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Granulomatosis with Poliangeitis or Wegner's granulomatosis has an incidence of5-10 cases per million of habitants and only 2-11% of cases present manifestationsin the central nervous system. There are no standardized diagnostic criteria, however,clinical suspicion, positive serology for ANCA’S, histological evidence of necrotizingvasculitis, necrotizing glomerulonephritis or granulomatous inflammation of organssuch as skin, lung or kidney, may suggest this pathology. Neurosurgery is a diagnosticand therapeutic option and could be a possibility in those cases in which the lesionsare in accessible areas and have low risk of generating comorbidities. We presentthe case of a 39-year-old female patient with granulomatosis and polyangiitis withinvolvement in the posterior fossa. After surgical management, it presents meningealinfection. Additionally, we conducted a review of the pathology. Granulomatosis with Poliangeitis or Wegner's granulomatosis has an incidence of 5-10 cases per million of habitants and only 2-11% of cases present manifestations in the central nervous system. There are no standardized diagnostic criteria, however, clinical suspicion, positive serology for ANCA’S, histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis or granulomatous inflammation of organs such as skin, lung or kidney, may suggest this pathology. Neurosurgery is a diagnostic and therapeutic option and could be a possibility in those cases in which the lesions are in accessible areas and have low risk of generating comorbidities. We present the case of a 39-year-old female patient with granulomatosis and polyangiitis with involvement in the posterior fossa. After surgical management, it presents meningeal infection. Additionally, we conducted a review of the pathology

Introducción: La granulomatosis con poliangeítis (GPA) es una enfermedad autoinmune con múltiples manifestaciones otorrinolaringológicas. La estenosis subglótica (ESG) se reporta entre 8%-50% de los pacientes. Objetivo: Describir la presentación clínica y tratamiento de la ESG en pacientes con GPA. Material y método: Estudio descriptivo retrospectivo de los pacientes atendidos en el Departamento de Otorrinolaringología de la Pontificia Universidad Católica de Chile entre 2011 y 2015 con diagnóstico de GPA y ESG. Resultados: Diez pacientes (90% mujeres) con edad promedio al diagnóstico de 44,6 años. Los síntomas fueron: disnea (9/10), disfonía (6/10) y estridor (5/10). En el 80%, la ESG fue la presentación inicial de la GPA. El 90% presentó GPA localizada y 10% GPA sistémica. El 60% presentó anticuerpos anticitoplasma de neutrófilos (ANCA) negativo. Todos los pacientes recibieron glucocorticoides sistémicos, asociados a inmunosupresores y/o anticuerpos monoclonales. Una paciente requirió traqueostomía y dos pacientes han requerido dilatación endoscópica con balón asociado a inyección local de metilprednisolona. Conclusiones: La ESG puede ser la única manifestación de GPA. Los síntomas inespecíficos y la alta probabilidad de presentar ANCA negativo hacen difícil el diagnóstico. El manejo debe ser individualizado y multidisciplinario. La mayoría de nuestros pacientes pudieron ser manejados adecuadamente con tratamiento médico. Introduction: Granulomatosis with polyangiitis (GPA) is an autoimmune disease that presents with multiple ENT manifestations. Subglottic stenosis (SGS) has been reported in 8-50% of the patients. Aim: To describe the clinical presentation and treatment of SGS in patients with GPA. Material and method: Retrospective review of clinical data from all patients consulting in the Otolaryngology department at Pontificia Universidad Católica de Chile from 2011 to 2015 with SGS and GPA diagnosis. Results: Ten patients (90% female) with average age at diagnosis of44.6 years. The symptoms were: dyspnea (9/10), dysphonia (6/10) and stridor (5/10). SGS was the initial presenting manifestation of GPA in 80% of patients. GPA was localized in 90% and systemic in 10% of patients, and anti-neutrophilic cytoplasmic antibodies (ANCA) were negative in 60% of patients. Systemic glucocorticoids were used in every patient, associated with immunosuppressants and/or monoclonal antibodies. One patient required tracheostomy and 2 patients were treated with endoscopic balloon dilation associated with local methylprednisolone injection. Conclusions: SGS can be GPA’s initial or unique manifestation. Diagnosis is hindered due to the unspecificity ofsymptoms and the high probability of presenting with negative ANCA. Individualized and multidisciplinary treatment is required. Most patients were treated pharmacologically with an adequate response in our series.

