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.

Clinical Manifestations of Wegener Granulomatosis in Iranian Ethnicities Using the K-Means Algorithm: A Descriptive Study

Abstract Introduction Wegener granulomatosis (WG) appears with clinical symptoms, including recurrent respiratory infection, renal manifestations, and nonspecific systemic symptoms. Objective To study the clinical manifestations of WG in Iranian ethnicities, and data on 164 patients were recorded from 2013 to 2018. Methods The data included demographics, symptoms, and the Birmingham Vasculitis Activity Score (BVAS). The symptoms involved the following sites: the nose, sinus, glottis, ears, lungs, kidneys, eyes, central nervous system, mucous membranes, skin, heart, stomach, intestine, as well as general symptoms. The clinical manifestations of nine ethnicities were analyzed. Results In total, 48% of the patients were male and 51% were female, with a median age of 51 years. The BVAS was of 15.4, the sites most involved were the sinus (n =155), nose (n = 126), lungs (n = 125), and ears (n =107). Gastrointestinal (n = 14) and cardiac (n = 7) involvement were less common. Among the patients, 48.17% were Persian, 13.41% were Azari, 11.17% were Gilaki, 11.17% were Kurd, and 10.9% were Lor. Conclusion Our findings indicated that the sinus, nose, lungs, and ears were the sites most involved, and gastrointestinal and cardiac involvement were less common. In the present study, involvement of the upper and lower respiratory tract was higher than that reported in Western and Asian case series. Moreover, we report for the first time that, in all patients with ear involvement, the left ear was the first to be affected. The clinical manifestations among Iranian ethnicities were not different, and the Gilaki ethnicity had the highest BVAS, mostly because the weather was humid; therefore, in Iran, in areas with humidity, the rate of the disease was higher.

Criterios broncoscópicos, laboratoriales y anatomopatológicos de enfermedad de la vía aérea en pacientes diagnosticados con granulomatosis con poliangitis / Bronchoscopic, laboratory and anatomopathological criteria for airway disease in patients diagnosed with granulomatosis with polyangiitis

Introducción: los pacientes con granulomatosis con poliangitis (GPA) pueden presentar compromiso de la vía aérea superior (VAS) o inferior (VAI). Objetivos: describimos las manifestaciones endoscópicas de las vías respiratorias, los hallazgos histológicos y los anticuerpos anticitoplasma de neutrófilos (ANCA) en un grupo de pacientes con GPA. Métodos: estudio retrospectivo de historias clínicas de pacientes con GPA sometidos a broncoscopia entre 2012 y 2019. Se analizaron hallazgos de la vía aérea, biopsias y ANCA. Resultados: se incluyeron 40 pacientes, con una edad media de 46,92±17,61 años, predominantemente del sexo femenino (67,5%). Se observó afectación de la vía aérea en el 90% (n=36). El C-ANCA fue reactivo en el 63,9%, P-ANCA en el 25%, ANCA doblemente reactivo en el 8,33% y no reactivo en el 20%. Los hallazgos comunes en la vía aérea superior (VS) fueron sinusitis crónica (41,7%), destrucción del tabique nasal (16,7%); y en la vía aérea inferior (AI): estenosis traqueobronquial (38,9%), traqueobronquitis (25%). Los hallazgos más frecuentes de las biopsias broncoscópicas fueron proceso inflamatorio polimorfonuclear (61,9%) y necrosis geográfica (47,6%). Conclusión: la vía aérea está comprometida hasta en un 90% de los pacientes con GPA. ANCA no reactivos no descartan esta posibilidad. La sinusitis crónica y los procesos fibroestenóticos traqueobronquiales fueron los hallazgos endoscópicos más comunes. La vasculitis en biopsias se encontró en una minoría de casos.

