Systemic Lupus Erythematosus with Retinal Vasculitis

History of Present Illness

A 36-year-old female presented with complaints of blurred vision in both eyes (OU). In 1990, at the age of 15, she had developed butterfly-shaped skin lesions on the face; treatment was unspecific. In 1993, swelling and pain of the knee and small joints of both hands occurred. She was now found positive for antinuclear antibodies (ANA) leading to the diagnosis of systemic lupus erythematosus (SLE). She was treated with prednisone 10 to 20 mg per day for years.

In 1998 she had developed proteinuria leading to the diagnosis of lupus nephritis. She had developed arterial hypertension and moderate anemia (both were controlled with medications). Additional immunosuppressive treatment was initiated with Resochin, azathioprine, and pulsed cyclophosphamide, but the patient either did not tolerate the medication or it was inefficient. Finally, mycophenolate mofetil was introduced and continued for the next 4 years. This improved the patient’s condition.

In 2005, 1 year after she stopped mycophenolate in remission, she was found to have large areas of retinal ischemia, occlusive retinal vasculitis, and retinal neovascularization elsewhere (NVE) in the temporal retina ( Figs. 52.1 and 52.2 ). Laser photocoagulation of the ischemic areas was performed. In 2011 she needed peritoneal dialysis due to terminal renal insufficiency. Systemic treatment included prednisone 20 mg daily, which caused moderate osteoporosis and cataracts in both eyes. She had one episode of peritonitis in 2012 (due to Klebsiella ).

At the first examination in our clinic, she complained about deterioration of vision. Optical coherence tomography (OCT) revealed macular edema OU and a chorioretinal scar in the left macula. The patient received sub-Tenon triamcinolone injections bilaterally, and macular edema resolved. Due to retinal ischemia, laser photocoagulation was performed.

Questions to Ask

• Have you ever had any problems with sugar levels, excessive thirst, urination, night sweats, or loss of body mass?

• Have you ever had any numbness, tingling, weakness on one side of the body, or bowel or bladder problems? Is there any history of neurologic disorders in the family?

• Have you had any cough, lung problems, or episodes of fever?

• Have you noticed any recent ticks or tick bites on your body?

• Have you ever had a cat scratch?

• Do you have a travel history?

She answers no to all of the questions.

Assessment

Occlusive retinal vasculitis (arteritis) OU, macular edema