Clinical profile and management of granulomatosis with polyangiitis–associated scleritis from a tertiary care hospital in South India

Purpose: To describe the clinical features and management of patients with scleritis associated with granulomatosis with polyangiitis (GPA) at a tertiary eye care center in South India. Methods: The clinical profile and management of patients presenting to a tertiary eye care center in South India with scleritis secondary to GPA from 2003 to 2021 were analyzed retrospectively. Scleritis was classified into anterior diffuse, nodular, and necrotizing scleritis with inflammation according to Watson and Hayreh’s classification. Demographic characteristics, clinical features, anti?neutrophil cytoplasmic antibody (ANCA) positivity, treatment response, ocular complications, and status at the last follow?up were analyzed. Statistical analysis of data was performed using Microsoft Excel 2019. Results: Nineteen eyes of 17 patients (15 cytoplasmic staining ANCA [c?ANCA], two p?ANCA positive) were included. Fifteen eyes had necrotizing scleritis, two had diffuse anterior scleritis, and two had nodular scleritis. Remission was induced using a combination of steroids and cyclophosphamide or rituximab. Maintenance therapy was instituted using tapering steroids and immunosuppressants like cyclophosphamide, mycophenolate mofetil, methotrexate, or rituximab. Three eyes required a scleral patch graft. Fourteen patients had good anatomical and visual outcomes, and three were lost to follow?up. Conclusion: GPA is a rare disease, while it is the most common ANCA?associated vasculitis with scleritis. As scleritis may be the presenting sign of the disease, ophthalmologists must be aware of the various features suggestive of GPA. GPA?associated scleritis can have a good prognosis when diagnosed promptly and managed aggressively in the acute stage, and remission is maintained with adequate systemic immunosuppression.

Granulomatosis with Polyangiitis Presenting as a Renal Mass: A Scarce Case Report with a Review of the Literature

Wegener granulomatosis (WG) now known as granulomatosis with polyangiitis (GPA) is an uncommon autoimmune disorder of undivulged etiology affecting the respiratory tract including paranasal sinuses, nasal cavity, lungs, and kidneys predominantly. GPA presenting as a solitary renal mass is rarely seen. We present a case report of a 27-year-old female presenting with a right renal mass along with pain, low-grade fever, and arthralgia. Computed tomography scan of the abdomen revealed a hypodense low attenuated renal mass with indistinct margins. Ultrasound-guided biopsy revealed features typical of GPA. She was started on oral steroids (prednisolone 40?mg) and azathioprine. She developed pain, vomiting, and diarrhea after starting treatment with azathioprine. Azathioprine was stopped and rituximab 1?g weekly was started for 4 weeks followed by 500?mg 6 monthly injections. She got symptomatic relief at 4 weeks with a diminution of renal mass at 6 months follow-up. We report this rare entity of WG presenting as renal mass. Suspecting and diagnosing renal mass as a part of GPA prevented us from undertaking unnecessary surgical treatment in this patient. Medical treatment with steroids and rituximab is effective in inducing remission and maintenance.

Small vessel vasculitis, different renal outcomes in pediatric patients. Case reports

Vasculitis mainly affects the walls of the blood vessels, and is an uncommon disease in the pediatric population. In general, they are classified according to the EULAR / PreS consensus in children and in adults according to the Chapel-Hill consensus conference. ANCA-associated vasculitis (AAV) is part of small-vessel disease and is represented by granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA), and others. The representative renal histopathological findings are focal necrotizing glomerulonephritis with crescents, variable interstitial inflammation, absence of immune complexes, or small deposits of immunoglobulins. Clinically, AAV can manifest with hematuria, proteinuria, high blood pressure, and/or rapidly progressive glomerulonephritis. GPA can severely affect the kidney in 75% of cases. In MPA, renal involvement (75-90%) can be rapid and severe with the possibility of requiring renal replacement therapy in more than half of the patients. Furthermore, up to 25% of patients may have high blood pressure, and the mortality at one year can be up to 85%. In EGPA the renal involvement is usually mild. Three pediatric cases of AAV with different renal outcomes are presented, including the need for renal replacement therapy with the recovery of renal function, kidney transplantation, and death, followed in a fourth level of care institution in Colombia.

