Rituximab Therapy in Refractory Neuropsychiatric Lupus: Current Clinical Evidence

• Published in: Seminars in arthritis and rheumatism Vol. 41; no. 3; pp. 364 - 372
• Main Authors: Narváez, Javier, MD, PhD, Ríos-Rodriguez, Valeria, MD, de la Fuente, Diana, MD, Estrada, Paula, MD, López-Vives, Laura, MD, Gómez-Vaquero, Carmen, MD, PhD, Nolla, Joan Miquel, MD, PhD
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.12.2011; Elsevier
• Subjects: Adult; Antibodies, Monoclonal, Murine-Derived - therapeutic use; Antirheumatic Agents - therapeutic use; Biological and medical sciences; Diseases of the osteoarticular system; Female; Humans; Immunomodulators; Lupus Vasculitis, Central Nervous System - drug therapy; Medical sciences; neuropsychiatric manifestations; Off-Label Use; Pharmacology. Drug treatments; Rheumatology; Rituximab; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; systemic lupus erythematosus; Treatment Outcome; neuropsychiatric manifestations; systemic lupus erythematosus; rituximab; Antineoplastic agent; Immunopathology; Connective tissue disease; Skin disease; Nervous system diseases; Rheumatology; Rituximab; Autoimmune disease; Immunomodulator; Refractory; Systemic lupus erythematosus; Treatment; Systemic disease; Mental disorder; Immunosuppressive agent; Neurological disorder
• ISSN: 0049-0172; 1532-866X
• DOI: 10.1016/j.semarthrit.2011.06.004

Objective To review and summarize published information on the effectiveness and safety of rituximab (RTX) in adult patients with refractory neuropsychiatric systemic lupus erythematosus (NPSLE). Methods We describe a patient with persistently active NPSLE, despite conventional therapy, who responded dramatically to RTX. Current evidence on the therapeutic use of RTX in this complex situation is also analyzed through a systematic review of the English-language literature, based on a PubMed search. Results Available data on the use of RTX in refractory NPSLE come from a large number of case reports and some open-label studies. Including our case, 35 patients have been well documented. A complete or partial therapeutic response was achieved in 85% of patients after 1 cycle of treatment. A positive correlation between serological markers of disease activity and clinical outcome has also been demonstrated in some of these patients. Clinical improvement was accompanied by a significant reduction in the daily dose of oral corticosteroids. Relapse after RTX treatment was noted in 45% of cases (median 9.5 months; range, 4-33 months). Infections were observed in 29% of patients. Conclusion Evidence for the effectiveness of RTX as induction therapy in NPSLE is based solely on several case reports and noncontrolled trials. Although it is not yet possible to make definite recommendations, the global analysis of these cases supports the off-label use of RTX in cases of severe refractory NPSLE.