title: Cyclophosphamide in combination with glucocorticoids for severe neuropsychiatric systemic lupus erythematosus: a retrospective, observational two-centre study.
abstract: Cyclophosphamide (CYC) is used in severe neuropsychiatric systemic lupus erythematosus (NPSLE), but long-term data regarding its efficacy and safety are lacking. We identified NPSLE cases who received CYC from two centres during the period 1999-2013 and had regular follow-up. General and neuropsychiatric outcome at last follow-up visit were determined, and major complications were documented. CYC was administered in 50 neuropsychiatric events. Median age was 45.0 years and 46% of patients were positive for antiphospholipid antibodies. Most frequent indications were psychosis (11 cases), polyneuropathy (six cases), and cerebrovascular disease, seizure disorder and cranial neuropathy (five cases). CYC was mainly administered as monthly pulses (median number: 8.0 (range 3-26), median cumulative dose: 7.2 g (range 2.4-33.8)). Cases were followed for a median of 46.5 months (range 5-408). At last follow-up, partial or complete response of NPSLE was observed in 84% of events; 10% had stable disease, whereas the remaining 6% failed to improve or worsened and were rescued with rituximab. In events that responded to CYC, maintenance therapy consisted of azathioprine in 31 events (65.9%), bimonthly or quarterly pulses of intravenous CYC in nine (19.1%), and mycophenolate mofetil in five (10.6%). Relapses were observed in six events (12%) at median eight months after initial response. No malignancies were observed, yet there were three cases of severe infections. Amenorrhea was recorded in three patients, who had not received gonadal protection. In conclusion, cyclophosphamide was efficacious and led to sustained response of severe NPSLE in a cohort with long follow-up.
authors.affiliation: Department of Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Heraklion, Greece Institute of Molecular Biology and Biotechnology, Foundation of Research and Technology-Hellas, Voutes, Heraklion, Greece afanouriakis@edu.med.uoc.gr.
authors.forename: A
authors.initials: A
authors.lastname: Fanouriakis
authors.affiliation: Department of Rheumatology, 'Iuliu Hatieganu' University of Medicine and Pharmacy, Cluj-Napoca, Romania.
authors.forename: C
authors.initials: C
authors.lastname: Pamfil
authors.affiliation: Department of Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Heraklion, Greece.
authors.forename: P
authors.initials: P
authors.lastname: Sidiropoulos
authors.affiliation: Emergency Clinical County Hospital, Cluj-Napoca, Romania.
authors.forename: L
authors.initials: L
authors.lastname: Damian
authors.affiliation: Department of Psychology, Babes-Bolyai University, Cluj-Napoca, Romania.
authors.forename: A
authors.initials: A
authors.lastname: Flestea
authors.affiliation: Department of Cardiology, Clinical Rehabilitation Hospital, 'Iuliu Hatieganu' University of Medicine and Pharmacy, Cluj-Napoca, Romania.
authors.forename: G
authors.initials: G
authors.lastname: Gusetu
authors.affiliation: Department of Rheumatology, 'Iuliu Hatieganu' University of Medicine and Pharmacy, Cluj-Napoca, Romania.
authors.forename: S
authors.initials: S
authors.lastname: Rednic
authors.affiliation: Department of Rheumatology, Clinical Immunology and Allergy, University Hospital of Heraklion, Heraklion, Greece Institute of Molecular Biology and Biotechnology, Foundation of Research and Technology-Hellas, Voutes, Heraklion, Greece.
authors.forename: G
authors.initials: G
authors.lastname: Bertsias
authors.affiliation: Institute of Molecular Biology and Biotechnology, Foundation of Research and Technology-Hellas, Voutes, Heraklion, Greece Biomedical Research Foundation of the Academy of Athens, Athens, Greece 4th Department of Internal Medicine, 'Attikon' University Hospital, Medical School, University of Athens, Athens, Greece Joint Academic Rheumatology Program, National and Kapodestrian University of Athens, Athens, Greece.
authors.forename: D T
authors.initials: DT
authors.lastname: Boumpas
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D017152:Antibodies, Antiphospholipid / Q000276:immunology; D003520:Cyclophosphamide / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D020945:Lupus Vasculitis, Central Nervous System / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D017152:Antibodies, Antiphospholipid; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide
keywords: Neuropsychiatric SLE; cyclophosphamide; efficacy; safety; treatment