Granulomatosis con poliangeítis. A propósito de un caso

La granulomatosis con poliangeítis es una vasculitis sistémica, necrosante y granulomatosa que afecta el tracto respiratorio superior e inferior y los riñones. Se presenta el caso de una paciente blanca, de 59 años de edad, hipertensa y fumadora inveterada, que ingresó por presentar síntomas constitucionales, orinas turbias y hemáticas, así como creatininemia elevada con valor previo normal. Con impresión diagnóstica inicial de infección del tracto urinario se indica antibioticoterapia. Después del tercer día de iniciado el tratamiento, mejoraron los síntomas constitucionales y, de forma progresiva, comenzó con manifestaciones multiorgánicas (renal, respiratoria, cutánea y musculoesquelética) sugestivas de vasculitis sistémica. Como elementos significativos, en exámenes complementarios se detectaron azoados y reactantes de fase aguda aumentados, sedimento urinario activo, imagen nodular en vértice pulmonar derecho y microhemorragias multifocales en ambos campos pulmonares, detectados mediante tomografía computarizada y c-ANCA altamente positivo. Se indicó tratamiento inmunosupresor y citotóxico potente, obteniéndose control inicial de las manifestaciones graves de la enfermedad. Evolutivamente desarrolló complicaciones propias de la entidad y secundarias al tratamiento médico, que la hicieron tributaria de terapia de sustitución renal, falleciendo a los dos años de recibir hemodiálisis iterada. El cuadro clínico, unido a los estudios imagenológicos e inmunológicos (c-ANCA), fueron elementos claves para realizar el diagnóstico.

Granulomatosis with polyangiitis is a systemic, necrotizing, granulomatous vasculitis that affects the upper and lower respiratory tract and kidneys. The case of a 59-year-old white female patient, who is hypertensive and inveterate smoker, is presented; she was admitted for presenting constitutional symptoms, turbid and bloody urine, as well as elevated creatininemia with a normal previous value. With the initial diagnostic impression of urinary tract infection, antibiotic therapy was indicated. After the third day of starting treatment the constitutional symptoms improved, and progressively began with multiorgan manifestations (renal, respiratory, skin and musculoskeletal) suggestive of systemic vasculitis. As significant elements, complementary examinations detected increased nitrogen and acute phase reactants, active urinary sediment, nodular image in the right lung apex and multifocal microhemorrhages in both lung fields through computed tomography, and highly positive c-ANCA. Potent immunosuppressive and cytotoxic treatment was indicated, obtaining initial control of severe manifestations of the disease. Evolutionarily she developed complications specific to the entity and secondary to medical treatment which made her subject to renal replacement therapy, dying two years after receiving iterated hemodialysis. The clinical characteristics, together with the imaging and immunological studies (c-ANCA) were key elements to make the diagnosis.

Granulomatosis con poliangeítis simulando una torsión testicular / Granulomatosis with poliangiitis mimicking testicular torsion

La granulomatosis con poliangeítis (GPA) es una vasculitis autoinmune que raramente afecta al tracto genitourinario inferior. Presentamos el caso de un varón de 53 años que debutó con una masa retroperitoneal y posteriormente desarrolló un hidrocele multiseptado izquierdo que condicionó un infarto testicular. El informe anatomopatológico de la orquiectomía reveló hallazgos sugestivos de GPA.(AU)

Granulomatosis with polyangiitis (GPA) is an autoimmune vasculitis which rarely affects the lower genitourinary tract. We share the case of a 53-year-old man who presented with a retroperitoneal mass and thereafter developed a left multiseptated hydrocele that conditioned a testicular infarction. The pathology report of the orchidectomy was consistent with GPA.(AU)

Granulomatosis with poliangiitis mimicking testicular torsion.

