A case of systemic sclerosis/lupus overlap syndrome presenting with bilateral cotton wool spots

Systemic sclerosis (SSc) is characterized by multi-system inflammation and fibrosis. Ophthalmologists must be aware of the uncommon ocular features of SSc to secure the diagnosis.

Here we report the rare occurrence of bilateral cotton wool spots in an 86-year-old woman with SSc/lupus overlap syndrome presenting with a history of chronic obstructive pulmonary disease, gastroesophageal reflux disease, polymyalgia rheumatica, scalp tenderness, and right jaw pain on chewing. Fundoscopy showed diffuse bilateral cotton wool spots that prompted the diagnosis of SSc/lupus overlap syndrome.

The confluence of patient symptoms was disguised as separate diseases, but the funduscopic finding of cotton wool spots in a patient without known risk factors prompted further investigation and the correct diagnosis. She was started on immunosuppressant therapy but unfortunately died four months later after developing right heart failure.

The differential diagnosis for bilateral cotton wool spots should include autoimmune processes such as SSc and systemic lupus erythematosus and may represent an early sign that can help direct treatment.