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Manifestaciones neurológicas de la granulomatosis de Wegener: Caso clínico Neurological involvement in Wegener's granulomatosis: Report of one case
Raimundo López-Rodríguez,Jorge García-González,Joaquín Campos-Franco,Nieves Mallo-González
Revista médica de Chile , 2007,
Abstract: 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
Manifestaciones neurológicas de la granulomatosis de Wegener: Caso clínico
López-Rodríguez,Raimundo; García-González,Jorge; Campos-Franco,Joaquín; Mallo-González,Nieves; Alende-Sixto,María Rosario;
Revista médica de Chile , 2007, DOI: 10.4067/S0034-98872007000700012
Abstract: 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
Estenosis subglótica secundaria a granulomatosis de Wegener: Reporte de un caso y revisión de la literatura Subglottic stenosis secondary to Wegener's granulomatosis: A case report and literature review  [cached]
Christian Olavarría L,Daniel Mu?oz S
Revista de Otorrinolaringología y Cirugía de Cabeza y Cuello , 2009,
Abstract: La granulomatosis de Wegener es una enfermedad multisistémica de origen autoinmune con numerosas manifestaciones otorrinolaringológicas. Dentro de éstas destaca la estenosis subglótica que puede eventualmente comprometer la vida del paciente. Su diagnóstico requiere un alto grado de sospecha, pues se ha visto que su desarrollo es independiente de la actividad de la enfermedad sistémica y debe basarse principalmente en la visión endoscópica de la lesión. El tratamiento de la estenosis subglótica en pacientes con granulomatosis de Wegener es complejo, requiere a menudo de repetidas intervenciones debido a reestenosis. Actualmente, no existe consenso en la técnica quirúrgica más adecuada, por lo que la decisión final deberá basarse en la evaluación de cada caso en particular. Presentamos el caso de un paciente con estenosis subglótica y granulomatosis de Wegener atendido en nuestro servicio y revisamos la literatura con énfasis en las alternativas de tratamiento de esta enfermedad. Wegener's granulomatosis is a autoimmune systemic disease with numerous upper respiratory tract manifestations. Among these, subglottic stenosis represents a potentially lethal complication. Its diagnosis is complicated by the fact that it develops independently from the systemic disease, as it is mainly based on endoscopic examination. Treating subglottic stenosis in patients suffering from Wegener's granulomatosis is complex, it often requires repeated interventions due to restenosis. There is no current consensus on the most adequate surgical technique, and thus it must be evaluated and decided on a case-to-case basis. We present the case of a subglottic stenosis and Wegener's granulomatosis patient from our department. In addition, we reviewed the literature, emphasizing treatment alternatives.
Abscesos como complicación infrecuente de la granulomatosis de Wegener: descripción de tres casos Abscess rare complication of Wegener's granulomatosis: report of three cases  [cached]
álvaro José Pérez,Rosa Elena Salazar,Luis Fernando Giraldo,Víctor Jiménez
Revista Colombiana de Reumatología , 2012,
Abstract: La granulomatosis con poliangeítis es una vasculitis necrotizante de peque o 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 a ade 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.
Manifestación orbitaria de la enfermedad de Wegener Orbital affectation of Wegener’s granulomatosis  [cached]
JC Pinto Bonilla,A Lanuza García,E Hernández Galilea,G Günes
Archivos de la Sociedad Espa?ola de Oftalmología , 2004,
Abstract: Caso clínico: Paciente que consulta por una tumoración orbitaria recidivante. La clínica y los estudios de imagen plantearon un diagnóstico diferencial entre una lesión tumoral de glándula lagrimal y un pseudotumor orbitario localizado. Sin embargo, la evolución clínica obligó a replantear este diagnóstico preliminar. El diagnóstico final se efectuó correlacionando los hallazgos serológicos, histológicos y la clínica. Discusión: La enfermedad de Wegener es una vasculitis necrotizante que afecta clásicamente las vías respiratorias superiores, pulmones y ri ones. Entre el 30% y el 70% de los pacientes presentarán alteraciones oftalmológicas a lo largo de la enfermedad. La afectación oftalmológica previa a las manifestaciones sistémicas es menos frecuente, pudiendo retrasar el diagnóstico definitivo. Case report: Patient who consults for a recidivant orbital mass. Preliminary clinical and image studies suggested an initial differential diagnosis between a lachrymal gland tumor or an orbital located pseudotumor. However, the clinical evolution of the case required a re-evaluation of the preliminary diagnosis. The clinical, histologic and serologic findings were key in the final diagnosis. Discussion: Wegener′s granulomatosis is a necrotizing vasculitis. Classically it affects the upper airways, lungs and kidneys. Between 30% and 70% of patients present ophthalmic symptoms during the course of the illness. Ophthalmic affectation prior to systemic manifestations is less frequent and could delay final diagnosis.