Granulomatosis with polyangiitis (GPA) is an autoimmune vasculitis which rarely affects the lower genitourinary tract. We share the case of a 53-year-old man who presented with a retroperitoneal mass and thereafter developed a left multiseptated hydrocele that conditioned a testicular infarction. The pathology report of the orchidectomy was consistent with GPA.

Granulomatosis with polyangiitis: A case report of early systemic fulminant presentation

Granulomatosis with polyangiitis is a systemic vasculitis that affects medium and small vessels, with high expression of anti-neutrophil cytoplasmic autoantibody. A case is pre sented on a patient with an initial compromise of the lower airway, who did not respond to management, required intensive care unit management, and died due to severe diffuse alveolar hemorrhage. His definitive diagnosis was established with a clinical autopsy. Gran-ulomatosis with polyangiitis is a disease with different ways of presentation, and can have fatal outcomes if it is not diagnosed early.

La granulomatosis con poliangeítis es un tipo de vasculitis que afecta a vasos de mediano y pequeño calibre de manera sistémica, con una alta expresión de anticuerpos contra el citoplasma del neutrófilo. Se presenta el caso de un paciente con un compromiso inicial de la vía área inferior, que no respondió al tratamiento y requirió manejo en unidad de cuidados intensivos. Finalmente, falleció por una hemorragia alveolar difusa severa. Su diagnóstico definitivo se estableció con una autopsia clínica. La granulomatosis con poliangeítis tiene diferentes formas de presentación y puede tener desenlaces fatales si no se diagnostica a tiempo.

Granulomatose de Wegener: reabilitação oral com prótese bucomaxilofacial / Wegener's granulomatosis: oral rehabilitation with maxillofacial prosthesis / Granulomatosis de Wegener: rehabilitación oral con prótesis bucomaxilofacial

Introdução: A granulomatose de Wegener é uma doença autoimune rara que ocorre através de mediadores imunológicos. Sua etiologia permanece desconhecida. No entanto, sabe-se que é caracterizada principalmente pela inflamação dos vasos sanguíneos que acometem preferencialmente as vias aéreas superiores, inferiores e os rins. A doença apresenta altas taxas de morbidade e mortalidade quando não tratada, seu principal tratamento é através do uso de corticoides e imunossupressores. Objetivo: realizar um relato de caso de Granulomatose de Wegener tratando a comunicação orosinusal através de prótese bucomaxilofacial e uma discussão baseada na literatura recente. Relato de caso: paciente do gênero masculino, 40 anos de idade, leucoderma, encaminhado ao serviço de Bucomaxilofacial do Hospital Universitário da Universidade Federal de Santa Catarina (HU-UFSC) queixando-se de comunicação oronasal em região de palato. Foi tratado com prótese BMF obturadora, o que devolveu ao paciente a capacidade de fonação, deglutição e convívio social. Conclusão: o tratamento com a prótese BMF reestabelece o velamento velofaríngeo, corrige hipernasalidade, melhora a deglutição e dá conforto psicológico ao paciente com estabilidade protética funcional... (AU)

Introduction: Wegeners' Granulomatulosis is a rare autoimmune disease that acts through immunologic mediators. It's etymology remains unknown. However, it is known that it's mainly characterized by the inflammation of blood vessels that affect, by preference, upper and lower airways, as well as kidneys. The disease presents high rates of morbidity and mortality when not treated, and it's main treatments are corticoids and immunosuppressants. Objective: Perform a Wegener's Granulomatulosis case report treating orosinusal communication through the use of a bucomaxilofacial prosthesis and a discussion based on current literature. Case report: Male patient, 40 years of age, leucoderma, brought to the Bucomaxilofacial department of Federal University of Santa Catarina (HU UFSC) with complaints regarding oronasal communication in the palate region. The patient was treated with a BMF prosthetics, which has allowed the patient to resume normal social activities, as well as returning phonation and deglutition. Conclusion: Treatment with a BMF prosthetics reestablishes velopharyngeal veiling, assists in the correction of nasal voice, improves deglutition and offers the patient psychological comfort with functional prosthetic stability... (AU)