Introduction: patients with granulomatosis with polyangiitis (GPA) may present upper airway (UA) and lower airway (LA) involvement. Objectives: we describe the endoscopic manifestations of the airways, histological findings from biopsied tissue and antineutrophilic cytoplasm antibody (ANCA) in a group of patients with GPA. Methods: retrospective study of medical records of patients with GPA undergoing bronchoscopy between 2012 and 2019. Airway findings, results of biopsies performed and ANCA results were analyzed. Results: 40 patients were included, with a mean age of 46.92±17.61 years and predominantly female (67.5%). Airway involvement was observed in 90% (n=36). The C-ANCA was reactive in 63.9%, P-ANCA in 25%, doubly reactive ANCA in 8.33% and non-reactive in 20%. The findings in upper airway (UA) were: chronic sinusitis (41.7%), destruction of the nasal septum (16.7%); and in lower airway (LA) were: tracheobronchial stenosis (38.9%) and tracheobronchitis (25%). The pathological findings most common of bronchoscopic biopsies were: polymorphonuclear inflammatory process (61.9%) and geographic necrosis (47.6%). Conclusion: the airway is involved in up to 90% of patients with GPA. Non-reactive ANCA does not rule out this possibility. Chronic sinusitis and tracheobronchial fibrostenotic processes were the most common endoscopic findings. Vasculitis in biopsies was found in a minority of cases.

Manifestaciones periodontales de la Granulomatosis de Wegener: Reporte de un caso clínico

La granulomatosis de Wegener o granulomatosis con Poliangitis (GPA) es una enfermedad caracterizada por inflamación y necrosis de las paredes de los vasos sanguíneos. Es de etiología desconocida, baja prevalencia y alta agresividad. Esta enfermedad puede comprometer los tejidos bucales causando agrandamiento e inflamación del tejido gingival. Se reporta el caso de un paciente de género masculino que manifiesta aumento de volumen de la encía e inflamación asociado al diagnóstico de granulomatosis de Wegener. La lesión fue eliminada quirúrgicamente y el diagnóstico se logró al combinar los hallazgos serológicos del test ANCA, manifestaciones periodontales y análisis histopatológico. El paciente fue tratado con metotrexato y corticoesteroides y no presenta recidiva de la lesión luego de 2 años de control. En este artículo se analizan las manifestaciones periodontales asociadas a la GPA resaltando la importancia de un adecuado diagnóstico de lesiones periodontales caracterizadas por agradamiento gingival e inflamación.

Wegener's granulomatosis or granulomatosis with polyangiitis (GPA) is a disease characterized by inflammation and necrosis of the blood vessel walls. It is of unknown etiology, low prevalence and high degree of aggressiveness. This disease can compromise the oral tissues, causing enlargement and inflammation of the gingival tissues. The case of a male patient who presented rapidly growing gingival tissue enlargement and inflammatory characteristics associated with the diagnosis of Wegener's granulomatosis is reported. The lesion was removed surgically and the diagnosis was achieved by combining the serological findings of the ANCA test, periodontal manifestations and histopathological analysis of the lesion. The patient was treated with methotrexate and corticosteroids and the lesion did not reappear after 2 years of control. In this article, the periodontal manifestations associated with GPA are analyzed, highlighting the importance of an adequate diagnosis of periodontal lesions characterized by gingival enlargement and inflammation.

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.

Hidronefrosis como manifestación inusual y distractora en el diagnóstico de una vasculitis ANCA positiva. A propósito de un caso / Hydronephrosis as an unusual and distracting manifestation in the diagnosis of ANCA-positive vasculitis. About a case

La nefropatía obstructiva se considera una manifestación inusual en las vasculitis ANCA. Se presenta el caso de un masculino de 38 años, con granulomatosis con poliangitis e hidronefrosis unilateral, y revisión de la literatura. Masculino de 38 años, sano, quien consulta por cuadro subagudo de odinofagia, síntomas constitucionales y lesión renal aguda anúrica. Inicialmente con hallazgo de hidronefrosis izquierda, manejado como nefropatía obstructiva, que eventualmente desarrolla hemorragia alveolar difusa, distrés respiratorio y fallece debido a un síndrome de distrés respiratorio agudo severo refractario asociado a su granulomatosis con poliangitis. La nefropatía obstructiva es una manifestación inusual de las vasculitis ANCA asociadas. Es importante la sospecha diagnóstica en estos cuadros multisistémicos para no dilatar el tratamiento inmunosupresor conjunto con el resto de las terapias requeridas.