Las vasculitis sistémicas idiopáticas se caracterizan por inflamación y necrosis de las paredes de los vasos de origen desconocido¹; se han propuesto medicamentos e infecciones como posibles disparadores de estas enfermedades². A continuación se presenta el caso de un paciente con diagnóstico de granulomatosis con poliangeítis, con anticuerpos contra el citoplasma de neutrófilos tanto antiproteinasa 3 como antimieloperoxidasa y con el hallazgo poco usual de granuloma renal. Idiopathic systemic vasculitis is characterized by inflammation and necrosis of the vessel walls of unknown origin. Medications and infections have been proposed as potential triggers of these diseases.The case is presented on a patient diagnosed with granulomatosis with polyangiitis with antibodies to neutrophil cytoplasm, as well as anti-proteinase 3 and anti-myeloperoxidase, plus the unusual finding of renal granuloma.

Granulomatosis with polyangiitis (GPA) or Wegener's disease is characterized by a granulomatous vasculitis of the upper and lower airways and kidney. It involves the lower respiratory tract causing subglottic tracheal stenosis, which occurs in approximately 22% of patients. We report two females aged 40 and 52 years, admitted to the hospital with a subglottic tracheal stenosis. Their symptoms and management are reviewed. The frst patient responded to rituximab. The second patient required a tracheostomy.

Granulomatosis with polyangiitis (GPA) or Wegener's disease is characterized by a granulomatous vasculitis of the upper and lower airways and kidney. It involves the lower respiratory tract causing subglottic tracheal stenosis, which occurs in approximately 22% of patients. We report two females aged 40 and 52 years, admitted to the hospital with a subglottic tracheal stenosis. Their symptoms and management are reviewed. The frst patient responded to rituximab. The second patient required a tracheostomy.

ANCA (anti-neutrophil cytoplasmic antibodies) associated vasculitis or AAVare a group of diseases with predominant inflammation of small vessels and the presence of detectable ANCA in serum. Due to these common features, it is considered that AAV share pathogenic mechanisms. Consequently, a similar therapeutic approach has been developed. A new nomenclature has been recently proposed, with AAV including "granulomatosis with polyangiitis" (GPA, formerly Wegener's granulomatosis), "microscopic polyangiitis" (MPA), "eosinophilic granulomatosis with polyangiitis" (EGPA, formerly Churg-Strauss disease) and renal limited vasculitis. Research on AAV has shown significant advances in the last two decades, including advances in the knowledge of pathogenic mechanisms such as the pro-inflammatory role of ANCA, AAV murine models and genetic links. Evidence on previous immunosuppressive therapies has improved significantly and new, promissory drugs have been introduced, Rituximab being the most important. We review the advances of pathogenic mechanisms and treatment for these diseases.

ANCA (anti-neutrophil cytoplasmic antibodies) associated vasculitis or AAV are a group of diseases with predominant inflammation of small vessels and the presence of detectable ANCA in serum. Due to these common features, it is considered that AAV share pathogenic mechanisms. Consequently, a similar therapeutic approach has been developed. A new nomenclature has been recently proposed, with AAV including "granulomatosis with polyangiitis" (GPA, formerly Wegener's granulomatosis), "microscopic polyangiitis" (MPA), "eosinophilic granulomatosis with polyangiitis" (EGPA, formerly Churg-Strauss disease) and renal limited vasculitis. Research on AAV has shown significant advances in the last two decades, including advances in the knowledge of pathogenic mechanisms such as the pro-inflammatory role of ANCA, AAV murine models and genetic links. Evidence on previous immunosuppressive therapies has improved significantly and new, promissory drugs have been introduced, Rituximab being the most important. We review the advances of pathogenic mechanisms and treatment for these diseases.