Las vasculitis, patologías cuyo hallazgo principal es la afectación de las paredes de los vasos sanguíneos, se presentan de forma infrecuente en la población pediátrica. En general, en niños se clasifican de acuerdo con el consenso de la EULAR/PReS, y en adultos, según la Conferencia de Consenso de Chapel-Hill. Las vasculitis asociadas con ANCA (VAA) hacen parte de las vasculitis de pequeños vasos y están representadas por la granulomatosis con poliangeítis (GPA), la granulomatosis eosinofílica con poliangeítis (EGPA) y la poliangeítis microscópica (PAM), entre otras. A nivel renal, los hallazgos histopatológicos representativos son la glomerulonefritis focal necrotizante con media luna, inflamación intersticial variable, ausencia de complejos inmunes o pequeños depósitos de inmunoglobulinas. Clínicamente, las VAA pueden manifestarse con hematuria, proteinuria, hipertensión arterial o glomerulonefritis rápidamente progresiva. La GPA puede afectar de forma severa el riñón en el 75% de los casos, mientras que, en la PAM, el compromiso renal (75-90%) puede ser rápido y severo con posibilidad de requerir terapia de reemplazo renal en más de la mitad de los pacientes. Además, hasta el 25% de los casos puede tener hipertensión arterial, con una mortalidad a un ario de 85%. En la EGPA, el compromiso renal suele ser leve. Se presentan 3 casos pediátricos de VAA con diferentes desenlaces renales, que incluyen necesidad de terapia de reemplazo renal con recuperación de función renal, trasplante renal y muerte, seguidas en una institución de IV nivel del suroccidente colombiano.

A Case Report and Literature Review of Pulmonary Wegener' Granulomatosis / 中国肺癌杂志

Wegener' granulomatosis is an autoimmune diseases, often involving the lung and kidney, has a high mortality rate in nontreatment patients. The low incidence and nonspecific features, often lead to misdiagnosis and delayed treatment. This paper reported the diagnosis and treatment of a 55-year-old female patient with primary Wegener' granuloma of the lung diagnosed by percutaneous lung biopsy of pulmonary nodules, and reviews the relevant literature.
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Granulomatosis Con Poliangeítis Y Compromiso En Fosa Posterior. Reporte De Un Caso / Granulomatosis With Polyangeitis In Posterior Fosa. Report of a Case

Resumen La Granulomatosis con Poliangeitis, también conocida como granulomatosis de Wegner presenta una incidencia de 5-10 casos por millón de habitantes y solo el 2-11% de los casos presentan manifestaciones en el sistema nervioso central. No existen unos criterios diagnósticos estandarizados, sin embargo, la sospecha clínica, la serología positiva para ANCA, la evidencia histológica de vasculitis necrotizante, la glomerulonefritis necrotizante o la inflamación granulomatosa de órganos como piel, pulmón o riñón, pueden hacer pensar en dicha patología. La neurocirugía es una opción tanto diagnostica como terapéutica y debería realizarse en aquellos casos en que las lesiones se encuentren en zonas accesibles y tengan bajo riesgo de generar comorbilidades. Presentamos el caso de una paciente femenina de 39 años con cuadro de Granulomatosis con Poliangeítis con compromiso en fosa posterior a quién se le realiza un abordaje occipitocervical derecho. Posterior al manejo quirúrgico presenta infección meningea. Adicionalmente, realizamos una revisión de la literatura sobre dicha patología.

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.

Granulomatosis de Wegener mimetizando una lesión tumoral de vértice de pulmón. Reporte de un caso clínico y revisión de la literatura / Wegener's granulomatosis mimicking a tumor of the lung vertex. Report of a clinical case and review of the literature

