Ocular manifestations in Systemic Lupus Erythematosus (SLE) are relatively frequent, with a major prevalence of the Keratoconjunctivitis sicca. Nevertheless, the appearance of unilateral exophthalmos secondary to orbital pseudotumor in patients with SLE is extremely rare(1-7), and on occasion it can be refractory to conventional pharmacological treatment (glucocorticoids and immunosuppressants). We present the case of a patient with SLE and orbital pseudotumor refractory to treatment with Cyclophosphamide (CF) and an excellent clinical response, with disappearance of the ophthalmological condition after the beginning of therapy with Rituximab (1g×2), continuing after the infusion of two complete cycles without incidents.
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