Rituximab as First-Line Therapy in Severe Lupus Erythematosus with Neuropsychiatric and Renal Involvement: A Case-Report and Review of the Literature

Abstract

Neuropsychiatric and renal involvement are common in systemic lupus erythematosus with negative impact on patient survival. Glucocorticoids, antiproliferative and cytotoxic agents represent first-line therapies, but are often ineffective and are burdened by significant toxicities. Despite the negative results of two randomized controlled trials, rituximab is still widely used as second- or third-line therapy in similar cases. No case has been reported so far where rituximab has been used as first-line therapy. We report the case of a 60-year-old cCaucasian woman with concurrent neuropsychiatric and renal lupus erythematous treated with one cycle of rituximab therapy at disease onset. Treatment was well tolerated and at 24 months the patient is in complete remission and free of immunosuppression. To the best of our knowledge, this is the first case of neuropsychiatric and renal lupus erythematosus successfully treated with rituximab as first-line therapy.

Three cases of systemic lupus erythematosus presenting with ischemic stroke as the initial symptom: Case reports and literature review

“…For patients with a confirmed diagnosis of NPSLE, current treatment options include glucocorticoids (GCs), immunosuppressants, biologics, intravenous immunoglobulins (IVIg), plasma exchange, and anticoagulant therapy 30–33 . Among the 20 cases reported in our study, only 12 reported treatment regimens.…”

Biologische Therapie bei systemischem Lupus erythematodes, Antiphospholipid-Syndrom und Sjögren-Syndrom: evidenz- und praxisbasierte Leitlinien

“…Die Wirksamkeit der RTX-Behandlung wurde auch bei refraktären Fällen mit neuropsychiatrischer Beteiligung, bei Patienten mit Delirium oder Psychose [52‒54] und bei ophthalmologischen Ausprägungen [55] berichtet. Darüber hinaus deuten einige Fallberichte darauf hin, dass RTX als Erstlinientherapie bei schwerem neuropsychiatrischem SLE [56], einschließlich des demyelinisierenden Syndroms als Folge von SLE, schwerer kognitiver Dysfunktion, Hirnstammerkrankung, Hirnnervenlähmungen und Schwäche und Taubheit in den Gliedmaßen [57], oder bei gleichzeitiger neuropsychiatrischer und renaler Beteiligung [58] nützlich sein kann.…”

Biological therapy in systemic lupus erythematosus, antiphospholipid syndrome, and Sjögren’s syndrome: evidence- and practice-based guidance

“…The effectiveness of RTX treatment was also reported in refractory cases with neuropsychiatric involvement, in patients with delirium or psychosis ( 52 – 54 ), and in the presence of ophthalmologic manifestations ( 55 ). Furthermore, some case reports suggest that RTX may be useful as 1 st line therapy in severe neuropsychiatric SLE ( 56 ), including demyelinating syndrome secondary to SLE, severe cognitive dysfunction, brainstem disease, cranial nerve palsies, and weakness and numbness in limbs ( 57 ) or with concurrent neuropsychiatric and renal involvement ( 58 ).…”