La granulomatosis con poliangeítis antes denominada con el epónimo de granulomatosis de Wegener, clasifica entre las vasculitis sistémicas, de causa desconocida, que afecta a los vasos de pequeño y mediano calibre. Se caracteriza por la afectación del aparato respiratorio en su tracto superior, pulmones, así como a los riñones, aunque puede afectar a otros órganos y sistemas del organismo humano. Los anticuerpos anti citoplasma de neutrófilos son positivos con una frecuencia que alcanza el 80 por ciento de casos, y la característica histológica más relevante es la presencia de lesiones granulomatosas necrosantes. El diagnóstico se basa en las manifestaciones clínicas, la biopsia con estudio histológico de los tejidos y órganos afectados así como la presencia de anticuerpos anti citoplasma de neutrófilos. Hoy en día se agrupa dentro de las denominadas vasculitis asociadas a anticuerpos anti citoplasma de neutrófilos positivas. Nuestro estudio tuvo como objetivo presentar un caso clínico inusual, en el cual reportamos a una paciente de 55 años de edad la cual fue atendida por presentar manifestaciones clínicas, de laboratorio y radiológicas que permiten confirmar el diagnóstico de una Granulomatosis de Wegener. Su forma clínica de expresión inicial mostró. una serie de signos y síntomas constitucionales como fiebre superior de 38 grados de varios días de evolución en horario vespertino, astenia, pérdida de apetito, artromialgias, manifestaciones respiratorias como dolor torácico e intercostal izquierdo, tos con expectoración mucosa, y disnea a esfuerzos físicos en aumento. Al examen físico se recogieron datos positivos como palidez cutánea mucosa, la presencia de disminución del murmullo vesicular a nivel del vértice del pulmón izquierdo con estertores crepitantes y una imagen a modo de masa tumoral redondeada del lóbulo superior del pulmón que orientaba al diagnóstico de una neoplasia de vértice de pulmón. Se realizaron múltiples estudios considerando el proteiforme cuadro clínico, los estudios inmunológicos realizados, y la histología por biopsia pulmonar, los que demostraron la existencia de lesiones de tipo granulomatosas compatibles con G. Wegener. Resultó efectiva la respuesta terapéutica con drogas esteroideas e inmunosupresoras en forma de bolos de ciclofosfamida acompañados de un fuerte tratamiento antibiótico de amplio espectro. Concluimos que se trata de un raro caso de granulomatosis de Wegener cuya forma de debut hizo plantear el diagnóstico inicial de una lesión tumoral de vértice de pulmón. No conocemos de otro caso similar reportado en nuestro medio(AU)

Granulomatosis with polyangiitis, formerly known as Wegener's Granulomatosis, is classified as systemic vasculitis, of unknown cause, affecting small and medium-sized vessels. It is characterized by the involvement of the respiratory system in its upper tract, lungs, as well as the kidneys, although it can affect other organs and systems. Neutrophil anticithoplasma antibodies are positive with a frequency that reaches over 80 percent of cases, and the most relevant histological feature is the presence of necrotizing granulomatous lesions. The diagnosis is based on clinical manifestations, biopsy with histological study of the affected tissues and organs as well as the presence of Neutrophil anticithoplasma antibodies. Nowadays it is grouped into the so-called Neutrophil anticithoplasma antibodies positive vasculitis. Arthromyalgia, respiratory manifestations such as thoracic and left intercostal pain, cough with mucous expectoration, and dyspnea on physical efforts in increase. The physical examination collected positive data such as mucous skin pallor, the presence of decreased vesicular murmur at the level of the left vertex of the lung with crackling rales and an image as a rounded tumor mass of the upper lobe of the lung that guided the diagnosis of a vertex neoplasm of lung. Multiple studies were carried out considering the proteiform clinical picture, the immunological studies performed, and the lung biopsy histology, which demonstrated the existence of granulomatous lesions compatible with G. Wegener. The therapeutic response was effective with steroidal and immunosuppressive drugs in the form of cyclophosphamide boluses accompanied by a strong broad-spectrum antibiotic treatment. We conclude that this is a rare case of Wegener's granulomatosis, whose debut form raised the initial diagnosis of a tumor of the lung vertex. We do not know of another similar case reported in our country(AU)

Granulomatosis de Wegener, una afección poco frecuente / Wegener's granulomatosis, a rare condition

Introducción: la granulomatosis de Wegener es una vasculitis sistémica, poco frecuente, cuyo sustrato etiopatogénico es la inflamación de los vasos sanguíneos impidiendo la libre circulación del flujo sanguíneo. Clinicamente se caracteriza por presencia de manifestaciones respiratorias, nasales, auditivas y renales; frecuentemente se asocian trastornos infecciosos y alérgicos. Objetivo: socializar los principales elementos etiopatogénicos, manifestaciones clínicas y exámenes complementarios que permiten definir el granulomatosis de Wegener. Caso clínico: se presenta el caso de una paciente de 32 años de edad la cual es referida desde la atención primaria de salud, por presentar manifestaciones clínicas y de laboratorio que permiten confirmar el diagnóstico de una granulomatosis de Wegener. Conclusiones: la granulomatosis de Wegener es una rara enfermedad que, aunque se presenta fundamentalmente en pacientes femeninas entre 40 y 50 años, puede presentarse en pacientes de cualquier edad sin distinguir preferencia por el sexo. Sus principales manifestaciones clínicas son la toma respiratoria y renal acompañada de manifestaciones generales. El uso de esteroides constituye la piedra angular del tratamiento, al cual se adicionan medicamentos para el control de la enfermedad como es el caso del methotrexate.

