RESUMO
Vasculitides associated with anti-neutrophil cytoplasmic antibodies are heterogeneous, systemic, low prevalence and high morbidity and mortality entities. They include granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. In Latin America, there are few descriptive registries of these patients. The objective of the study was to describe the demographic and clinical characteristics and in-hospital morbidity and mortality of patients with vasculitis associated with anti-neutrophil cytoplasmic antibodies in a university hospital in Colombia. This was a cross-sectional descriptive study. We performed computer searches with terms related to patients with anti-neutrophil cytoplasmic antibody-associated vasculitis, between 2005 and 2017 who met the American College of Rheumatology classification criteria for vasculitis associated with anti-neutrophil cytoplasmic antibodies, and their clinical and laboratory characteristics. One hundred and six patients with anti-neutrophil cytoplasmic antibody-associated vasculitis were included in the study. The average age was 55 years, and 57.5% were women. In 68.8% of the cases, the diagnosis was made during hospitalization, with an average hospital stay of 16.6 days (± 12.22). The distribution by type of vasculitis was: granulomatosis with polyangiitis 52%, microscopic polyangiitis 45.2% and eosinophilic granulomatosis with polyangiitis 1.8%. Alveolar hemorrhage occurred in 35% of patients; 20.7% had variable renal involvement, of which 53.8% progressed to advanced kidney disease. Treatment included glucocorticoids 91.5%, cyclophosphamide 62.2%, plasmapheresis 14.1%, and 41.5% required renal replacement therapy. In-hospital mortality was 16.5%, Sepsis was the most common cause of death. We present clinical information on a group of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis; renal involvement was the the most common type of affectation. Both the clinical and serological characteristics of our registry were similar to those described in other Latin American and European cohorts, and a lower in-hospital mortality rate was evidenced.
Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/mortalidade , Adulto , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/fisiopatologia , Anticorpos Anticitoplasma de Neutrófilos/análise , Colômbia/epidemiologia , Estudos Transversais , Feminino , Mortalidade Hospitalar , Humanos , Nefropatias/etiologia , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Sistema de RegistrosAssuntos
Endoscopia do Sistema Digestório/métodos , Gastroenteropatias , Granulomatose com Poliangiite , Administração dos Cuidados ao Paciente/métodos , Adulto , Anticorpos Anticitoplasma de Neutrófilos/análise , Biópsia/métodos , Diagnóstico Diferencial , Gastroenteropatias/etiologia , Gastroenteropatias/patologia , Gastroenteropatias/fisiopatologia , Gastroenteropatias/terapia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/fisiopatologia , Granulomatose com Poliangiite/terapia , Humanos , Masculino , Recidiva , Índice de Gravidade de DoençaRESUMO
Clinical and histological factors have been identified as predictors of early and late renal outcome in ANCA-associated vasculitides (AAV). The presence and severity of kidney involvement at diagnosis are associated with poor prognosis in both patient and renal survival. Histologic findings remain the gold standard for diagnosing patients with AAV. In order to quantify the extent of the morphological parameters in the renal biopsies and to identify the histopathological lesions that predict renal outcome, several scoring systems have been proposed to systematically assess kidney biopsies in AAV. Renal pathologists from an international working group proposed in 2010 a new histopathological classification. This scheme comprises four general categories, based on the predominance of the glomerular histological lesions: focal (≥50% normal glomeruli); crescentic (≥50% glomeruli with cellular crescents); mixed (<50% normal, <50% crescentic, <50% globally sclerotic glomeruli), and sclerotic (≥50% globally sclerotic glomeruli). This article reviews the background and the main studies that have validated the histopathologic classification of ANCA-associated glomerulonephritis, the conclusions derived from these studies, and the perspectives for the assessment of renal outcome in AAV.
Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Glomerulonefrite/classificação , Glomerulonefrite/patologia , Rim/patologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Anticorpos Anticitoplasma de Neutrófilos/análise , HumanosRESUMO
The Henoch-Schönlein purpura is the vasculitis associated with deposits of immunoglobulin A in small vessels. Its association with cytoplasmic antineutrophil cytoplasmic antibodies is possible, but rare. This vasculitis is uncommon in adults and the main clinic manifestations are purpuric lesions in lower limbs with gastrointestinal symptoms and renal involvement. The present work describes a rare case of Henoch-Schönlein purpura in an adult with cytoplasmic antineutrophil cytoplasmic antibodies.
Assuntos
Anticorpos Anticitoplasma de Neutrófilos/análise , Vasculite por IgA/diagnóstico , Técnica Direta de Fluorescência para Anticorpo , Humanos , Masculino , Adulto JovemRESUMO
Abstract: The Henoch-Schönlein purpura is the vasculitis associated with deposits of immunoglobulin A in small vessels. Its association with cytoplasmic antineutrophil cytoplasmic antibodies is possible, but rare. This vasculitis is uncommon in adults and the main clinic manifestations are purpuric lesions in lower limbs with gastrointestinal symptoms and renal involvement. The present work describes a rare case of Henoch-Schönlein purpura in an adult with cytoplasmic antineutrophil cytoplasmic antibodies.