Introducción: la granulomatosis de Wegener es una enfermedad autoinmune rara que se presenta a través de mediadores inmunológicos. Su etiologia permanece desconocida. Sin embargo, se sabe que se caracteriza principalmente por la inflamación de los vasos sanguíneos que afectan preferentemente las vías respiratorias superiores e inferiores y los riñones. La enfermedad tiene altas tasas de morbilidad y mortalidad cuando no se trata, su principal tratamiento es mediante el uso de corticosteroides e inmunosupresores. Objetivo: realizar un reporte de caso de Granulomatosis de Wegener en el tratamiento de la comunicación orosinusal mediante prótesis maxilofacial y una discusión basada en la literatura reciente. Caso clínico: paciente de sexo masculino, 40 años, leucoderma, remitido al Servicio Maxilofacial del Hospital Universitario de la Universidad Federal de Santa Catarina (HU-UFSC) por comunicación oronasal en región paladar. Fue tratado con una prótesis obturatriz de BMF, que le devolvió al paciente la capacidad de hablar, tragar y socializar. Conclusión: el tratamiento con la prótesis BMF restablece el velo velofaríngeo, corrige la hipernasalidad, mejora la deglución y proporciona confort psicológico al paciente con estabilidad protésica funcional... (AU)

Serum cytokines in ANCA-associated vasculitis: Correlation with disease-related clinical and laboratory findings / Citocinas séricas en la vasculitis asociada a ANCA: correlación con los hallazgos clínicos y de laboratorio relacionados con la enfermedad

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic, autoimmune disease. Cytokine dysregulation during active disease and clinical remission, reflects significant immunological activity in various disease stages, and might be responsible for the potential relapse of ANCA-vasculitis.ObjectivesThis study aimed to screen serological profiles in active granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), and to determine their associations with clinical characteristics.Materials and methodsSerum IL-10, IL-12, IL-17, IL-21, IL-23, B cell activating factor (BAFF) concentrations were determined by Quantikine HS ELISA in 71 patients, 47 with GPA and 24 with MPA, and compared with 16 healthy controls. Subsequently, the correlations between serum IL-10, IL-12, IL-17, IL-21, IL-23, BAFF levels, and both laboratory and clinical abnormalities were investigated.ResultsBAFF levels were significantly higher in GPA than MPA, and healthy controls. IL-10 and BAFF levels were elevated in GPA patients with pulmonary involvement. Higher BAFF levels might reflect severe GPA. IL-10 and IL-12 levels were higher in MPA than GPA. In MPA, IL-10 levels were highest in patients with short disease duration, and young individuals. IL-12 correlated positively with BVAS and was elevated in patients with cardiovascular involvement and nasal S. aureus carriers.ConclusionsIn MPA, IL-12 correlates positively with disease activity, and is significantly increased in patients with cardiovascular involvement and nasal S. aureus carriers. Increased IL-10 is observed in young MPA patients and in those with short MPA duration. Elevated BAFF and IL-10 levels are associated with pulmonary involvement in GPA. High BAFF levels might reflect severe GPA. (AU)