Introduction: Wegener's granulomatosis is a rare systemic vasculitis, whose etiopathogenic substrate is the inflammation of the blood vessels, preventing the free circulation of blood flow. Clinically it is characterized by the presence of respiratory, nasal, auditory and renal manifestations; Infectious and allergic disorders are frequently associated. Objective: to socialize the main etiopathogenic elements, clinical manifestations and complementary examinations that allow us to define Wegener's granulomatosis. Clinical case: the case of a 32-year-old female patient is reported from primary health care, due to clinical and laboratory manifestations that confirm the diagnosis of Wegener's granulomatosis. Conclusions: Wegener's granulomatosis is a rare disease that, although it occurs mainly in female patients between 40 and 50 years, can occur in patients of any age without distinguishing preference for sex. Its main clinical manifestations are respiratory and renal uptake accompanied by general manifestations. The use of steroids is the cornerstone of the treatment, to which are added medications for the control of the disease such as methotrexate.

Actualización en el estudio de granulomatosis con poliangeitis (granulomatosis de Wegener) / Update in study of granulomatosis with polyangiitis (Wegener's granulomatosis)

Resumen:La granulomatosis con poliangeítis (GPA) es una vasculitis sistémica de pequeño vaso, que afecta más frecuentemente el tracto respiratorio y el riñón. Sus criterios diagnósticos se basan en la clínica, exámenes de laboratorio, imágenes e histología. El 90% son ANCA (anticuerpos anticitoplasma de neutrófilos) positivos. La histología muestra inflamación granulomatosa, necrosis y vasculitis. Los exámenes de imagen son de vital importancia en su estudio inicial y seguimiento, correspondiendo principalmente a técnicas tomográficas. La tomografía Computada (TC) es el método de elección para la evaluación de vía aérea superior y pulmón, con alta sensibilidad en afectación de cavidades nasal/paranasales, árbol bronquial y pulmón. La Resonancia Magnética está indicada en compromiso del sistema nervioso central y corazón. El PET/CT presenta alta sensibilidad en enfermedad tóraco-abdominal, es de utilidad en detectar lesiones no visibles con otras técnicas, y en control de tratamiento. El compromiso renal, de alta ocurrencia en GPA, presenta escasa traducción en las imágenes y es frecuentemente indetectable con imágenes, aunque el PET/CT puede ser positivo en casos de glomerulonefritis acentuada. La radiología simple no debe ser utilizada en el estudio de GPA dado su bajo rendimiento diagnóstico. El tratamiento se basa en terapia corticoidea e inmunosupresora. Las recaídas son frecuentes, por lo que estos pacientes requieren seguimiento a largo plazo.

Abstract:Granulomatosis with polyangiitis (GPA) is a systemic type of vasculitis that affects small vessels, most commonly involving the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, imaging and histology. Ninety percent are ANCA (anti-neutrophilic cytoplasmic antibodies) positive. Histology demonstrates granulomatous inflammation, necrosis and vasculitis. Imaging studies are vital for the initial work-up and follow-up. Computed Tomography (CT) is the method of choice for evaluation of the upper airway and lungs, because of its high sensitivity detecting anomalies of paranasal sinuses, bronchial tree and lungs. Magnetic Resonance is indicated for evaluation of the central nervous system and heart. PET/CT has high sensitivity for thoracic and abdominal disease, is useful at detecting lesions not seen with other imaging techniques, and for follow-up. Renal involvement, very frequent on GPA, is usually undetectable at imaging, but may be seen at PET/CT in cases of marked glomerulonephritis. Plain X-rays should not be used for evaluation of GPA because of their low diagnostic performance. Treatment is based on corticosteroid and immunosuppressive therapy. Relapses are frequent, so these patients require long-term follow-up.

Granulomatosis de wegener: A propósito de un caso infantil / Wegener's granulomatosis: A pediatric case report

La granulomatosis de Wegener es una vasculitis granulomatosa necrotizante, que afecta las vías aéreas y los glomérulos. Ésta y la poliangitis microscópica son enfermedades vasculares, asociadas con anticuerpos anticitoplasma del neutró?lo (ANCA). La granulomatosis de Wegener es una enfermedad rara, de causa aún no de?nida, con incidencia de 0.4 casos por cada 100.000 habitantes. Se reporta el caso de un paciente masculino de 14 años de edad, sin antecedentes médicos de importancia pero con reporte de C3 y C4 normales, ANA positivo y c-ANCA positivos 1:40. La revisión bibliográ?ca pone al día los conocimientos acerca de esta enfermedad, que permiten al clínico tenerla en mente para diagnosticarla con oportunidad.