Obstructive nephropathy is considered an unusual presentation in ANCA-associated vasculitis. The following case describes a 38-year-old male with granulomatosis with polyangiitis and unilateral hydronephrosis, as well as a literature review. A 38-year-old male with an unremarkable medical background presents with a 3-week history of odynophagia, constitutional symptoms and anuric kidney injury. Initially managed as an obstructive nephropathy due to a left hydronephrosis finding, the patient eventually develops a diffuse alveolar hemorrhage, acute respiratory distress and perishes due to granulomatosis with polyangiitis. Obstructive nephropathy is an unusual manifestation of ANCA-associated vasculitis. Diagnostic suspicion is important in these multisystem pictures so as not to delay immunosuppressive treatment together with the rest of the required therapies.

Linfoma y granulomatosis eosinofílica con poliangeitis (síndrome de Churg-Strauss): reporte de un caso / Lymphoma and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): case report

Diversas etiologías pueden desencadenar a las vasculitis ANCA (anticuerpos anticitoplasma de neutrófilo). Entre ellas se encuentran las neoplasias hematológicas, como los linfomas no Hodgkin, que pueden asociarse con diferentes autoanticuerpos y manifestaciones reumatológicas. Es esencial sospechar estas causas secundarias si la enfermedad tiene un curso crónico con respuesta tórpida al tratamiento. En el presente artículo se reporta un caso inusual de asociación entre granulomatosis eosinofílica con poliangeitis y linfoma no Hodgkin de bajo grado de agresividad.

Diverse etiologies can trigger ANCA (antineutrophil cytoplasmic antibodies) vasculitis. These include hematological neoplasms, such as non-Hodgkin lymphomas, which can be associated with different autoantibodies and rheumatological manifestations. These secondary causes are essential to suspect if the disease has a chronic course with a poor response to treatment. In this article, we report an unusual association between eosinophilic granulomatosis with polyangiitis and low-grade non-Hodgkin lymphoma.

A case of anti-neutrophil cytoplasmic antibodies-associated vasculitis with ocular masses as the main manifestation / 中华肾脏病杂志

A case of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) with ocular masses as the main manifestation was reported. The patient was a middle-aged female, the initial symptom was eye swelling, pulmonary nodules were found before eye surgery, and further examination revealed proteinuria, hematuria and renal insufficiency. Renal pathology showed ANCA-associated glomerulonephritis. The final diagnosis was eye, kidney and lung lesions caused by AAV. Treatment with glucocorticoids and cyclophosphamide resulted in improvement in eye, kidney, and pulmonary lesions. Atypical clinical manifestations of AAV may lead to delayed diagnosis, and attention should be paid to the exclusion of AAV for ocular masses of unknown cause.

Hipoacusia como primera manifestación de granulomatosis con poliangeítis

Introducción: Las vasculitis asociadas a anticuerpos anti citoplasma de neutrófilos (ANCA) son entidades clínicas poco frecuentes con manifestaciones amplias que se asocian a retos diagnósticos. El tratamiento oportuno se asocia a mejoría pronóstica significativa. Caso clínico: Femenina de 45 años que acude con síntomas constitucionales de 2 meses de evolución, asociado en las últimas 2 semanas a hipoacusia progresiva. Se evidencia lesión renal aguda asociada a datos de sedimento urinario activo. El perfil inmune solicitado resultó positivo para ANCA PR-3, y la biopsia renal confirma el diagnóstico de Granulomatosis con Poliangeitis. Se inicia tratamiento inmunosupresor con ciclofosfamida, con poca mejoría de la función renal y requerimiento de hemodiálisis crónica.  La amplia variedad de manifestaciones clínicas asociadas a las vasculitis ANCA conducen a la incapacidad de diagnosticarlas tempranamente y la hipoacusia, aunque descrita, es una manifestación inicial poco frecuente. Conclusión: Las VAA son entidades clínicas poco frecuentes y cuando se presentan con manifestaciones atípicas, suelen ser un reto diagnóstico. Resulta imperativo considerarla en casos de síntomas constitucionales, asociados a manifestaciones renales, otorrinolaringológicas y pulmonares. (provisto por Infomedic International)