Antecedentes: Las vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos (ANCA) son enfermedades sistémicas autoinmunes. La desregulación de las citocinas durante la enfermedad activa y la remisión clínica refleja una actividad inmunológica significativa en diversas etapas de la enfermedad, pudiendo ser presumiblemente responsable de la recidiva potencial de la vasculitis ANCA.ObjetivosEl objetivo de este estudio fue cribar los perfiles serológicos de la granulomatosis con poliangeítis activa (GPA) y la poliangeítis microscópica (PAM), así como determinar sus asociaciones con las características clínicas.Materiales y métodosSe determinaron las concentraciones séricas de IL-10, IL-12, IL-17, IL-21, IL-23, y el factor activador de célula B (BAFF) utilizando quantikine® HS ELISA en 71 pacientes, 47 con GPA y 24 con PAM, comparándose con 16 controles sanos. A continuación, se estudiaron las correlaciones entre los niveles séricos de IL-10, IL-12, IL-17, IL-21, IL-23 y BAFF, así como las alteraciones de laboratorio y clínicas.ResultadosLos niveles de BAFF fueron significativamente más altos en GPA que en PAM y los controles sanos. Los niveles de IL-10 y BAFF fueron elevados en los pacientes de GPA con compromiso pulmonar. Los niveles más altos de BAFF podrían reflejar presumiblemente GPA severa. Los niveles de IL-10 eIL-12 fueron más altos en PAM que en GPA. En PAM, los niveles de IL-10 fueron más altos que en pacientes con corta duración de la enfermedad y en individuos jóvenes. IL-12 guardó una correlación positiva con puntuación de actividad de vasculitis de Birmingham (BVAS), y los niveles fueron elevados en pacientes con compromiso cardiovascular y portadores de S. aureus nasal. (AU)

Paquimeningitis hipertrófica y tumoración orbitaria asociada a Granulomatosis con poliangeitis: reporte de un caso clínico / Hypertrophic pachymeningitis and orbital tumour associated with Granulomatosis with polyangiitis: case report

Paciente de 65 años, con diagnóstico de Granulomatosis con Poliangeitis (GPA) de 18 años de evolución cuyo debut fue por insuficiencia respiratoria aguda asociado a hemoptisis recibiendo tratamiento con corticoides sistémicos y ciclofosfamida de inducción. Luego recibió mantenimiento con azatioprina 150 mg día, con periodos de recrudecimiento de enfermedad que respondieron al tratamiento con corticoides por períodos cortos. Acude a consulta por cefalea crónica de tres meses de evolución refractaria al tratamiento con antiinflamatorios no esteroides (AINES), asociado a proptosis ocular izquierda y dolor orbitario homolateral, presentando reactantes de fase aguda elevados (eritrosedimentación y Proteína C reactiva). Se evidencia por resonancia magnética nuclear cerebral con gadolinio, realce de la duramadre cerebral y tienda de cerebelo, presentando además una formación orbitaria izquierda.

A 65-year-old patient, with a diagnosis of Granulomatosis with Polyangeitis (GPA) of 18 years of evolution, whose debut was with respiratory failure and hemoptysis, receiving induction treatment with corticosteroids together with cyclophosphamide, and then maintenance treatment with azathioprine 150 mg per day, with periods of flare-up of the disease that responded to treatment with corticosteroids for short periods. He came to the clinic for a 3-month-long chronic headache refractory to treatment with non-steroidal anti-inflammatory drugs (NSAIDs), associated with left ocular proptosis and ipsilateral orbital pain, presenting elevated acute phase reactants (ers and c-reactive protein). It is evidenced by brain magnetic resonance with gadolinium, enhancement of the cerebral dura and cerebellum store, also presenting formation in the left orbit.

[Serum cytokines in ANCA-associated vasculitis: Correlation with disease-related clinical and laboratory findings]. / Citocinas séricas en la vasculitis asociada a ANCA: correlación con los hallazgos clínicos y de laboratorio relacionados con la enfermedad.

