Τίτλος:
Cyclophosphamide in combination with glucocorticoids for severe neuropsychiatric systemic lupus erythematosus: A retrospective, observational two-centre study
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
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. © SAGE Publications.
Συγγραφείς:
Fanouriakis, A.
Pamfil, C.
Sidiropoulos, P.
Damian, L.
Flestea, A.
Gusetu, G.
Rednic, S.
Bertsias, G.
Boumpas, D.T.
Περιοδικό:
Lupus Science and Medicine
Εκδότης:
SAGE Publications Ltd
Λέξεις-κλειδιά:
azathioprine; cyclophosphamide; glucocorticoid; gonadorelin derivative; methylprednisolone; mycophenolate mofetil; phospholipid antibody; rituximab; cyclophosphamide; glucocorticoid; immunosuppressive agent; phospholipid antibody, adolescent; adult; aged; Article; cerebrovascular disease; clinical article; cranial neuropathy; disease severity; drug efficacy; drug safety; drug treatment failure; female; follow up; gonadal disease; hepatitis B; human; maintenance therapy; male; miliary tuberculosis; neuropsychiatric systemic lupus erythematosus; neuropsychiatric systemic lupus erythematosus; observational study; polyneuropathy; priority journal; psychosis; relapse; respiratory tract infection; retrospective study; secondary amenorrhea; seizure; septic shock; systemic lupus erythematosus; treatment outcome; treatment response; clinical trial; combination drug therapy; immunology; Lupus Vasculitis, Central Nervous System; middle aged; multicenter study; pathophysiology; severity of illness index; young adult, Adolescent; Adult; Aged; Antibodies, Antiphospholipid; Cyclophosphamide; Drug Therapy, Combination; Female; Follow-Up Studies; Glucocorticoids; Humans; Immunosuppressive Agents; Lupus Vasculitis, Central Nervous System; Male; Middle Aged; Retrospective Studies; Severity of Illness Index; Treatment Outcome; Young Adult
DOI:
10.1177/0961203315622821
