MALT lymphoma of the lacrimal gland in the context of systemic lupus erythematosus: complete remission after treatment with rituximab.

Mucosa-associated lymphoid tissue (MALT) lymphoma of the lacrimal glands has been reported in only two patients with systemic lupus erythematosus (SLE) in the literature. We describe a 41-year-old female with SLE who had multiple relapses and remissions of her disease during the last 20 years and developed a right eyelid swelling. Magnetic resonance imaging showed a lesion in the right lacrimal gland with increased enhancement on T1- and T2-weighted images after intravenous contrast administration, and the biopsy of lacrimal gland was consistent with the diagnosis of marginal zone B-cell lymphoma of MALT type. The patient received treatment with four once-weekly doses of rituximab 375 mg/m(2) every 6 months for 2 years resulting in complete remission. Lacrimal gland MALT lymphoma is mainly treated with local radiotherapy, or chemotherapy in cases with systemic lymphoma. This is the first case of rituximab treatment in a patient with SLE who developed lacrimal gland MALT lymphoma, resulting in complete durable remission.