Assuntos
Humanos , Masculino , Adulto Jovem , Vasculite por IgA/diagnóstico , Anticorpos Anticitoplasma de Neutrófilos/análise , Técnica Direta de Fluorescência para AnticorpoRESUMO
The Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with IgA immune complex deposition. The presentation in adults is rare and severe. Reported cases of HSP in patients infected with HIV are scarce. Neutrophil cytoplasmic antibodies (ANCA) are commonly found in other systemic vasculitis, but rarely in HSP and even more unusual the perinuclear pattern. Beside small vessel vasculitis, positivity of ANCA can be detected in a number of different pathological conditions in association with infectious processes, including HIV, or cocaine use, and especially the pattern of ANCA-p, associated with drugs, inflammatory bowel or autoimmune diseases. We report the case of a 35 years old man with toxic habits (cocaine, marijuana) who consulted for abdominal pain, hematochezia and purpura on lower extremities, and later fever, joint pain and progression of purpura associated with nephritic syndrome and ANCA-p (+). During hospitalization HIV infection was detected. Renal biopsy showed IgA nephropathy with favorable response to corticosteroid and antiproteinuric treatment. The communication of the case is due to the rarity of the presentation and therapeutic diagnostic challenge. It remains to elucidate the role of ANCA in the pathogenesis and management of adult PSH.
Assuntos
Anticorpos Anticitoplasma de Neutrófilos , Transtornos Relacionados ao Uso de Cocaína , Glomerulonefrite por IGA , Infecções por HIV , Vasculite por IgA , Adulto , Antibacterianos/uso terapêutico , Anticorpos Anticitoplasma de Neutrófilos/análise , Transtornos Relacionados ao Uso de Cocaína/complicações , Glomerulonefrite por IGA/complicações , Glucocorticoides/uso terapêutico , Infecções por HIV/complicações , Infecções por HIV/diagnóstico , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/tratamento farmacológico , Vasculite por IgA/imunologia , Masculino , Abuso de Maconha/complicações , Metilprednisolona/uso terapêuticoRESUMO
La púrpura de Schönlein-Henoch (PSH) es una vasculitis de pequeños vasos con depósito de inmunocomplejos de IgA. La presentación en adultos es rara y grave. Los casos comunicados de PSH en pacientes infectados por HIV son escasos. Los anticuerpos anticitoplasma de neutrófilos (ANCA), detectados por inmunofluorescencia indirecta (IFI), se encuentran comúnmente en otras vasculitis sistémicas primarias pero rara vez en PSH y es aún más inusual el patrón perinuclear (ANCA-p). Por otra parte, la positividad de los ANCA puede ser detectada en una serie de condiciones patológicas distintas de las vasculitis de pequeños vasos en asociación a procesos infecciosos, incluyendo el HIV, o a consumo de cocaína, y en especial el patrón de ANCA-p vinculado a medicamentos o enfermedades autoinmunes e inflamatorias intestinales. Se presenta el caso de un varón de 35 años con hábitos tóxicos (cocaína, marihuana) que consultó por dolor abdominal, hematoquecia y lesiones purpúricas en extremidades inferiores, que agregó fiebre, artralgias y progresión de lesiones purpúricas asociadas a síndrome nefrítico y ANCA-p (+). Durante la internación se detectó infección por HIV. La biopsia renal evidenció una nefropatía a IgA que tuvo una respuesta favorable al tratamiento corticoideo y antiproteinúrico. La comunicación del caso obedece a la rareza de la presentación y a la complejidad para establecer el diagnóstico y la terapéutica. Aún resta dilucidar el rol de los ANCA en la etiopatogenia y el manejo de la PSH del adulto.
The Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with IgA immune complex deposition. The presentation in adults is rare and severe. Reported cases of HSP in patients infected with HIV are scarce. Neutrophil cytoplasmic antibodies (ANCA) are commonly found in other systemic vasculitis, but rarely in HSP and even more unusual the perinuclear pattern. Beside small vessel vasculitis, positivity of ANCA can be detected in a number of different pathological conditions in association with infectious processes, including HIV, or cocaine use, and especially the pattern of ANCA-p, associated with drugs, inflammatory bowel or autoimmune diseases. We report the case of a 35 years old man with toxic habits (cocaine, marijuana) who consulted for abdominal pain, hematochezia and purpura on lower extremities, and later fever, joint pain and progression of purpura associated with nephritic syndrome and ANCA-p (+). During hospitalization HIV infection was detected. Renal biopsy showed IgA nephropathy with favorable response to corticosteroid and antiproteinuric treatment. The communication of the case is due to the rarity of the presentation and therapeutic diagnostic challenge. It remains to elucidate the role of ANCA in the pathogenesis and management of adult PSH.
Assuntos
Humanos , Masculino , Adulto , Infecções por HIV/complicações , Infecções por HIV/diagnóstico , Abuso de Maconha/complicações , Anticorpos Anticitoplasma de Neutrófilos/análise , Transtornos Relacionados ao Uso de Cocaína/complicações , Glomerulonefrite por IGA/complicações , Metilprednisolona/uso terapêutico , Glucocorticoides/uso terapêutico , Antibacterianos/uso terapêuticoRESUMO
The ANCA-associated vasculitis (AAV) is a group of systemic conditions characterized by inflammation and necrosis in small and medium vessels. AAV with different levels of disease severity could respond to different therapeutics protocols. Early diagnosis and treatment could significantly improve the outcome of the disease. The objective was to investigate the quality evidence in different therapeutical protocols proposed to AAV with renal involvement in pediatrics and adults patients and evaluate the ANCA applicability in AAV diagnosis and outcome. Using methodological search filters, we identified literature in Medline, Embase, Lilacs and Cochrane Trials Register published between 1997 and July 2015. From 4236 articles, 59 were included. The quality of evidence was assessed using the check list designed by the Cochrane Renal Group. The strength of recommendation was determinated by Levels of Evidence (Oxford Centre for Evidence-based Medicine). On the basis of current evidence, 20 recommendations were elaborated for the treatment and monitoring of patients with AAV with renal involvement in several clinical scenarios, in order to provide physicians a rational approach in daily clinical practice.