Introduction: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare clinical entities with broad manifestations that are associated with diagnostic challenges. Prompt treatment is associated with significant prognostic improvement. Case report: 45-year-old female presenting with constitutional symptoms of 2 months of evolution, associated in the last 2 weeks with progressive hypoacusis. Acute renal lesion associated with active urinary sediment data was evidenced. The requested immune profile was positive for ANCA PR-3, and the renal biopsy confirmed the diagnosis of Granulomatosis with Polyangiitis. Immunosuppressive treatment with cyclophosphamide was started, with little improvement in renal function and requiring chronic hemodialysis. The wide variety of clinical manifestations associated with ANCA vasculitis lead to the inability to diagnose them early and hypoacusis, although described, is a rare initial manifestation. Conclusion: AAV are rare clinical entities and when they present with atypical manifestations, they are often a diagnostic challenge. It is imperative to consider it in cases of constitutional symptoms, associated with renal, otorhinolaryngologic and pulmonary manifestations. (provided by Infomedic International)

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.

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.

Fístula orbitonasal, un desafío terapéutico frente a una manifestación infrecuente en la granulomatosis con poliangitis / Orbito-nasal fistula, a therapeutic challenge against an infrequent manifestation in granulomatosis with polyangiitis

La granulomatosis con poliangitis (GPA) es una condición clínico-patológica que cursa con inflamación granulomatosa del tracto respiratorio superior e inferior, glomerulonefritis necrotizante focal y vasculitis necrotizante de vasos de pequeño y mediano tamaño (capilares, vénulas, arteriolas y arterias). La afectación de la GPA orbitaria oscila entre el 7 y el 45%. En ocasiones, la glándula lagrimal puede estar afectada de forma aislada y ser la manifestación inicial de la enfermedad. Presentamos el caso de una paciente con GPA y fístula nasolagrimal.

Granulomatosis with polyangiitis (GPA) is a clinical-pathological condition that presents with granulomatous inflammation of the upper and lower respiratory tract, focal necrotizing glomerulonephritis and necrotizing vasculitis of small and medium-sized vessels (capillaries, venules, arterioles and arteries). Orbital GPA involvement ranging from 7-45%. The lacrimal gland can occasionally be affected in isolation and be the initial manifestation of the disease. We present the case of a patient with GPA and nasolacrimal fistula.

Compromiso pulmonar en vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos / Pulmonary manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis

This article reviews the pulmonary manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Its frequency in the different phenotypes of the disease, clinical manifestations and updated therapeutic recommendations are reviewed, aiming to alert the medical community about the existence of these diseases. We pretend to stimulate a timely suspicion, diagnostic precision, and the implementation of effective therapies, to reduce the eventual sequelae derived from a diagnostic omission or an inappropriate treatment for the different clinical scenarios in which these diseases appear.

A Case of Eosinophilic Granulomatosis with Polyangiitis Mimicking Cutaneous Tuberculosis and Tuberculous Lymphadenitis

Summary@#Eosinophilic granulomatosis with polyangiitis (EGPA), or Churg-Strauss Syndrome (CSS) is a rare granulomatous necrotizing vasculitic disease characterized by the presence of asthma, sinusitis, and hypereosinophilia. We describe a patient who was initially diagnosed with tuberculous lymphadenitis and later diagnosed with EGPA.

Facial Palsy with Tongue Ulcer: A Rare Initial Presentation of Granulomatosis with Polyangiitis

ABSTRACT@#Granulomatosis with polyangiitis (GPA) is a rare multisystem disease. Although GPA is rare, it commonly presents in a localised stage where its manifestation involves the upper or lower respiratory tract before progressing to a generalised stage. Therefore, most patients with GPA will visit an oral surgeon or an otolaryngologist to seek treatment. However, the diagnosis of GPA is often delayed as GPA is not frequently considered as a differential diagnosis in common oral and facial diseases. The lack of gold standard investigation for the diagnosis of GPA makes management of this case, a diagnostic conundrum. We herein report a patient who was diagnosed with bilateral acute otitis media and left mastoiditis complicated with facial nerve palsy, and later developed tongue ulceration one month after his initial presentation. The ear, facial and oral symptoms represent a diagnostic red herring to a full-blown generalised stage of GPA.

