RESUMEN
Anticytoplasmic neutrophil antibodies (ANCA)-associated vasculitis (AAV) are rare systemic immune-mediated diseases characterized by small vessel necrotizing vasculitis and/or respiratory tract inflammation. Over the last 2 decades, anti-MPO vasculitis mouse model has enlightened the role of ANCA, neutrophils, complement activation, T helper cells (Th1, Th17) and microbial agents. In humans, CD4T cells have been extensively studied, while the dramatic efficacy of rituximab demonstrated the key role of B cells. Many areas of uncertainty remain, such as the driving force of GPA extra-vascular granulomatous inflammation and the relapse risk of anti-PR3 AAV pathogenesis. Animal models eventually led to identify complement activation as a promising therapeutic target. New investigation tools, which permit in depth immune profiling of human blood and tissues, may open a new era for the studying of AAV pathogenesis.
Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Animales , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/tratamiento farmacológico , Anticuerpos Anticitoplasma de Neutrófilos , Modelos Animales de Enfermedad , Humanos , Inflamación , Ratones , NeutrófilosRESUMEN
INTRODUCTION: Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue disease (CTD). Data on use of prostanoids in this particular subset of patients are lacking. We aimed to describe the characteristics of patients with PAH-CTD treated with prostanoids and the outcomes under treatment. METHODS: In this multicenter retrospective study, all patients treated with prostanoids since 2006 were included. Data on PAH and CTD were collected at the time of prostanoid introduction and under treatment. RESULTS: Twenty-one patients were included, of whom 20 (95%) had limited cutaneous systemic sclerosis. Nineteen patients were treated with oral monotherapy or combination before addition of prostanoid. Treprostinil was the most used molecule (57% of patients). At the time of prostanoid introduction, 90% of patients were considered at high risk for death. Among patients who had right heart catheterization during follow-up, there was no significant difference in haemodynamics. No extrarespiratory worsening of the CTD was reported. The 1-year survival under prostanoid was 62%. In univariate analysis, NYHA functional class was associated with survival under treatment. CONCLUSION: This study provides original data on use of prostanoids in a cohort consisting mainly of systemic sclerosis. It underlines the difficulty to achieve a standardized assessment in this subset of patients. Safety profile was comparable with data reported in idiopathic PAH.
Asunto(s)
Enfermedades del Tejido Conjuntivo , Hipertensión Pulmonar , Hipertensión Arterial Pulmonar , Enfermedades del Tejido Conjuntivo/complicaciones , Enfermedades del Tejido Conjuntivo/tratamiento farmacológico , Enfermedades del Tejido Conjuntivo/epidemiología , Humanos , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/epidemiología , Hipertensión Pulmonar/etiología , Prostaglandinas , Estudios RetrospectivosAsunto(s)
Absceso Abdominal/complicaciones , Conductos Biliares/lesiones , Colecistectomía Laparoscópica/efectos adversos , Cálculos Biliares/complicaciones , Complicaciones Posoperatorias/etiología , Absceso Abdominal/diagnóstico , Anciano , Fiebre/etiología , Cálculos Biliares/diagnóstico , Humanos , Masculino , Tomografía Computarizada por Rayos X , Pérdida de PesoAsunto(s)
Antineoplásicos Inmunológicos/uso terapéutico , Linfoma de Células B Grandes Difuso/diagnóstico por imagen , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Piel/patología , Disnea/etiología , Femenino , Fiebre/etiología , Genes bcl-2 , Genes myc , Humanos , Persona de Mediana Edad , Tomografía Computarizada por Tomografía de Emisión de PositronesAsunto(s)
Neoplasias Abdominales/inducido químicamente , Neoplasias de las Glándulas Suprarrenales/inducido químicamente , Leucemia Experimental/inducido químicamente , Neoplasias Pulmonares/inducido químicamente , Neoplasias Mamarias Experimentales/inducido químicamente , Fenilendiaminas/efectos adversos , Neoplasias Hipofisarias/inducido químicamente , Adenocarcinoma/inducido químicamente , Adenofibroma/inducido químicamente , Adenoma/inducido químicamente , Animales , Carcinógenos , Fenómenos Químicos , Química , Femenino , Linfoma de Células B Grandes Difuso/inducido químicamente , Masculino , Ratones , Neoplasias Experimentales/inducido químicamente , RatasAsunto(s)
Industria Química , Enfermedades Profesionales/prevención & control , Toluidinas/efectos adversos , Neoplasias de la Vejiga Urinaria/prevención & control , Adolescente , Adulto , Animales , Exposición a Riesgos Ambientales , Femenino , Humanos , Masculino , Concentración Máxima Admisible , Ratones , Ratones Endogámicos C57BL , Persona de Mediana Edad , Neoplasias Experimentales/inducido químicamente , Enfermedades Profesionales/inducido químicamente , Ratas , Factores de Tiempo , Neoplasias de la Vejiga Urinaria/inducido químicamenteRESUMEN
Under study was the urine from 22 workers being in long-term contact with direct azo dyes (direct black 3, direct diazo black, direct pure blue, direct light fast KU). Benzidine was found in the urine of 8 workers and dianisidine--in 3. Consequently, there occurs metabolic decomposition of the dyes concerned to free blastomogenic agents.