prednisolone was increased, tacrolimus dosing was optimised and clopidogrel commenced. Later that year she was given rituximab for her organising pneumonia to which her skin showed good response. However, 8 months later she had a further central retinal vein occlusion with macular oedema treated with Anti-VEGF injections. Her subsequent visual acuity is 6/6. Patient 3: A 36-year-old male with Anti-MDA-5 DM with skin, joint and lung involvement was initially treated with IV cyclophosphamide. He then developed severe Raynaud’s, perniotic skin rash and vasculitic digital ulceration. He noted reduction in visual acuity. Fundoscopy revealed bilateral cotton wool spots and wide-field fundus fluorescein angiography showed microaneurysms. A diagnosis of a mild retinal vasculitis was made. He wa