BACKGROUND: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic, autoimmune disease. Cytokine dysregulation during active disease and clinical remission, reflects significant immunological activity in various disease stages, and might be responsible for the potential relapse of ANCA-vasculitis. OBJECTIVES: This study aimed to screen serological profiles in active granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), and to determine their associations with clinical characteristics. MATERIALS AND METHODS: Serum IL-10, IL-12, IL-17, IL-21, IL-23, B cell activating factor (BAFF) concentrations were determined by Quantikine HS ELISA in 71 patients, 47 with GPA and 24 with MPA, and compared with 16 healthy controls. Subsequently, the correlations between serum IL-10, IL-12, IL-17, IL-21, IL-23, BAFF levels, and both laboratory and clinical abnormalities were investigated. RESULTS: BAFF levels were significantly higher in GPA than MPA, and healthy controls. IL-10 and BAFF levels were elevated in GPA patients with pulmonary involvement. Higher BAFF levels might reflect severe GPA. IL-10 and IL-12 levels were higher in MPA than GPA. In MPA, IL-10 levels were highest in patients with short disease duration, and young individuals. IL-12 correlated positively with BVAS and was elevated in patients with cardiovascular involvement and nasal S. aureus carriers. CONCLUSIONS: In MPA, IL-12 correlates positively with disease activity, and is significantly increased in patients with cardiovascular involvement and nasal S. aureus carriers. Increased IL-10 is observed in young MPA patients and in those with short MPA duration. Elevated BAFF and IL-10 levels are associated with pulmonary involvement in GPA. High BAFF levels might reflect severe GPA.

Hypertrophic pachymeningitis in a patient with ANCA negative granulomatosis with polyangiitis: A case report / Paquimeningitis hipertrófica en un paciente con granulomatosis con poliangeítis ANCA negativo: reporte de un caso

ABSTRACT Granulomatosis with polyangiitis (GPA) is a vasculitic disease with an infrequent involvement of the central nervous system. This can lead, in rare cases, to hypertrophic pachymeningitis (HP), which is characterized by inflammation and fibrosis that cause a thickening of dura mater. At present, it is crucial to consider GPA in the differential diagnosis of elderly patients with intracranial hypertension. The case is presented of a 60-year-old male with progressive severe headache, vomiting, and wasting syndrome. Physical examination showed pallor, weight loss, and unilateral papilloedema. A gadolinium-enhanced brain MRI scan showed sinusitis, chronic otomastoiditis, and hypertrophic pachymeningitis. Finally, a meningeal biopsy concluded a necrotising granulomatous vasculitis compatible with GPA. However, PR3- and MPO-ANCA were negative. After corticosteroid therapy was initiated, the patient had a favorable outcome during his hospital stay.

RESUMEN La granulomatosis con poliangeítis (GPA) compromete excepcionalmente el sistema nervioso central, conllevando en raras ocasiones a una paquimeningitis hipertrófica (PH), caracterizada por inflamación y fibrosis, que originan un engrosamiento de la duramadre. Actualmente, su consideración es crucial en el diagnóstico diferencial de pacientes ancianos con hipertensión endocraneana. Presentamos el caso de un adulto de 60 anos con cefalea severa progresiva, vómitos, papiledema unilateral y síndrome consuntivo en donde la resonancia magnética cerebral contrastada con gadolinio muestra sinusitis, otomastoiditis y PH. Finalmente, la biopsia de meninges reveló vasculitis granulomatosa necrosante de pequenos y medianos vasos compatible con GPA. Empero, PR3- y MPO-ANCA resultaron negativos. Se inició terapia con corticoides, presentando una evolución clínica favorable durante su hospitalización.

Acute myocardial infarction, as a rare manifestation in granulomatosis with polyangiitis.

La granulomatosis con poliangeítis es una vasculitis de pequeños vasos asociada a la presencia de anticuerpos anticitoplasma de neutrófilos, con manifestaciones cardíacas que son poco frecuentes, como pericarditis, miocarditis, arteritis coronaria y enfermedad valvular. Reportamos el caso de un paciente de 49 años con reciente diagnóstico de granulomatosis con poliangeítis, quien presentó infarto agudo del miocardio. Se consideró la actividad de la enfermedad como causa del infarto. Las manifestaciones clínicas cardiovasculares en la granulomatosis con poliangeítis son relevantes por ser marcadores de mal pronóstico.Granulomatosis with polyangiitis is a small vessel vasculitis associated to anti-neutrophil cytoplasmic antibodies, in which the cardiac manifestations are not common, as pericarditis, cardiomyopathy, coronary artery disease and vascular disease. We report a clinical case of a 49-year-old man with a recent diagnosis of granulomatosis with polyangiitis, he presented myocardial infarction. Disease activity was considered the cause of myocardial infarction. Cardiovascular clinical manifestations in granulomatosis with polyangiitis are relevant because are markers of poor prognosis.