La granulomatosis con poliangitis como causa del síndrome pulmón-riñón es infrecuente en niños. Presentamos el caso de un paciente de 13 años con hemorragia alveolar, glomerulonefritis rápidamente progresiva, escleritis y anticuerpos anti-PR3 (proteinasa 3). Hacemos referencia a las características de esta vasculitis asociada a anticuerpos contra el citoplasma de los neutrófilos (ANCA) en los niños y a su enfoque terapéutico. Granulomatosis with polyangiitis as a cause of kidney-lung syndrome is uncommon in children. We report the case of a 13 year old patient with alveolar hemorrhage, rapidly progressive glomerulonephritis, episcleritis and anti PR3. We refer to features of the ANCA-associated vasculitis in children and to its therapeutic approach.

La granulomatosis con poliangeítis es una vasculitis necrotizante de pequeño vaso que puede afectar a múltiples órganos, se describen formas clínicas localizadas y generalizadas, esta última caracterizada por compromiso renal y pulmonar grave, asociada a la presencia de PR3- ANCA circulante en más del 90% de los casos. Se presentan tres casos de pacientes con diagnóstico patológico de granulomatosis con poliangeítis, dos de ellos con lesiones pulmonares detectadas en radiografías convencionales y tomografía computarizada (TC), imágenes compatibles con abscesos pulmonares; hallazgos poco comunes en las manifestaciones imaginológicas de la granulomatosis con poliangeítis en el sistema respiratorio, se añade además un tercer caso complicado con absceso paraesofágico. Se destaca la importancia y utilidad de la radiología convencional y la tomografía computarizada (TC) para el diagnóstico de esta enfermedad y de sus complicaciones. Wegener's granulomatosis is a small vessel necrotizing vasculitis that can affect multiple organs, clinical forms are described localized and generalized the latter characterized by severe pulmonary and renal impairment associated with the presence of circulating PR3-ANCA in 90% of the cases. We describe three cases of patients with pathologic diagnosis of Wegener's granulomatosis, two of them had lung lesions detected on conventional radiography and computed tomography (CT) images consistent with lung abscesses, uncommon findings in the imaging manifestations of Wegener's granulomatosis the respiratory system also adds a third case peri esophageal abscess complicated. The importance and utility of conventional radiography and computed tomography (CT) for diagnosis of this disease and its complications.

Las vasculitis primarias constituyen un grupo de enfermedades reumáticas con expresión clínica variable y pronóstico reservado cuando no se tratan adecuadamente. En esta revisión haremos un análisis pormenorizado del tratamiento en las diferentes formas de vasculitis primaria, iniciando con el uso de los corticoides, desde casi su descubrimiento en 1949, pasando por otros inmunosupresores como: ciclofosfamida, metotrexate, azatioprina, mofetil, micofenolato, al igual que medicamentos biológicos como rituximab y anti-TNF. Una mención especial se hace sobre las guías de tratamientos para las vasculitis, tanto de grandes como de pequeños vasos, implementadas desde 2009 por el Grupo Europeo de Estudio de las Vasculitis. Primary vasculitis is one of the rheumatic diseases with variable clinical expression and poor prognosis when not treated properly. In this review we analyze detailed treatment of this disease in different forms of primary vasculitis, starting with the use of corticosteroids from almost its discovery in 1949, to other immunosuppressant's such as cyclophosphamide, methotrexate, azathioprine, mycophenolate mofetil, as well as biological drugs such as rituximab and anti-TNF. Special mention is made on the guidelines of treatment for vasculitis of both large and small vessel, implemented since 2009 by the European Group Study of Vasculitis.