Linfoma de Burkitt en un portador de granulomatosis de Wegener
Kryssia Rodríguez-Castro,Henry Zamora-Barquero
Acta Médica Costarricense , 2005,
Abstract: Se reporta el caso de un paciente costarricense portador de granulomatosis de Wegener, en tratamiento con ciclofosfamida y prednisona, quien desarrolla un linfoma de Burkitt que lo lleva a la muerte. Se menciona la posible relación entre estas dos patologías. This is a report of a costarrican male with Wegener' s granulomatosis undergoing treatment with prednisone and cyclophosphamide, he developed a fatal Burkitt' s lymphoma. The possible relationship between these two disorders is addressed.
Granulomatosis de Wegener: Una enfermedad de varias caras Wegener's granulomatosis: A disease with many faces
Jorge De Jesús Cantillo,Jorge Ernesto Adrés Díaz,Rafael Enrique Andrade
Acta Medica Colombiana , 2007,
Abstract: La granulomatosis de Wegener (GW) es una vasculitis de peque os vasos de etiología desconocida y comúnmente asociada a anticuerpos anticitoplasma del neutrófilo (ANCA). Es un desorden multisistémico autoinmune caracterizado por inflamación granulomatosa necrotizante, necrosis de tejidos y grados variables de vasculitis. El patrón clínico clásico es una triada que involucra las vías aéreas superiores, pulmones y ri ones; sin embargo, la GW es actualmente reconocida como una enfermedad sistémica altamente polimorfa que puede afectar cualquier órgano. El diagnóstico está basado en las manifestaciones clínicas, el resultado de biopsias de lesiones relacionadas y la positividad de títulos de ANCA. La gran mayoría de pacientes con vasculitis asociada a ANCA alcanzan el control temporáneo de la enfermedad con la terapia inmunosupresora actualmente disponible. Informamos cuatro nuevos casos de GW en Colombia. Wegener's granulomatosis is a vasculitis of the small blood vessels. Its causes are unknown. It is commonly associated with anti-neutrophile cytoplasm antibodies (ANCA). It is a multi-systemic autoimmune disorder characterized by necrotizing granulomatous inflammation, necrotizing tissue and various grades of vasculitis. The classic clinical model is a triad involving the upper respiratory tract, lungs, and kidneys. However, Wegener's granulomatosis is currently recognized as a highly polymorphic systemic illness which can affect any organ. Diagnosis is based on clinical manifestations including biopsies of lesions and positive tests for ANCA. The great majority of patients with ANCA associated vasculitis can control the illness with currently available immunosuppressant therapy. We report on four case of Wegener's granulomatosis in Colombia.
Manifestaciones clínicas de la granulomatosis de Wegener y la poliangeítis microscópica en Santiago-Chile, 1990-2001 Clinical features of Wegener granulomatosis and microscopic polyangiitis in Chilean patients  [cached]
Marcela Cisternas M,Lilian Soto S,Sergio Jacobelli G,María Angélica Marinovic M
Revista médica de Chile , 2005,
Abstract: 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)
Estenosis subglótica secundaria a granulomatosis de Wegener: Reporte de un caso y revisión de la literatura
Olavarría L,Christian; Mu?oz S,Daniel;
Revista de otorrinolaringología y cirugía de cabeza y cuello , 2009, DOI: 10.4067/S0718-48162009000200009
Abstract: wegener's granulomatosis is a autoimmune systemic disease with numerous upper respiratory tract manifestations. among these, subglottic stenosis represents a potentially lethal complication. its diagnosis is complicated by the fact that it develops independently from the systemic disease, as it is mainly based on endoscopic examination. treating subglottic stenosis in patients suffering from wegener's granulomatosis is complex, it often requires repeated interventions due to restenosis. there is no current consensus on the most adequate surgical technique, and thus it must be evaluated and decided on a case-to-case basis. we present the case of a subglottic stenosis and wegener's granulomatosis patient from our department. in addition, we reviewed the literature, emphasizing treatment alternatives.
Manifestaciones clínicas de la granulomatosis de Wegener y la poliangeítis microscópica en Santiago-Chile, 1990-2001
Cisternas M,Marcela; Soto S,Lilian; Jacobelli G,Sergio; Marinovic M,María Angélica; Vargas B,Alex; Sobarzo V,Elizabeth; Saavedra M,Jorge; Chauan I,Karina; Meléndez T,Gabriela; Foster B,Carolina; Pacheco R,Daniel; Wainstein G,Eduardo; ,;
Revista médica de Chile , 2005, DOI: 10.4067/S0034-98872005000300001
Abstract: 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)
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