[Efficacy and safety of rituximab in the treatment of primary antiphospholipid syndrome: analysis of 24 cases from the bibliography review].
OBJECTIVE
Antiphospholipid syndrome (APS) is characterized by the presence of antiphospholipid antibodies (aPL) and thrombotic and/or obstetric manifestations. Patients without another associated autoimmune disease are considered to have primary APS. Some patients develop thrombosis recurrence despite anticoagulant treatment and some clinical features do not respond to standard therapy. Rituximab may be an alternative in these cases. We review the published scientific evidence on the use of rituximab in the treatment of primary APS.


METHODS
Description of a case and review of the literature with descriptive analysis of the demographic, clinical, and immunologic features, treatment and outcome of patients.


RESULTS
We identified 24 patients (15 women [62.5%]), with a mean age of 37.0 ± 13.4 years. The reasons for the use of rituximab were thrombocytopenia (41.7%), skin involvement (33.3%), neurologic and heart valve involvement (12.5%), hemolytic anemia (8.3%) and pulmonary and renal involvement (4.2%). Lupus anticoagulant was present in 72.7% of the cases, the IgG and IgM isotypes of anticardiolipin antibodies in 75 and 50%, respectively, and the anti-β2GPI (IgG e IgM) antibodies in 80% of patients. Thirteen (54.1%) patients received 2 doses of 1,000 mg of rituximab fortnightly, 10 (41.7%) 4 doses of 375 mg/m(2) weekly and one (4.2%) 8 doses of 375 mg/m(2) weekly. Eleven (45.8%) patients presented a complete clinical response, 7 (29.2%) a partial response and 6 (25%) did not respond to rituximab. Four patients with clinical improvement presented with aPL titer decrease and in one patient, aPL levels did not change. In one patient without clinical response, aPL remained positive. A clinical-immunologic dissociation existed in 2 additional cases.


CONCLUSIONS
The results obtained suggest a possible potential benefit of rituximab in the treatment of some clinical manifestations of primary APS such as thrombocytopenia, skin and heart valve involvement.
Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Hospital d'Igualada, Igualada, Barcelona, España.
Isaac
I
Pons
Servicio de Enfermedades Autoinmunes Sistémicas, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, España. Electronic address: gespino@clinic.cat.
Gerard
G
Espinosa
Servicio de Enfermedades Autoinmunes Sistémicas, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, España.
Ricard
R
Cervera
D000026:Abortion, Habitual / Q000209:etiology; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D017152:Antibodies, Antiphospholipid / Q000097:blood; D000925:Anticoagulants / Q000009:adverse effects / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D004341:Drug Evaluation; D004359:Drug Therapy, Combination; D005260:Female; D005313:Fetal Death / Q000209:etiology; D006474:Hemorrhagic Disorders / Q000139:chemically induced; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011247:Pregnancy; D012189:Retrospective Studies; D000069283:Rituximab / Q000009:adverse effects / Q000627:therapeutic use*; D019851:Thrombophilia / Q000188:drug therapy / Q000209:etiology; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D017152:Antibodies, Antiphospholipid; D000925:Anticoagulants; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000069283:Rituximab
Antiphospholipid syndrome; Heart valve disease; Primary antiphospholipid syndrome; Rituximab; Síndrome antifosfolipídico; Síndrome antifosfolipídico primario; Thrombocytopenia; Trombocitopenia; Valvulopatía