Anti-CD20 therapy in patients with refractory systemic lupus erythematosus: a longitudinal analysis of 52 Hispanic patients.

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.

Antiphospholipid syndrome in Latin American patients: clinical and immunologic characteristics and comparison with European patients.

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.

Catastrophic antiphospholipid syndrome: clinical description and triggering factors in 8 patients.

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.

[A quality of life study in women with systemic lupus erythematosus and its relation to disease activity]. / Estudio de la calidad de vida en mujeres con lupus eritematoso sistémico y su relación con la actividad de la enfermedad.

OBJECTIVE: To determined in a prospective study the quality of life (QoL) in the systemic lupus erythematosus (SLE) females. We also studied the relationship between QoL and the activity of lupus disease. METHODS: We studied 67 consecutive SLE females. To study the QoL we administered the Medical Outcomes Study Short form Scale SF-36 (MOS-SF36). This questionnaire is composted for 36 question and comprises eighth subscales of QoL. We also measured the activity with the Mexico-SLE Disease Activity Index (MEX-SLEDAI). RESULTS: The mean age of the 67 SLE patients at disease onset was 38.3 +/- 14 years, and mean duration of disease was 66 +/- 42 months. The values of QoL answers were lower. No relationship was found between the age of patients and QoL. The subscales with poor punctuation were the general heath and the role physical. Multiple variate analyses showed that activity was associated with vitality (r = 0.36; p = 0.003). CONCLUSIONS: The MOS-SF36 questionnaire indicate that SLE females patients had lower results about her quality of life.

Estudio de la calidad de vida en mujeres con lupus eritematoso sistémico y su relación con la actividad de la enfermedad / Study the relationship between quality life and the activity of systemic lupus erythematosus in females

Fundamento: El pronóstico de los pacientes con lupus eritematoso sistémico (LES) ha mejorado en los últimos años. Ello ha incrementado el interés por conocer la calidad de vida (CDV) de estos pacientes. Se estudia de manera prospectiva la CDV en un grupo de mujeres con LES y se examina la relación entre la CDV y el grado de actividad de la enfermedad. Métodos: Se estudiaron 67 mujeres consecutivas con LES y se valoró la CDV mediante el Medical Outcomes Study Short form Scale SF-36 (MOS-SF36), constituido por 36 preguntas que valoran ocho dimensiones de CDV, y la presencia de actividad con el México-SLE Disease Activity Index (MEX-SLEDAI). Resultados: La edad media al inicio de la enfermedad fue 38,3ñ14 años. El tiempo medio de seguimiento era de 66 ñ 42 meses. Las pacientes dieron valores bajos de CDV, independientemente de su edad. Las subescalas que recibieron la peor puntuación fueron la de salud general y la del rol físico. EL análisis de correlación múltiple, solo mostró una asociación entre la actividad y la vitalidad (r=0,36; p=0,003). Conclusiones: Se confirma mediante el MOS-SF36 que las mujeres con LES cursan con calificaciones bajas de calidad de vida (AU)

Nodular regenerative hyperplasia of the liver and antiphospholipid antibodies: report of two cases and review of the literature.

Nodular regenerative hyperplasia of the liver (NRHL) is a rare disorder characterized by diffuse micronodular transformation of the hepatic parenchyma without fibrous septa between the nodules. This condition appears to be associated in many occasions with systemic autoimmune diseases. We describe two new patients with NRHL in whom antiphospholipid antibodies (aPL) were detected in their sera and review the few similar cases reported previously. We also discuss the possible relationship between aPL and NRHL and suggest that these antibodies may play a role in the pathogenesis of some cases of NRHL, specially those with an associated antiphospholipid syndrome.

Optic neuropathy in a child with primary Sjögren's syndrome.

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.

[Influence of coronary risk factors in secondary prevention: tobacco]. / Influencia de los factores de riesgo coronario en prevención secundaria: tabaco.

Tobacco smoking is a strong independent factor for atherosclerotic disease, equivalent to hypertension or high cholesterol levels. Middle age people are especially involved, with a mortality rate of about 20% as a consequence of smoking, and a mean loss of life expectancy of 20 years. There is a positive correlation between smoking and severity of atherosclerotic disease in the coronary and cerebral arteries, and the aorta. It has been shown that smoking cessation clearly enhances the prognosis of patients with myocardial infarction. Twice a increase in mortality rate has been found among nonstopping smokers compared with those who stopped smoking eight years after myocardial infarction. In addition, progression of atherosclerosis as shown by angiography is slowed by stopping to smoke. As the coronary risk factors act in a synergistic way, a comprehensive approach to the patient is recommended, especially in smokers with myocardial infarction. It is justified an intensive intervention because of the advantages in this population. The physician should clearly communicate to the patient the need of stopping to smoke, which carries sometimes as beneficial effects as other interventions. A wise use of replacement therapy with transdermal nicotine, together with other useful measures, allows us to manage patients with a broad margin of safety, especially in coronary patients, who win most benefit from ceasing to smoke.