RESUMO
The objective of this study was to investigate the efficacy and safety of anti-CD20 treatment in Hispanic patients with refractory systemic lupus erythematosus and to determine whether baseline parameters predict disease flare. Fifty-two patients with systemic lupus erythematosus, 13 with active lupus nephritis, eight with thrombocytopenia, three with leukocytopenia, 25 with severe musculoskeletal involvement and three with skin involvement) refractory to conventional therapy were treated with anti-CD20 treatment (rituximab; MabThera, Roche) plus ongoing immunosuppressive treatment. Disease activity was assessed monthly using the SLEDAI validated for the Mexican population with a follow-up period of 6 months. At 6 months of follow-up, significant clinical improvements were detected, with a reduction in the global SLEDAI validated for the Mexican population score. Five of the 13 patients with lupus nephritis (38.4%) had a complete renal response and five (38.4%) had a partial response. Rituximab was also effective in patients with autoimmune thrombocytopenia, inducing a significant increase in platelet counts (p = 0.012). Nineteen of 25 patients with severe musculoskeletal involvement had remission of arthritis. Only one of the three patients with skin involvement had no lesions at 6 months. Rituximab treatment also allowed a reduction of the oral prednisone dose in the majority of patients. No baseline predictors of flare were found. Treatment was discontinued after the first infusion in two patients due to serum sickness and in another due to pulmonary infection. In conclusion, the addition of rituximab to conventional immunosuppressive therapy may be an effective strategy for lupus nephritis, autoimmune thrombocytopenia and inflammatory polyarthritis in patients with refractory systemic lupus erythematosus.
Assuntos
Anticorpos Monoclonais/uso terapêutico , Antígenos CD20/efeitos dos fármacos , Fatores Imunológicos/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Adulto , Idoso , Anticorpos Monoclonais/efeitos adversos , Anticorpos Monoclonais/farmacologia , Anticorpos Monoclonais Murinos , Antígenos CD20/imunologia , Feminino , Seguimentos , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Fatores Imunológicos/efeitos adversos , Fatores Imunológicos/farmacologia , Imunossupressores/uso terapêutico , Estudos Longitudinais , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Prednisona/administração & dosagem , Prednisona/uso terapêutico , Estudos Retrospectivos , Rituximab , Índice de Gravidade de Doença , Resultado do Tratamento , Adulto JovemRESUMO
The objective of this study was to analyse the prevalence and characteristics of the main clinical and immunological manifestations at the onset and during the evolution of the disease in a cohort of patients from Latin America (mainly of mestizo origin) and to compare the Latin American with the European patients. Clinical and serological characteristics of 100 APS patients from Mexico and Ecuador were collected in a protocol form that was identical to that used to study the ;Euro-Phospholipid' cohort. The cohort consisted of 93 female patients (93.0%) and seven (7.0%) male patients. There were 91 mestizos (91.0%), seven whites (7.0%) and two Amerindians (2.0%). The most common manifestations were livedo reticularis (40.0%), migraine (35.0%), inferior extremity deep vein thrombosis (32.0%), thrombocytopenia (28.0%) and hemolytic anemia (20.0%). Several clinical manifestations were more prevalent in Latin American than in European patients and they included mainly neurological (migraine, transient global amnesia, acute ischemic encephalopathy, amaurosis fugax) and cutaneous (livedo reticularis, skin ulcerations, superficial cutaneous necrosis, multiple subungual splinter hemorrhages) manifestations as well as hemolytic anemia. The APS has a wide variety of clinical and immunological manifestations at the onset and during the evolution of the disease and the ethnic origin in addition to environmental and socioeconomic factors can modify the disease expression.
Assuntos
Síndrome Antifosfolipídica/epidemiologia , Síndrome Antifosfolipídica/imunologia , Adolescente , Adulto , Idoso , Síndrome Antifosfolipídica/patologia , Criança , Estudos de Coortes , Equador/epidemiologia , Europa (Continente)/epidemiologia , Feminino , Humanos , Masculino , México/epidemiologia , Pessoa de Meia-IdadeRESUMO
In 1992, an attempt to single out a different and important group of patients was adopted by introducing the term "catastrophic" antiphospholipid syndrome (APS) to describe their potentially life threatening clinical course, characterized by acute multiorgan failure (3 or more organ systems damaged). Patients previously described in the literature showed certain differences that appear to distinguish this minority group of patients with catastrophic APS from the majority of APS patients. We describe 8 patients with catastrophic APS, focusing especially on the possible extrinsic factors (mainly infections) that might trigger this "catastrophic" situation.
Assuntos
Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/etiologia , Adolescente , Adulto , Feminino , Humanos , Pessoa de Meia-IdadeRESUMO
An eight-year-old girl developed optic neuritis followed by primary Sjögren's syndrome confirmed by a lip biopsy. Glucocorticoid therapy combined during six months with monthly intravenous pulse cyclophosphamide ensured resolution of the sicca syndrome but failed to improve the visual impairment. This is the second pediatric case of optic neuritis associated with primary Sjögren's syndrome, and the first pediatric case in which optic neuritis was the only neurologic manifestation.