Wegener's granulomatosis is a necrotizing granulomatous vasculitis, that affects the upper respiratory tract, lower and glomeruli. This and microscopic polyangiitis are vascular diseases associated with neutrophil cytoplasmic antibodies (ANCA). Wegener's granulomatosis is a rare disease, cause not yet de?ned, with an incidence of 0.4 cases per 100.000 inhabitants. We report the case of a male patient of 14 years old, no medical history of importance but with C3 and C4 report normal, positive ANA and positive c-ANCA 1:40. The literature review updates the knowledge about this disease that allows the clinician to diagnose keeps it in mind to try.

Renal compromise in a patient with Wegener's granulomatosis: clinical case and literature review / Compromiso renal en un paciente con granulomatosis de Wegener: caso clínico y revisión de la literatura

Absctrat Wegener's granulomatosis (WG) is characterized by small vessel vasculitis that affects the upper respiratory tract, lungs and kidneys. Renal involvement may occur between 40 and 100% of cases. The disease varies from asymptomatic to fulminant rapidly progressive glomerulonephritis forms, leading to terminal renal failure in days. Methodology We present a case of a patient diagnosed with WG in the Hospital de San José in Bogotá, Colombia, and a literature review focused on renal involvement. Literature search was conducted in the following databases: PubMEed, LILAC, SciELO and Cochrane. Articles in english and spanish were selected and no restriction date was made, but at least 70% of selected references were published after 2010. Conclusions Renal involvement in patients with WG is common and can be lethal. A high percent may even require renal replacement therapy, although patients can return to normal renal function. Factors associated with return to normal renal function are unclear, but it depends on early therapy and high doses of immunomodulators. Rituximab has shown greater effectiveness and fewer side effects than conventional treatments, so it's a valid therapeutic option for the management of patients with WG and renal involvement.

Resumen El presente manuscrito se hace con el fin de reportar un caso del tema en mención y realizar una revisión de la literatura disponible más reciente con respecto al diagnóstico y tratamiento. Antecedentes La granulomatosis de Wegener (GW) se caracteriza por ser una vasculitis de pequeños vasos que afecta generalmente la vía respiratoria alta, pulmones y riñones. La afección renal puede presentarse entre el 40 % y el 100 % de los casos. La enfermedad varía en su presentación clínica, desde formas asintomáticas hasta glomerulonefritis rápidamente progresiva, llevando a la insuficiencia renal terminal en pocos días. Metodología Se realiza la presentación del caso de una paciente diagnosticada con granulomatosis de Wegener (GW) en el Hospital San José (Bogotá, Colombia), y se muestra una revisión de la literatura enfocada en el compromiso renal de la patología. La revisión bibliográfica se realizó en las bases de datos: PubMEed, LILAC, SciELO y Cochrane, seleccionando para revisión artículos en inglés y español, sin restricción de fecha, pero asegurándose de que el 70 % de las referencias seleccionadas se hubieran publicado después del año 2010. Conclusiones El compromiso renal en pacientes con GW es frecuente y puede llegar a ser letal. Una proporción de pacientes considerable puede llegar a requerir terapia de reemplazo renal, con potencial recuperación de la función renal. No están claros los factores que se asocian al retorno de la función renal normal, pero sí se ha descrito que se asocia a tratamiento temprano con altas dosis de inmunomoduladores. El rituximab es un medicamento con uso frecuente en estos pacientes, que ha demostrado efectividad y menores efectos secundarios que los tratamientos convencionales, constituyendo una opción válida para el manejo de pacientes con GW y compromiso renal asociado.

Estenosis subglótica como manifestación inicial de granulomatosis de Wegener en una adolescente: Caso clínico / Subglottic stenosis as the initial manifestation of Wegener's granulomatosis in a teenager: Case report

La granulomatosis de Wegener es una vasculitis granulomatosa necrotizante de origen autoinmune que afecta, principalmente, a la vía aérea superior e inferior y los riñones. Es muy rara en los niños y adolescentes. Cuando se inicia a edad temprana, se asocia, con frecuencia, a estenosis subglótica. La estenosis subglótica es una manifestación potencialmente fatal de la granulomatosis de Wegener. Su diagnóstico requiere un alto grado de sospecha porque puede desarrollarse en ausencia de otros signos de actividad y, en ocasiones, es la manifestación inicial de la enfermedad. Se confirma mediante la visualización endoscópica de la lesión. El tratamiento es complejo; requiere, a menudo, de repetidas intervenciones, debido a reestenosis. Presentamos a una paciente de 13 años de edad con estenosis subglótica secundaria a granulomatosis de Wegener. Describimos las manifestaciones clínicas, el diagnóstico y el tratamiento de esta rara causa de dificultad respiratoria en la población pediátrica.