Proptosis ocular como una manifestación de la granulomatosis con poliangeítis (Wegener): Reporte de un caso y revisión de la literatura / Ocular proptosis as a manifestation of Wegener's granulomatosis: A case report

RESUMEN La granulomatosis con poliangeítis (Wegener) es considerada como una enfermedad granulomatosa sistémica, no infecciosa, caracterizada histológicamente por una vasculitis necrosante de pequeño vaso. El tracto respiratorio superior e inferior son los más frecuentemente afectados, en asociación a manifestaciones renales. Sin embargo, también se describe el compromiso aislado de un solo órgano, como es el caso del globo ocular y la órbita. Presentamos el caso de una mujer cuya principal manifestación consistió en un proceso inflamatorio de tejido periorbitario y proptosis del globo ocular izquierdo. La paciente fue valorada en manejo conjunto con el servicio de plástica ocular, otorrinolaringología y finalmente se derivó a reumatología para inicio de terapia inmunosupresora. Se realizó una revisión de la literatura, en búsqueda de reportes de caso y series de caso que permitieran comparar las presentaciones clínicas y desenlaces más frecuentes.

A B S T R A C T Granulomatosis with polyangiitis (Wegener) is considered as a systemic, non-infectious granulomatous disease, histologically noted for its small vessel necrotising vasculitis. The upper and lower respiratory tracts are the most frequently affected, in association with renal manifestations. However, the isolated involvement of a single organ, such as the eyeball and the orbit is also described. The case is presented of a woman whose main manifestation was an inflammatory process of periorbital tissue and proptosis of the left eyeball. The patient was evaluated jointly with eye plastic surgery, and the ear, nose and throat, and rheumatology departments. A literature review was carried out, looking for case reports and case series that allowed comparisons to made between the clinical manifestations and the most frequent outcomes.

Lesiones destructivas de la línea media: diagnóstico diferencial con Vasculitis Asociada a ANCA / Destructive lesions of midline: differential diagnosis with ANCA-associated Vasculitis

Presentamos los casos clínicos de tres pacientes adultos jóvenes de origen boliviano, que fueron hospitalizados en salas de clínica médica de un hospital de tercer nivel por manifestaciones de la vía aérea superior y lesiones de las estructuras de la línea media. Reumatología evaluó la posibilidad del diagnóstico de Vasculitis asociada a ANCA, la cual es un diagnóstico diferencial de la entidad conocida como "lesión destructiva de la línea media". En todos los casos se arribó al diagnóstico definitivo de Linfoma T luego de un exhaustivo estudio histopatológico.

We present clinical cases of three Bolivian young adults who were hospitalized in the medical clinic rooms of a third level hospital for upper airway manifestation and lesions of the midline structures. Rheumatology service evaluated the diagnosis of ANCA associated Vasculitis, which is a differential diagnosis of the entity known as midline destructive lesion. In all cases, the final diagnosis of T lymphoma was reached after an exhaustive histopathological study.

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.

Mycophenolate mofetil for induction and maintenance of remission in naïve patients with granulomatosis with polyangiitis without renal involvement.

Antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss). In this report we used mycophenolate mofetil (MMF) and steroids to induce and maintain remission in two newly diagnosed cases with c-ANCA associated GPA. The two patients' maintained remission with no disease relapses during one year follow-up.

