RESUMO
The different sets of criteria for diagnosis or classification of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) lead to numerous overlapping and reclassified diagnoses in clinical practice. We designed this study to assess the difficulties in classifying patients with AAV. As a secondary objective, different variables were tested to predict prognosis. We conducted a retrospective chart review in a Western Spain multicentre survey. A total of 115 adult patients diagnosed with AAV from 2002 to 2013 and followed for at least 3 years were included. They were classified according to (1) Chapel Hill Consensus Conference (CHCC), (2) European Medicines Agency algorithm and (3) French Vasculitis Study Group/European Vasculitis Society phenotypes. Fifty-three patients (46%) had neither distinctive histopathological data of a single AAV definition nor any surrogate markers for granulomatous inflammation and thus did not fulfill any diagnostic criteria. Ocular, ear, nose, throat, skin, and lung involvement were more frequent with proteinase 3 (PR3) antibodies, whereas peripheral neuropathy was more frequent with myeloperoxidase (MPO) antibodies. When the disease was severe at diagnosis, the HR for mortality was 10.44. When induction treatment was not given in accordance with the guidelines, the HR for mortality was 4.00. For maintenance treatment, the HR was 5.49 for mortality and 2.48 for relapse. AAV classification is difficult because many patients had neither specific clinical data nor distinctive histological features of a single CHCC definition. A structured clinical assessment of patient severity is the best tool to guide the management of AAV.
Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/classificação , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/fisiopatologia , Mortalidade , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Síndrome de Churg-Strauss/classificação , Síndrome de Churg-Strauss/imunologia , Síndrome de Churg-Strauss/patologia , Síndrome de Churg-Strauss/fisiopatologia , Epistaxe/imunologia , Epistaxe/patologia , Epistaxe/fisiopatologia , Oftalmopatias/imunologia , Oftalmopatias/patologia , Oftalmopatias/fisiopatologia , Feminino , Gastroenteropatias/imunologia , Gastroenteropatias/patologia , Gastroenteropatias/fisiopatologia , Granulomatose com Poliangiite/classificação , Granulomatose com Poliangiite/imunologia , Granulomatose com Poliangiite/patologia , Granulomatose com Poliangiite/fisiopatologia , Humanos , Hipertensão/imunologia , Hipertensão/patologia , Hipertensão/fisiopatologia , Nefropatias/imunologia , Nefropatias/patologia , Nefropatias/fisiopatologia , Falência Renal Crônica/fisiopatologia , Pneumopatias/imunologia , Pneumopatias/patologia , Pneumopatias/fisiopatologia , Masculino , Poliangiite Microscópica/classificação , Poliangiite Microscópica/imunologia , Poliangiite Microscópica/patologia , Poliangiite Microscópica/fisiopatologia , Pessoa de Meia-Idade , Mieloblastina/imunologia , Doenças do Sistema Nervoso Periférico/imunologia , Doenças do Sistema Nervoso Periférico/patologia , Doenças do Sistema Nervoso Periférico/fisiopatologia , Peroxidase/imunologia , Prevenção Primária , Prognóstico , Modelos de Riscos Proporcionais , Recidiva , Estudos Retrospectivos , Índice de Gravidade de Doença , Sinusite/imunologiaRESUMO
La púrpura trombocitopénica inmune es un trastorno inmunológico caracterizado por la disminución del recuento de plaquetas de forma transitoria o persistente. Puede ser primaria o secundaria en función a la presencia o ausencia de trastornos asociados. Algunos casos se asocian con la infección por Helicobacter pylori, y en estos pacientes el tratamiento erradicador tiene impacto positivo en el recuento de plaquetas. Presentamos cuatro pacientes adultos (dos varones y dos mujeres) con púrpura trombocitopénica inmune secundaria a infección por Helicobacter pylori, los cuales mostraron respuestas favorables al tratamiento erradicador, con mejoría de los recuentos plaquetarios y remisión de las manifestaciones hemorrágicas. Consideramos que debe realizarse la detección rutinaria de esta bacteria en pacientes con púrpura trombocitopénica inmune debido a la elevada prevalencia de la infección en nuestra región y el impacto favorable del tratamiento en el recuento de plaquetas
Immune thrombocytopenic purpura is an immune disorder characterized by a decrease in platelet count transiently or persistently. It can be primary or secondary depending on the presence or absence of associated disorders. Some cases are associated with Helicobacter pyloriinfection, and in these patients eradication treatment has a positive impact on the platelet count. We present four adult patients (two males and two females) with immune thrombocytopenic purpura secondary to Helicobacter pylori infection, which showed favorable responses to eradication treatment, with improvement of platelet counts and remission of hemorrhagic manifestations. We believe that routine detection of this bacterium should be performed in patients with immune thrombocytopenic purpura due to the high prevalence of infection in our region and favorable impact of treatment on the platelet count.