Wegener's granulomatosis is a necrotizing granulomatous vasculitis of autoimmune origin that primarily affects the upper and lower airways and kidneys. It is very rare in children and adolescents. When started at a young age it is often associated with subglottic stenosis. Subglottic stenosis is a potentially fatal manifestation of Wegener's granulomatosis. Its diagnosis requires a high index of suspicion since it might develop in the absence of other signs of activity. Occasionally, subglottic stenosis may present as the initial manifestation of the disease. Diagnosis is confirmed by endoscopic visualization of the lesion. The treatment is complex, and it often requires repeated interventions due to restenosis. We present a 13-year-old patient with subglottic stenosis secondary to Wegener's granulomatosis. We describe the clinical manifestations, diagnosis and treatment of this rare cause of respiratory distress in the pediatric population.

Uso de rituximab en queratopatía ulcerativa periférica y uveítis secundaria a granulomatosis con poliangeítis / Use of rituximab for granulomatosis with polyangiitis-associated peripheral ulcerative keratitis

RESUMEN Mujer de 31 años con cuadro de poliartralgias, poliartritis asimétrica, fiebre, lesiones vasculíticas en piel, asociado a disminución de agudeza visual en ojo derecho, el cual presentaba compromiso corneal con adelgazamiento periférico severo, queratitis ulcerativa periférica, hipopión de fibrina y sinequias posteriores. Ante el cuadro clínico y paraclínicos con C-ANCA positivos, se realiza el diagnóstico de granulomatosis con poliangeítis con compromiso ocular (adelgazamiento corneal, queratitis ulcerativa periférica, uveítis) y sistémico. Se inicia tratamiento con metilprednisolona y ciclofosfamida con poco control, por lo que se inicia rituximab sistémico, evolucionando hacia la mejoría.

ABSTRACT A 31-year-old female, presented with polyarthralgia, asymmetrical polyarthritis, fever, vasculitis skin lesions, together with decreased visual acuity in the right eye with severe peripheral corneal melting, peripheral ulcerative keratitis, fibrin hypopyon and posterior synechiae. Given the clinical and paraclinical findings with positive C-ANCA (anti-neutrophil cytoplasmic antibodies), the patient was diagnosed with granulomatosis with polyangiitis with ocular (corneal melting, peripheral ulcerative keratitis and uveitis), and systemic involvement. Treatment was started with methylprednisolone and cyclophosphamide, but due to poor control, Rituximab was subsequently administered, with medical improvement.

IgG4 immunohistochemistry in granulomatosis with polyangiitis / 中国耳鼻咽喉头颈外科

OBJECTIVE To obser ve t he histopathological features and immunohistochemical expression of IgG4 in nasal granulomatosis with polyangiitis(GPA) and to study the relationship between GPA and IgG4-related disease(IgG4-RD). METHODS A total of 12 nasal GPA patients were collected from the Department of Pathology, Beijing TongRen Hospital during January 2012 to December 2016. EnVision immunohistochemical technique was used to detect the expressions of CD38, CD138, IgG and IgG4. RESULTS Nine cases were found pulmonary abnormalities. Elevated titer of anti-neutrophil cytoplasmic antibody was presented in 8 cases in this study. On histopathologic evaluation, 8 of 12 cases were graded as pathologic score 3, one as score 2, and the remaining 3 as score 1. Eight cases showed increased number of IgG4+ cells higher than 10/HPF, 2 of which showed the ratio of IgG4+/IgG cells were more than 40％. CONCLUSION IgG4+ cells can be increased in biopsies of nasal GPA. Since the morphologic and clinical manifestations of GPA and IgG4-RD may overlap, it could be a significant diagnostic pitfall in the differential diagnosis of these two entities. The diagnosis of GPA should combine clinical manifestations, imaging findings, serological examinations and pathological features.

