ABSTRACT
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.
Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Animals , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Antibodies, Antineutrophil Cytoplasmic , Disease Models, Animal , Humans , Inflammation , Mice , NeutrophilsABSTRACT
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.
Subject(s)
Connective Tissue Diseases , Hypertension, Pulmonary , Pulmonary Arterial Hypertension , Connective Tissue Diseases/complications , Connective Tissue Diseases/drug therapy , Connective Tissue Diseases/epidemiology , Humans , Hypertension, Pulmonary/drug therapy , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/etiology , Prostaglandins , Retrospective StudiesSubject(s)
Abdominal Abscess/complications , Bile Ducts/injuries , Cholecystectomy, Laparoscopic/adverse effects , Gallstones/complications , Postoperative Complications/etiology , Abdominal Abscess/diagnosis , Aged , Fever/etiology , Gallstones/diagnosis , Humans , Male , Tomography, X-Ray Computed , Weight LossSubject(s)
Antineoplastic Agents, Immunological/therapeutic use , Lymphoma, Large B-Cell, Diffuse/diagnostic imaging , Lymphoma, Large B-Cell, Diffuse/drug therapy , Skin/pathology , Dyspnea/etiology , Female , Fever/etiology , Genes, bcl-2 , Genes, myc , Humans , Middle Aged , Positron Emission Tomography Computed TomographySubject(s)
Abdominal Neoplasms/chemically induced , Adrenal Gland Neoplasms/chemically induced , Leukemia, Experimental/chemically induced , Lung Neoplasms/chemically induced , Mammary Neoplasms, Experimental/chemically induced , Phenylenediamines/adverse effects , Pituitary Neoplasms/chemically induced , Adenocarcinoma/chemically induced , Adenofibroma/chemically induced , Adenoma/chemically induced , Animals , Carcinogens , Chemical Phenomena , Chemistry , Female , Lymphoma, Large B-Cell, Diffuse/chemically induced , Male , Mice , Neoplasms, Experimental/chemically induced , RatsSubject(s)
Chemical Industry , Occupational Diseases/prevention & control , Toluidines/adverse effects , Urinary Bladder Neoplasms/prevention & control , Adolescent , Adult , Animals , Environmental Exposure , Female , Humans , Male , Maximum Allowable Concentration , Mice , Mice, Inbred C57BL , Middle Aged , Neoplasms, Experimental/chemically induced , Occupational Diseases/chemically induced , Rats , Time Factors , Urinary Bladder Neoplasms/chemically inducedABSTRACT
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.