Extraocular myositis as an initial manifestation of polyangiitis granulomatosis / Miositis extraocular como manifestación inicial de granulomatosis con poliangeítis

ABSTRACT Granulomatosis with polyangiitis (GPA) is a systemic vasculitis with multiorgan involvement. Although ocular involvement has frequently been described, there are few reported cases of extraocular myositis in this disease. The case is presented of a Mexican woman with GPA who debuted with extraocular myositis.

RESUMEN La granulomatosis con poliangeítis (GPA) es una vasculitis sistémica con afectación multiorgánica. El compromiso ocular ha sido descrito frecuentemente, sin embargo, existen pocos casos reportados de miositis extraocular en esta enfermedad. Presentamos el caso de una mujer mexicana con GPA que comenzó con miositis extraocular.

Hipoacusia y vasculitis cutánea, manifestación inicial de granulomatosis con poliangeítis en una niña de 14 años / Hypoacusis and cutaneous vasculitis as first clinical manifestation of granulomatosis with polyangiitis in a 14 year-old girl

Resumen La granulomatosis con poliangeítis (GPA) es una vasculitis de pequeno y mediano calibre caracterizada por la formación de granulomas e inflamación necrosante con predilección de las vías respiratorias y los glomérulos. Se reporta el caso de una adolescente de 14 an os que comienza con hipoacusia y lesiones cutáneas; sinusitis, epiescleritis, PR3-ANCA positivo y biopsia de piel con infiltrado neutrofílico y necrosis. La GPA puede presentar manifestaciones otológicas y dermatológicas como signo inicial, es importante tomarlo en consideración en el diagnóstico diferencial.

Abstract Granulomatosis with polyangiitis is a small- and medium-sized vasculitis. It is characterised by formation of granulomas and necrotising inflammation with a predilection for the respiratory tract and glomeruli. The case is presented of a 14-year-old female debuting with hypoacusis, skin lesions, sinusitis; episcleritis, anti-PR3 ANCA positivity and histological findings from the skin lesions with neutrophilic infiltration, necrosis and fibrin. Granulomatosis with polyangiitis can present otological and skin manifestations as initial signs, and it is important to take the differential diagnosis into consideration.

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.

Clinical Significance of Neutrophil/lymphocyte Ratio in Patients With Granulomatosis With Polyangiitis.

OBJECTIVE: To determine to neutrophil-to-lymphocyte ratio (NLR) in granulomatosis with polyangiitis (GPA) patients and to study its relation to disease manifestations and activity. METHODS: The study included 44 GPA patients and 44 matched age and sex controls. Full history taking, thorough clinical examination with more attention to ocular examination, laboratory and radiological investigations were considered. Disease activity was assessed using the Birmingham Vasculitis Activity Score (BVAS). RESULTS: The patients (21 males and 23 females) had a mean age of 45.66±7.24 years, disease duration 6.8±3.6 years and BVAS 50.1±14.3. All patients had a positive cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) while only 5 had a positive p-ANCA. The NLR was significantly increased in the GPA patients (5.1±2.4) compared to the control (1.5±0.8) (P<.0001). Ten patients with uveitis had a significantly higher NLR (6.5±1.9) compared to those without (4.7±2.4) (0.03) while those with proptosis (n=10), cutaneous manifestations (n=17) or ischemic heart disease (n=9) had a significantly lower NLR than those without (P=.0001, P=.017 and P=.046 respectively). The NLR did not significantly correlate with any of the patients' characteristics. The NLR inversely yet insignificantly correlated with the disease activity (r=-0.02, P=.93). CONCLUSION: The NLR may have a significant role in the pathogenesis of GPA, the development of uveitis or proptosis, cutaneous manifestations and ischemic heart disease. NLR may serve as a future potential companion to c-ANCA positivity in diagnosing and evaluating GPA and may play a role in the tissue-specific and clinical characteristics.

Tuberculosis, granulomatosis with polyangiitis, or both? A case report. / ¿Tuberculosis, granulomatosis con poliangeítis, o ambos? Caso clínico inusual.