A case report of the vasculitis misdiagnosed as pneumonia with stubborn heart failure / 天津医药

Wegener's granulomatosis (WG) is a kind of systemic necrotizing granulomatous vasculitis characterized by capillary and small arteriovenous involvement.The typical WG triad is lesions of the upper and lower respiratory tract and kidneys.Only changes in the respiratory tract without renal lesions are localized WG.Localized WG is very easy to be misdiagnosed because of its nonspecific symptoms and radiological manifestations.The author found one case of localized WG,which showed pulmonary symptoms and refractory heart failure.The chest CT showed that multiple irregular nidus,blurred and density enhanced lesions in both lungs.The patient was misdiagnosed as pulmonary infection for a long time.The patient was eventually diagnosed as asantineutrophil cytoplasmic antibody (ANCA) associated vasculitis and WG after two times of examination of protease-3(PR3) ANCA were positive.This article reviewed the diagnosis and treatment of the patient,and reviewed the relevant literatures.

A Retrospective Analysis of Granulomatosis with Polyangiitis with Ocular Manifestations

PURPOSE: To analyze the treatment and prognosis of patients from our tertiary medical center with ocular and orbital involvement of granulomatosis with polyangiitis. METHODS: A retrospective analysis of the medical records of patients diagnosed with granulomatosis with polyangiitis that visited our single tertiary referral center from July 2008 to September 2014 was performed. RESULTS: A total of 51 patients diagnosed with granulomatosis with polyangiitis visited our center, and 21 of those patients had received an ophthalmologic examination. Of these, 9 patients (4 males, 5 females) had symptoms of the eye and orbit, and the clinical presentations were as follows: episcleritis, scleritis, marginal keratitis, orbital inflammation, orbital abscess, retinal vasculitis, and nasolacrimal duct obstruction. The patients each received treatments according to clinical presentation with topical, oral, or intravenous steroids or immunomodulatory agents such as cyclophosphamide. Nasolacrimal duct obstruction was treated with surgery in some cases. After an average follow-up period of 58 ± 30 months, all patients showed clinical improvement of their ocular and orbital involvement of granulomatosis with polyangiitis. CONCLUSIONS: Granulomatosis with polyangiitis is a relatively rare disease that sometimes has ocular or orbital involvement and can lead to blindness. Therefore, when ocular symptoms and signs present without a definitive cause, granulomatosis with polyangiitis must be ruled out, and appropriate treatment is needed. However, there are few published reports on the clinical presentation and prognosis of ocular and orbital involvement of granulomatosis with polyangiitis in Asians. This study showed that the incidence of ocular and orbital involvement in granulomatosis with polyangiitis was lower than previous reports.

A Retrospective Analysis of Granulomatosis with Polyangiitis with Ocular Manifestations

PURPOSE: To analyze the treatment and prognosis of patients from our tertiary medical center with ocular and orbital involvement of granulomatosis with polyangiitis. METHODS: A retrospective analysis of the medical records of patients diagnosed with granulomatosis with polyangiitis that visited our single tertiary referral center from July 2008 to September 2014 was performed. RESULTS: A total of 51 patients diagnosed with granulomatosis with polyangiitis visited our center, and 21 of those patients had received an ophthalmologic examination. Of these, 9 patients (4 males, 5 females) had symptoms of the eye and orbit, and the clinical presentations were as follows: episcleritis, scleritis, marginal keratitis, orbital inflammation, orbital abscess, retinal vasculitis, and nasolacrimal duct obstruction. The patients each received treatments according to clinical presentation with topical, oral, or intravenous steroids or immunomodulatory agents such as cyclophosphamide. Nasolacrimal duct obstruction was treated with surgery in some cases. After an average follow-up period of 58 ± 30 months, all patients showed clinical improvement of their ocular and orbital involvement of granulomatosis with polyangiitis. CONCLUSIONS: Granulomatosis with polyangiitis is a relatively rare disease that sometimes has ocular or orbital involvement and can lead to blindness. Therefore, when ocular symptoms and signs present without a definitive cause, granulomatosis with polyangiitis must be ruled out, and appropriate treatment is needed. However, there are few published reports on the clinical presentation and prognosis of ocular and orbital involvement of granulomatosis with polyangiitis in Asians. This study showed that the incidence of ocular and orbital involvement in granulomatosis with polyangiitis was lower than previous reports.