Introducción: la granulomatosis de Wegener (GW) es una vasculitis granulomatosa sistémica que afecta primariamente tracto respiratorio y riñones. Objetivos: analizar las características clínicas de pacientes con GW asistidos en la Clínica Médica "C" del Hospital de Clínicas. Material y método: realizamos un estudio descriptivo retrospectivo, incluyendo pacientes con diagnóstico clínico-anatomopatológico definitivo de GW. Se analizaron cinco casos diagnosticados entre 2002 y 2006. Resultados: la media de edad al diagnóstico fue de 51 años (SD ± 24,5). La afectación respiratoria alta y baja fue el hallazgo clínico más frecuente. En los cinco casos el diagnóstico inicial fue de infección respiratoria. Todos presentaron anticuerpos anticitoplasma de neutrófilos (ANCA). La confirmación diagnóstica se realizó mediante biopsia del tracto respiratorio. En dos pacientes el diagnóstico de GW fue precedido por la presencia de una neoplasia maligna. La media de seguimiento fue de 26 meses (SD ± 19,2), falleciendo tres pacientes luego de una media de 20 meses de hecho el diagnóstico. El tratamiento de inducción se realizó en base a metilprednisolona y ciclofosfamida, y el de mantenimiento utilizando prednisona y azatioprina. Conclusión: la GW puede ser interpretada como una infección respiratoria en su etapa inicial, donde los hallazgos de la tomografía de tórax y la positividad de los ANCA constituyen puntos de apoyo esenciales en el diagnóstico, permitiendo dirigir las biopsias hacia "sitios sintomáticos" e iniciar el tratamiento inmunosupresor de manera precoz Summary Introduction: Wegener’s granulomatosis (WG) is a type of systemic granulomatous vasculitis primarily affecting the respiratory tract and kidneys. Objectives: to study the signs and symptoms presented by patients cared for at Clinica Médica "C" of the Clinicas Hospital. Methods: we conducted a descriptive, restrospective study including patients with a clinical and anatomopathological definitive diagnosis of WG. Five cases, diagnosed between 2002 and 2006, were analysed. Results: average age of diagnosis was 51 years old (SD ± 24,5).Affection of the upper and lower respiratory tract was the most frequent clinical finding. Initial diagnosis in the 5 cases was respiratory tract infection. They all evidenced anticytoplasm of the neutrophil antibodies. (ACNA). Diagnosis was confirmed by a biopsy of the respiratory tract. Two patients developed malignant neoplasia prior to the diagnosis of WG. Average follow-up was 20 months after the diagnosis. Induction treatment of methylprednisolone and cyclophosphamide, and follow-up treatment with prednisone and azathioprine, were applied. Conclusions: WG may be interpreted as a respiratory tract infection in its initial stage, when thorax tomography and positive ACNA findings constitute essential factors for diagnosis, and thus enable the orientation of biopsies toward "symptomatic sites" and the early initiation of immunosuppressor treatment. Résumé Introduction: la granulomatose de Wegener (GW) est une vasculite qui atteint primairement les voies respiratoires et les reins. Objectifs: analyser les caractéristiques cliniques de ces patients avec GW assistés dans la Clinique Médicale "C" de l’Hôpital de Clinicas. Matériel et méthode: on fait une étude descriptive rétrospective qui inclut des patients à diagnostic définitif de GW. Cinq cas sont analysés entre 2002 et 2006. Résultats: l ‘âge moyen au diagnostic fut de 51 ans (SD±24,5). L’atteinte respiratoire haute et basse fut clini-quement la plus fréquente. Dans les 5 cas le diagnostic fut d’infection respiratoire.. Tous présentaient des anticorps anti cytoplasme de neutrophiles (ANCA). La confirmation diagnostique a été faite au moyen de biopsie du tract res-piratoire. Chez deux patients le diagnostic de WG fut précédé de la présence d’une néoplasie maligne. Le suivi fut de 26 mois en moyenne (SD±19,2), trois patients ont décédé après 20 mois en moyenne de leur diagnostic. Le traitement d’induction s’est fait avec metilprednisone et cyclophosphamide, puis avec prednisone et azathioprine. Conclusion: la GW peut être interprétée comme une infection respiratoire au début ; là, la tomographie de thorax et la possibilité des ANCA constituent des points de repère essentiels pour le diagnostique, permettant de diriger les biopsies vers des "champs symptômatiques" et de com-mencer le traitement immunodépresseur de façon précoce. Resumo Introdução: a granulomatose de Wegener (GW) é uma vasculite granulomatosa sistêmica que afeta primariamente o trato respiratório e os rins. Objetivos: analisar as características clínicas de pacientes com GW atendidos na Clínica Médica "C" do Hospital das Clínicas. Material e método: realizamos um estudo descritivo retrospectivo, incluindo pacientes com diagnóstico clínico-anatomopatológico definitivo de GW. Foram analisados cinco casos diagnosticados entre 2002 y 2006. Resultados: a idade média no momento do diagnóstico foi 51 anos (SD ± 24,5). O comprometimento respiratório alto e baixo foi o achado clínico mais freqüente. O diagnóstico inicial nos cinco casos foi infecção respiratória. Todos apresentaram anticorpos anticitoplasma de neutrófilos (ANCA). A confirmação diagnóstica foi realizada por biópsia do trato respiratório. Em dois pacientes o diagnóstico de GW foi precedido pela presença de uma neoplasia maligna. A média de seguimento foi de 26 meses (SD ± 19,2); três pacientes faleceram em um período médio de 20 meses depois do diagnóstico. O tratamento de indução foi realizado com etilprednisolona e ciclofosfamida, e o de manutenção com prednisona e azatioprina. Conclusão: a GW, em sua etapa inicial, pode ser interpretada como uma infecção respiratória, sendo que os resultados da tomografia de tórax e a positividade dos ANCA são essenciais para o diagnóstico, permitindo orientar as biopsias a "regiões sintomáticas" e iniciar o tratamento imunossupressor precocemente.

