[Severe occlusive bilateral retinal vasculitis within the scope of seronegative systemic lupus erythematosus]

Retinal vascular manifestation is the most common form of ophthalmic involvement in patients with systemic lupus erythematosus (SLE). Most frequently these consist of cotton-wool spots with or without intraretinal hemorrhages. Although rare, a more severe retinal vaso-occlusive disease, termed retinal vasculitis, has been described. We report on a 37-year-old white female with a 13-year follow-up of chronic discoid lupus erythematosus, which suffered massive bilateral visual loss coincident with the systemic exacerbation of her disease (proteinuria, pneumonia, serositis, leucopenia). The diagnosis of SLE was established with reference to the revised ARA-criteria (American Rheumatism Association). Ophthalmoscopy and fluorescein angiography revealed the typical aspect of a SLE-associated vaso-occlusive retinopathy on both eyes with marked ischemia of the macula. Immediate maximal immuno-suppressive therapy, early performed panretinal photocoagulation and subsequent cryoretinopexy did not stop the progression of the disease. Six months after the initial event vascularisations of the disc and rubeosis iridis occurred, but no secondary glaucoma up to date. In this patient, the almost complete absence of characteristic autoantibodies and immunological markers was striking. The correlation with other lupus manifestations, different therapeutic concepts and prognostic factors in SLE-associated retinal vasculitis are discussed.