Assuntos
Helicobacter pylori , Epistaxe/imunologiaRESUMO
Objective Epistaxis is a primary complaint in 90% to 96% of patients with hereditary hemorrhagic telangiectasia (HHT). Numerous surgical and medical treatments aim to decrease the frequency and severity of epistaxis in this patient population. Bevacizumab is a recombinant, humanized monoclonal antibody to vascular endothelial growth factor, an angiogenic factor elevated in HHT. It has been used in several forms to treat epistaxis in HHT but thus far, evidence-based recommendations are limited. Study Design Systematic review with evidence-based recommendations. Methods A systematic review of the literature following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was performed using Embase, MEDLINE, MEDLINE In-Process/Epub, and Cochrane databases. English language abstracts were reviewed for relevance. Results Eleven manuscripts met inclusion criteria and were analyzed. Submucosal injection, submucosal injection plus laser coagulation, intravenous (IV), and topical formulations of bevacizumab were evaluated for their therapeutic impact on epistaxis in patients with HHT. Three randomized controlled trials failed to show topical bevacizumab to be more effective in controlling epistaxis than saline or other moisturizers. Conclusions The use of submucosal and IV bevacizumab shows promise, but further study is necessary to determine the true efficacy in the treatment of epistaxis as only grade C level exists currently. Based on the available literature, the use of topical bevacizumab is not recommended (grade B).
Assuntos
Inibidores da Angiogênese/uso terapêutico , Bevacizumab/uso terapêutico , Epistaxe/prevenção & controle , Imunoterapia/métodos , Telangiectasia Hemorrágica Hereditária/terapia , Animais , Epistaxe/etiologia , Epistaxe/imunologia , Medicina Baseada em Evidências , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , Telangiectasia Hemorrágica Hereditária/complicações , Telangiectasia Hemorrágica Hereditária/imunologia , Fator A de Crescimento do Endotélio Vascular/imunologiaRESUMO
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia and severe, recurrent epistaxis is a common clinical phenotype associated with HHT. An intranasal treatment regime of diluted Avastin™ (Bevacizumab; recombinant humanized anti-vascular epithelial growth factor immunoglobin G1) using apulsatile nasal irrigator has proven efficacious in clinical practice. However, concerns regarding the stability of Avastin™ following dilution and prolonged storage in standard containers used for drug delivery, such as polyethylene bottles, have so far prevented a more widespread clinical use. Compatibility with the preservative benzalkonium chloride was also unknown. OBJECTIVE: This study aimed at determining, whether dilution, prolonged refrigerated storage and the presence of the preservative benzalkonium chloride - as required for novel Avastin™ formulations - affected the biochemical and electrochemical properties of the drug. METHODS: We performed a detailed biochemical and electrochemical analysis of Avastin™, including native and sodium dodecyl sulfate polyacrylamide gel electrophoresis, enzyme-linked immunosorbent assay and isoelectric focusing. RESULTS: We did not detect any evidence of degeneration or aggregation following dilution and prolonged, refrigerated storage or from the presence of benzalkonium chloride. All biochemical and electrochemical properties of Avastin™ after dilution and prolonged, refrigerated storage were undistinguishable from control. CONCLUSIONS: Our data provide important insight into the stability of Avastin™ and allow the consideration of novel Avastin™ formulations, including its use in a metered-dose nasal spray for the treatment of HHT and other applications.
Assuntos
Receptores de Activinas Tipo II/imunologia , Inibidores da Angiogênese/imunologia , Anticorpos Monoclonais Humanizados/imunologia , Compostos de Benzalcônio/química , Epistaxe/tratamento farmacológico , Telangiectasia Hemorrágica Hereditária/tratamento farmacológico , Fator A de Crescimento do Endotélio Vascular/antagonistas & inibidores , Receptores de Activinas Tipo II/genética , Administração Intranasal , Inibidores da Angiogênese/administração & dosagem , Inibidores da Angiogênese/uso terapêutico , Anticorpos Monoclonais Humanizados/administração & dosagem , Anticorpos Monoclonais Humanizados/uso terapêutico , Bevacizumab , Estabilidade de Medicamentos , Eletroforese em Gel de Poliacrilamida , Ensaio de Imunoadsorção Enzimática , Epistaxe/complicações , Epistaxe/imunologia , Epistaxe/metabolismo , Humanos , Focalização Isoelétrica , Sprays Nasais , Polietileno/química , Conservantes Farmacêuticos/química , Telangiectasia Hemorrágica Hereditária/complicações , Telangiectasia Hemorrágica Hereditária/imunologia , Telangiectasia Hemorrágica Hereditária/metabolismo , Fator A de Crescimento do Endotélio Vascular/imunologiaRESUMO
Silver nitrate cautery is frequently used to control epistaxis. Although relatively free of side effects, we have encountered a case of a severe mucocutaneous reaction to silver nitrate cautery to the nose. The pathogenesis of this adverse effect is discussed.