ABSTRACT
The objective of this study was to evaluate the 25-year outcome of patients with primary Sjögren's syndrome (pSS). One hundred and fifty-two patients diagnosed with pSS (American-European classification criteria) were retrospectively and descriptively analysed (1986-2011). Of all 152 patients, 55.9% were alive, 18.4% had died and 25.7% discontinued follow-up (mostly due to old age). Malignancy affected 28.3% and non-Hodgkin's lymphoma (NHL) affected 10.5%. The adjusted risk for development of NHL was an odds ratio (OR) of 10.5 (95% confidence interval [CI]: 3.05-36.42) in patients with vasculitis (p<0.001), and OR 3.4 (95% CI 1.05-11.2) in the presence of glandular complications (parotid swelling, lymphadenopathy) (p < 0.041). Seventy-five patients (49.3%) developed other autoimmune diseases (autoimmune thyroid disease [15.8%], pulmonary fibrosis [7.2%] and vasculitis [10.5%]). Although the course of pSS is relatively benign, over 25 years patients experience more clinical complications than previously described. In addition, vasculitis and glandular manifestations were significant predictors for NHL.
Subject(s)
Autoimmune Diseases/epidemiology , Lymphoma, Non-Hodgkin/epidemiology , Sjogren's Syndrome/epidemiology , Adult , Aged , Cohort Studies , Female , Follow-Up Studies , Humans , Lymphatic Diseases/etiology , Male , Middle Aged , Neoplasms/epidemiology , Odds Ratio , Prognosis , Pulmonary Fibrosis/epidemiology , Retrospective Studies , Risk Factors , Sjogren's Syndrome/complications , Sjogren's Syndrome/mortality , Thyroiditis, Autoimmune/epidemiology , Vasculitis/epidemiologyABSTRACT
Fundamento y objetivo: Comunicamos nuestra experiencia con rituximab más ciclofosfamida en el tratamiento de pacientes con miopatía inflamatoria idiopática refractaria. Pacientes y método: Estudio prospectivo abierto no controlado sobre 17 pacientes.Resultados: Evaluación cumplimentada tras 1, 6 y 12 meses en el 95,2, el 85,7 y el 52,4% de los ciclos, respectivamente. Remisión total o parcial tras 1, 6 y 12 meses en el 65, el 100 y el 63,6% de los ciclos evaluados, respectivamente. Depleción absoluta de linfocitos B en sangre periférica en los 18 casos con datos disponibles, con tendencia a la normalización tras 6 a 12 meses. Hubo 5 recaídas; la mediana de tiempo hasta la recaída fue de 11 meses; hubo repetición del tratamiento en 4 casos. Cuatro pacientes tenían afectación respiratoria; uno (etiología multifactorial) no mejoró, pero sí los otros 3, con neumopatía intersticial aislada o asociada a debilidad muscular respiratoria. Hubo 5 pacientes con anticuerpos anti-Jo-1 positivos (6 ciclos), con respuesta al tratamiento superponible al resto. Se observaron escasos efectos adversos; solo cabe destacar un caso de meningitis por Corynebacterium, con buena evolución.Conclusiones: El rituximab parece una alternativa válida en el tratamiento de pacientes con polimiositis o dermatomiositis resistentes (AU)
Background and objective: We report our experience with rituximab plus cyclophosphamide in the treatment of patients with resistant idiopathic inflammatory myopathies. Patients and method: Open-label uncontrolled prospective sudy on 17 patients.Results: Evaluation was completed after 1, 6 and 12 months in 952, 857 y 524% of cycles, respectively. Total or partial remission was achieved after 1, 6 and 12 months in 65, 100 y 636% of evaluated cycles, respectively. Absolute depletion of B lymphocites from peripheral blood was found in the 18 cases with available data. There were 5 relapses; median of time to relapse: 11 months; treatment was repeated in 4. Four patients (6 cycles) had impaired pulmonary function; one (with a multifactorial etiology) did not improve but the other 3, with interstitial pneumonia associated or not with respiratory muscle weakness, did. Five patients with positive anti-Jo-1 antibodies (6 cycles) displayed similar results. The only adverse event observed was a case of meningitis caused by Corynebacterium, with good results. Conclusion: Rituximab seems a valid alternative for the treatment of patients with resistant polymyositis or dermatomyosytis (AU)
Subject(s)
Humans , Cyclophosphamide/therapeutic use , Antibodies, Monoclonal/therapeutic use , Myositis/drug therapy , Prospective Studies , Drug Therapy, Combination , Drug Resistance , Treatment OutcomeABSTRACT
BACKGROUND AND OBJECTIVE: We report our experience with rituximab plus cyclophosphamide in the treatment of patients with resistant idiopathic inflammatory myopathies. PATIENTS AND METHOD: Open-label uncontrolled prospective study on 17 patients. RESULTS: Evaluation was completed after 1, 6 and 12 months in 95'2, 85'7 y 52'4% of cycles, respectively. Total or partial remission was achieved after 1, 6 and 12 months in 65, 100 y 63'6% of evaluated cycles, respectively. Absolute depletion of B lymphocites from peripheral blood was found in the 18 cases with available data. There were 5 relapses; median of time to relapse: 11 months; treatment was repeated in 4. Four patients (6 cycles) had impaired pulmonary function; one (with a multifactorial etiology) did not improve but the other 3, with interstitial pneumonia associated or not with respiratory muscle weakness, did. Five patients with positive anti-Jo-1 antibodies (6 cycles) displayed similar results. The only adverse event observed was a case of meningitis caused by Corynebacterium, with good results. CONCLUSION: Rituximab seems a valid alternative for the treatment of patients with resistant polymyositis or dermatomyosytis.
