Paracentral acute middle maculopathy in systemic sclerosis and subsequent branch retinal artery occlusion

PURPOSE: We report a case of systemic sclerosis-associated paracentral acute middle maculopathy (PAMM) in a young woman who subsequently developed branch retinal artery occlusion. OBSERVATIONS: A 22-year-old woman presented with a paracentral scotoma. Optical coherence tomography (OCT) revealed bilateral paracentral acute middle maculopathy. Upon systemic examination, she was diagnosed with systemic sclerosis (SSc). She subsequently developed branch retinal artery occlusion despite vasodilator medications. After the prescription of aspirin, she did not experience a new event for one year. CONCLUSION AND IMPORTANCE: This case illustrates that SSc may affect the retinal vascular system and vision and cause PAMM. The optimal prophylaxis for patients with recurrent retinal events should be investigated in future studies.