En este artículo se hace una revisión extensa sobre las vasculitis primarias y se recopila la información latinoamericana que aparece referen-ciada en las más importantes bases de datos mundiales en inglés, español y portugués, desde el año 1945 hasta septiembre del año 2007. Igualmente se establecieron varios contactos con reumatólogos o médicos interesados en este tópico. Se realizaron búsquedas secundarias de los estudios que aparecieran citados en los artículos seleccionados y se revisaron manualmente abstracts de congresos. In this paper an extensive review about primary vasculitis and Latino American information that appears referenced in the most important world wide data bases in English, Spanish and Portuguese from 1945 to september 2007, is compiled. Several contacts with rheumatologist or physician interested in this topic were made. Also secondary searches of the studies that appeared mentioned in selected articles were made and the abstracts of congresses were reviewed manually.

Wegener's granulomatosis is a systemic necrotizing vasculitis that affects medium size and small vessels. Neurological involvement occurs in 22% to 54% of patients, mainly in the form of mononeuritis multiplex. Central nervous system involvement is reported in only 2% to 8% of the cases. We report a 42-year-old male who presented with headache, diplopia, third and sixth cranial nerve palsies and ¡eft eye amaurosis associated to mass located in the ¡eft Meckel cavum and diffuse meningeal involvement. A biopsy of the mass disclosed a chronic granulomatous necrotizing inflammation with Langhans giant cells. A chest CAT scan showed three cavitated lung nodules and ANCA antibodies were positive in a titer of 1:80. Treatment with steroid and cyclophosphamide was started and cranial nerve palsies resolved and the number and size of lung nodules decreased. The patient was lost from follow up

Background: Systemic vasculitis are a group of heterogeneous diseases characterized by inflammation and necrosis of blood vessel walls. The etiology is not known, but geographic and environmental factors are implicated. Aim: To describe the clinical features of microscopic polyangiitis (MPA) and Wegener's granulomatosis (WG) in a Chilean cohort of patients. Patients and methods: Retrospective review of the medical records of 123 patients with the diagnosis of systemic vasculitis (65 MPA and 58 WG), seen from 1990 to 2001. The diagnosis were made based on the American College of Rheumatology and Chapel Hill criteria. Results: The mean follow-up for MPA was 15 months (1-120) and for WG, 20 months (1-120). The median age (years) at diagnosis for MPA was 61 (19-82) and WG 50 (20-82). Gender distribution was similar in both groups (male: 68% and 57% respectively).The main clinical features in the MPA group were renal involvement (68%), peripheral nervous system involvement (57%), pulmonary hemorrhage (28%), and skin disease (32%). In the WG group were alveolar hemorrhage (62%), renal involvement (78%), paranasal sinus involvement (57%), and ocular disease (26%). In both, creatinine levels above 2.0 mg/dl were associated with a higher mortality (p< 0.01). ANCA by immunofluorescence was performed in 56 MPA patients (75% had pANCA, 4% had cANCA and 21% were ANCA negative) and in 55 WG patients (17% had pANCA, 79% had cANCA and 4% were ANCA negative). Global mortality was 18% and 17% respectively, and the most common causes of death were infections. Conclusions: The clinical features of our patients are similar to other published data. In our WG and MPA patients the main predictor for death was a serum creatinine above 2 mg/dl. (Rev Méd Chile 2005; 133: 273-8)