INTRODUCTION: Tuberculosis (TB) is a chronic granulomatose infection, and granulomatosis with polyangiitis (GP) is a small vessel vasculitis, both of which affect the lungs. The combination of these diseases is rare. Both have similar clinical features, making the differential diagnosis difficult. CASE REPORT: It concerns a 37 year-old female undergoing treatment for pulmonary TB, who presented with left ocular proptosis, eyelid and conjunctival edema and erythema. Orbital biopsy revealed GP. C-Anti-neutrophil cytoplasmic antibodies were elevated. The patient responded well to immunosuppressive treatment. CONCLUSION: TB and GP can associate. Diagnosis should include not only C-anti-neutrophil cytoplasmic antibodies, but also a biopsy, in order to select the appropriate treatment.

Granulomatosis con Poliangeitis: caso clínico / Granulomatosis with Polyangiitis: clinical case

Introducción: La granulomatosis con poliangeitis conocida, previamente, como Granulomatosis de Wegener es una enfermedad multisistémica caracterizada por necrosis de las porciones superior e inferior del aparato respiratorio, glomerulonefritis y vasculitis necrosante. Su presentación es rara, la clínica suele ser aguda y su evolución fulminante. El patrón radiológico de la enfermedad incluye alteración parenquimatosa en el 50% de los pacientes, con áreas de consolidación y opacidades en vidrio esmerilado, secundarias a hemorragia pulmonar difusa, generalmente bilateral. La mediana de supervivencia con tratamiento específico es mayor a 20 años. Caso: Este es el caso de una paciente femenina menor a 40 años que presentó una masa cavitada pulmonar. Recibió varios esquemas antibióticos en otro hospital y también recibió terapia antifímica. En el Hospital Carlos Andrade Marín, el diagnóstico se estableció en base a los reportes histopatológicos de las biopsias de riñón y pulmón; y, con los resultados de pruebas inmunológicas específicas (ANCA PR3). La terapia con corticoesteroides y drogas inmunosupresoras hizo una gran diferencia en la evolución clínica y radiológica de la paciente. Discusión: La Granulomatosis con Poliangeitis es una inflamación necrosante que involucra la vía respiratoria y el riñón. Se define por la presencia de anticuerpos ANCA PR3 positivo en la mayoría de pacientes; tiene una evolución variable que tiende a ser crónica y se trata con corticosteroides e inmunosupresores. Después de entrar en remisión, la probabilidad de recurrencia de la enfermedad varía del 50 al 70%. Esto puede prevenirse con el seguimiento adecuado y la terapia de mantenimiento.

Introduction: Granulomatosis with poliangeitis previously known as Wegener's granulomatosis, is a multisystemic disease characterized by necrosis in the upper or lower portions of the respiratory system, glomerulonephritis and necrotizing vasculitis. Though a rare disease, its presentation is acute and rapidly progressive. The radiological pattern includes parenchymal alteration in 50% of the patients, with lung consolidation seen on the CT SCAN or ground glass opacities secondary to diffuse pulmonary hemorrhage, usually bilateral. Currently, the median survival time with treatment is longer than 20 years. Case: This is the case of a female patient younger than 40 years-old who had a cavitated mass in her lungs. She was transferred from another hospital after receiving several antibiotic schemes without improving her clinical condition. She also received antituberculous treatment. At CAM Hospital, the diagnosis was made on the ground of histopatologic reports and immunologic tests. Corticoisteroids and inmunosupressive treatment improved definitively her clinical and radiologic picture. Discussion: Granulomatosis with Poliangiitis is a necrotizing inflammation involving the respiratory tract, lungs and kidneys. It is defined by the presence of ANCA PR3 positive antibodies in most patients. Its evolution varies and often tends to be chronic. It is treated with corticosteroids and immunosuppressors. After remission, the probability of recurrence goes from 50 to 70%. This could be be prevented with adequate follow-up and maintenance treatment.