A Case of Coronary Artery Bypass Grafting for a Patient with Eosinophilic Granulomatosis and Polyangiitis / 日本心臓血管外科学会雑誌

We present herein the case of a 45-year-old man with a coronary artery aneurysm (diameter 19 mm) in the proximal part of the left anterior descending branch associated with eosinophilic granulomatosis with polyangiitis (EGPA). As coronary angiography showed #6 : 100% and #12-2 : 90%, and Tc-99 m myocardial scintigraphy showed exertional ischemia in the anterior septum, revascularization was considered to be indicated. Prednisolone and mepolizumab were administered preoperatively to suppress the activity of vasculitis due to eosinophilia, and surgery was performed when the eosinophil count normalized. The patient underwent off-pump coronary artery bypass grafting (LITA-LAD, SVG-OM2). The patient was discharged, and the postoperative course was uneventful. In coronary artery bypass grafting for EGPA, eosinophils may infiltrate the internal thoracic artery and result in vasculitis, which may affect the patency rate. Perioperative management of vasculitis may thus be important.

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)

Mepolizumab as a steroid-sparing treatment option in a patient with eosinophilic granulomatosis with polyangiitis / Mepolizumabe como terapêutica poupadora de esteroides num paciente com granulomatose eosinofílica com poliangiite

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disorder characterized by asthma, peripheral blood eosinophilia, and signs of vasculitis. Glucocorticoids are considered the cornerstone of treatment, but most patients remain steroid-dependent and carry a significant burden of adverse effects. We report a case of a patient with steroid-dependent EGPA successfully treated with mepolizumab. A 36-year-old man presented with persistent rhinitis, dyspnea, wheezing, and dry cough poorly controlled with inhaled therapy. Eosinophilia in peripheral blood and bronchoalveolar lavage fluid was seen. Histological findings from nasal mucosa revealed eosinophilic microabscesses and vasculitis without granulomas compatible with EGPA diagnosis. After daily oral prednisolone (PSL) was started, symptoms and eosinophilia improved, but adverse effects emerged. Attempts at tapering off PSL resulted in worsening of symptoms. He started mepolizumab 300 mg monthly, with clinical improvement and sustained disease remission, which allowed reducing the need for PSL. We present a very disabling steroiddependent EGPA. Mepolizumab was able to taper off PSL while maintaining symptomatic control.

Granulomatose eosinofílica com poliangiite (EGPA) é uma doença multissistêmica caracterizada por asma, eosinofilia no sangue periférico e sinais de vasculite. Os corticoides são considerados a base do tratamento, no entanto, a maioria dos pacientes permanece dependente deste tratamento com os seus efeitos adversos associados. Relatamos o caso de um paciente com granulomatose eosinofílica dependente de esteroides com poliangiite (EGPA) tratado com sucesso com mepolizumabe. Um homem de 36 anos apresentou rinite persistente, dispneia, sibilos e tosse seca mal controlada com terapia inalada. Observou-se eosinofilia no sangue periférico e no lavado broncoalveolar. Os achados histológicos da mucosa nasal revelaram microabscessos eosinofílicos e vasculite sem granulomas compatíveis com o diagnóstico de EGPA. Após o início da prednisolona oral diária (PSL), os sintomas e a eosinofilia melhoraram, mas surgiram efeitos adversos. As tentativas de redução gradual da PSL resultaram no agravamento dos sintomas. Iniciou mepolizumabe 300 mg mensalmente, com melhora clínica e remissão sustentada da doença, o que permitiu reduzir a necessidade de PSL. Apresentamos um EGPA dependente de esteroides muito incapacitante. O mepolizumab foi capaz de diminuir o PSL mantendo o controle sintomático sustentado.

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.