Afectación cerebral en la granulomatosis de Wegener. Reporte de caso / Brain involvement in Wegener's granulomatosis. Case report

La granulomatosis con poliangeítis (GPA) ­antes denominada granulomatosis de Wegener­ es una enfermedad autoinmunitaria multisistémica poco frecuente, de etiología desconocida. En esta enfermedad el compromiso del sistema nervioso central es muy raro aunque tiene mucha importancia porque se relaciona con períodos refractarios. El diagnóstico diferencial de las manifestaciones cerebrales de la GPA es difícil porque no hay signos radiográficos excluyentes de otras lesiones intra o extra-axiales. Este artículo presenta el caso de un paciente con granulomatosis de Wegener de larga evolución, que debuta con crisis convulsivas y focalidad neurológica secundarias a una lesión intraaxial parietal izquierda. Las características sintomáticas, histopatológicas y radiográficas se describen cuidadosamente y se discute el papel de la cirugía para esclarecer el diagnóstico en este paciente. (AU)

The granulomatosis with polyangiitis (GPA), known before as Wegener's granulomatosis, is an autoimmune multisystemic disease, not so common, of unknown etiology. The compromise of this particular disease to the neurological central system is very rare although it's very important because of its relationship with refractory disease periods. The differential diagnosis of the manifestations of the GPA at cerebral level it's not easy to see because there are not that allows the exclusion of other lesions intra or extra axials. We present the case of the patient with Wegener granulomatosis of long evolution that begins with seizures and motor deficit secondary to a left parietal intra axial mass. The clinical characteristics histopathological and radiological are careful described and the role of surgery in management is discussed. (AU)

Concurrent Extracorporeal Membrane Oxygenation, Plasmapheresis and Continuous Renal Replacement Therapy in a Case of Wegener’s Granulomatosis.

Wegener's Granulomatosis is one of the ANCA-associated vasculitis characterized by multifocal vascular necrotizing inflammation and granulomas that commonly present with a pulmonary-renal syndrome. We report a case of 38 year-old female patient who presented with acute respiratory distress syndrome due to diffuse alveolar hemorrhage and multiorgan failure. The patient being in a hemodynamic collapse was aggressively intubated and supported by mechanical ventilation and vasopressor therapy. Despite all effort refractory hypoxemia could not be coped with. Venovenous extracorporeal membrane oxygenation was started through a femoral and jugular vein. Her oxygenation dramatically ameliorated after the beginning of this procedure. 1 gr/day pulse metylprednisolone was administered followed by cyclophosphamide. Concurrent continious renal replacement therapy and plasmapheresis was begun. Her clinical status improved in days giving rise to weaning of the extracorporeal membrane oxygenation by the day twelve. Extracorporeal membrane oxygenation is reported to be a successful mode of therapy in various cases in the literature including diffuse alveolar hemorrhage due to ANCA-associated vasculitis and concurrent therapy with continuous venovenous hemodialysis is reported to increase survival. Clinicians should be encouraged to use this life saving mode of treatment more often.

Estenosis subglótica en pacientes con granulomatosis con poliangeítis / Subglottic stenosis in patients with granulomatosis with polyangiitis

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.

Granulomatosis con poliangeítis con una presentación otorrinolaringológica

La granulomatosis con poliangitis (GPA), previamente conocida como granulomatosis de Wegener (GW), es una enfermedad autoinmune sistémica. Presentamos el caso de una adolescente de 14 años de edad que acude a consulta con otalgia, otorrea, secreción sanguinolenta bilateral asociada a tinnitus, hipoacusia, cefalea, pérdida de peso de +/- 20 libras, fiebre intermitente no cuantificada , y antecedentes patológicos de sinusitis y asma bronquial. La otoscopía evidenció granulomas a nivel del piso de ambos conductos auditivos externoas y sobre la membrana timpánica. Posteriormente se realiza biopsia que reporta inflamación crónica de aspecto granulomatoso con necrosis, asociado ANCA PR3 positivo. Con los hallazgos anteriores se concluye el diagnóstico de GPA. Ha sido tratado exitosamente con medicamentos citotóxicos e inmunosupresores. El manejo apropiado con estos medicamentos ha mejorado la expectativa de vida de los pacientes con GPA.

Granulomatosis with polyangitis (GPA), previously known as Wegener's granulomatosis (GW), is a systemic autoimmune disease. A male of a 14-years who comes to the hospital with a chief complain of ear pain,otorrhea, bilateral bloody discharge associated with tinnitus, hearing loss, headache, weight loss of +/- 20 pounds and occasional unquantified intermittent, fever that has history of sinusitis and bronchial asthma. Otoscopy revealed granulomas at the floor level of both external auditory passages and the tympanic membrane. Subsequently, a biopsy is performed that reports chronic granulomatous inflammation with necrosis associated with ANCA PR3 positive. With the previously findings we can diagnose GPA. It has been successfully treated with cytotoxic and immunosuppressive drugs for the management and control of this disease. It has dramatically increased life expectancy.