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.

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.

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.

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.

Analysis of clinical features of 147 cases of ANCA-associated vasculitis / 中华风湿病学杂志

Objective:To analyze the clinical characteristics of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).Methods:A retrospective study was conducted on AAV patients with long-term follow-up in the rheumatology outpatient clinic of Peking Union Medical College Hospital between February 2015 and February 2022. The demographic characteristics, clinical manifestations, concurrent events, treatment, and prognosis of the three clinical subtypes of AAV were collected and analyzed.Results:There were 71, 45, and 31 cases of granulomatous polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatous polyangiitis (EGPA), respectively, among 147 patients. The ANCA positivity rates in the three groups were 91.5%, 95.6%, and 19.4% ( χ2=76.68, P<0.01), respectively. The upper respiratory tract and lungs were the most frequently affected organs in GPA and EGPA, and the kidneys and lower respiratory tract were the main organs involved in MPA. In addition, cardiac and neurological involvement and thrombosis rates were significantly higher in EGPA patients than in GPA and MPA (12.9%, 9.7%, 41.9% and 19.4%, respectively; χ2=8.51、7.13、7.54、0.02, P<0.05) .The median follow-up time for the three groups of patients was 43, 28, and 46 months respectively.Relapse was more common in patients with GPA and EGPA (up to 59.2% and 64.5%; χ2=11.26, P=0.004), with the lungs and ENT being the most common relapse organs (GPA of 61.9% and 40.5%, EGPA of 55.0% and 50.0%), the lungs and kidneys were the most common manifestations in MPA relapse (64.3% and 60.0%, respectively). The main therapeutic agents were glucocortoid (95.9%), cyclophosphamide (71.4%), methotrexate (54.4%), tripterygium wilfordiz (34.0%),mycophenolate mofetil (31.3%), azathioprine (29.3%), leflunomide (19.0%), rituximab (19.0%), and tacrolimus/cyclosporine (8.8%). There were 6 deaths (4.1%) occurred during the follow-up period of this study. Conclusion:The clinical features of AAV are similar to those reported in the literature and relapses are common>he vast majority of patients need to be treated with glucocorticoid combined with immunosuppressive agents.

The central nervous system involvement of patients with microscopic polyangiitis / 中华风湿病学杂志

Objective:To explore the clinical features and prognosis of central nervous system involvement in patients with microscopic polyangiitis (MPA).Methods:We retrospectively investigated the clinical data of 138 MPA patients hospitalized with MPA in Tianjin Medical University General Hospital from January 1, 2010 to November 1, 2019. Patients were divided into two groups according to whether they had the central nervous system (CNS) involvement or not and then Kaplan-Meier survival curve was used to analyze the survival rate between the two groups, Logistic regression model analysis was adopted to analyze risk factors, and P<0.05 was considered statistically significant. Results:①29 patients (21.0%)among the 138 MPA had CNS-affected, including 13(44.8%) males and 16(55.2%) females. CNS involvement was present at the diagnosis of MPA in 20 cases (69.0%) and after the diagnosis of MPA in 9 cases (31.0%). ②The clinical manifestations were motor impairment in 14 cases (48.3%), sensory impairment in 10 cases (34.5%), speech loss in 9 cases (31.0%), headache in 8 cases (27.6%), consciousness disorder in 7 cases (24.1%), dysphagia and bucking in 4 cases (13.8%), cranial nerves involvement in 3 cases (10.3%). The imaging manifestations of the head included infarction, hemorrhage, infarction with hemorrhage and linear dural thickening. Five patients received lumbar puncture. One patient showed elevation of cerebrospinal fluid pressure, 1 patient had elevated protein and 5 patients showed elevation of LDH.③Eighteen patients received glucocortoid combined with cyclophosphamide. CNS symptoms recurred in 6 patients, four patients had recurrent cerebral infarction. ④Median survival time was 55 months in the CNS affected group [95% CI=(14.215, 95.785)] and 86 months in the N-CNS group [95% CI=(24.378, 147.622)]. Kaplan-Meier survival curve showed that there was no significant difference in survival rate between the two groups ( χ2=0.07, P=0.794) . Conclusion:The central nervous system involvement of microscopic polyangiitis is not uncommon. The clinical manifestations are various, with motor impairment the most. The most common imaging manifestation is cerebral infarction and the patients mainly presenteas multiple cerebral infarction. However, the CNS involvement of microscopic polyangiitis is not associated with mortality.

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.

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.

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.

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.

Peripheral ulcerative keratitis as presenting manifestation of systemic microscopic polyangiitis: a case report / Ceratite ulcerativa periférica como manifestação de poliangiíte microscópica sistêmica: relato de caso

ABSTRACT Microscopic polyangiitis is a rare autoimmune disease of unknown etiology, characterized by inflammation and necrosis of blood vessels. It forms a part of the antineutrophil cytoplasmic antibody-associated vasculitides-a heterogeneous group of disorders characterized by vasculitis. It is a systemic disease affecting multiple organs. The patients may present with a wide variety of symptoms. Ocular manifestations may present as its initial clinical symptoms, necessitating a multidisciplinary approach for reducing the morbidity and mortality. Early diagnosis aids in the formulation of appropriate treatment and prevention of further complications. Aggressive treatment, including surgery, is often necessary to limit structural damage and preserve visual function. We present the case of an 82-year-old woman who initially presented with peripheral ulcerative keratitis that led to the diagnosis of microscopic polyangiitis.

RESUMO A poliangeíte microscópica é uma doença autoimune rara de etiologia desconhecida, caracterizada por inflamação e necrose dos vasos sanguíneos. Faz parte das vasculites associadas a anticorpos citoplasmáticos antineutrófilos - um grupo heterogêneo de doenças caracterizadas por vasculite. É uma doença sistêmica que afeta vários órgãos. Os pacientes podem apresentar uma grande variedade de sintomas. As manifestações oculares podem apresentar-se como seus sintomas clínicos iniciais, necessitando de abordagem multidisciplinar para redução da morbimortalidade. O diagnóstico precoce ajuda na formulação do tratamento adequado e na prevenção de complicações futuras. O tratamento agressivo, incluindo cirurgia, muitas vezes é necessário para limitar o dano estrutural e preservar a função visual. Apresentamos o caso de uma mulher de 82 anos que inicialmente apresentou ceratite ulcerativa periférica que levou ao diagnóstico de poliangite microscópica.

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.

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 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.

Efficacy of rituximab in maintenance therapy for antineutrophil cytoplasmic antibody-associated vas-culitis / 中华风湿病学杂志

Objective:To evaluate the efficacy and safety of rituximab(RTX) as remission-mainten-ance therapy in antineutrophil cytoplasmic antibody(ANCA) associated vasculitis(AAV).Methods:Patients with AAV, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), treated with rituximab (RTX) in Peking Union Medical College Hospital during September 2005 to June 2021 were included into this study. Clinical data, relapse rate, time of first relapse and adverse events were collected and analyzed. The cumulative relapse rate was calculated by Kaplan-Meier, t test, and Man-Whithey U test and chi-square were used to compare differences between two groups. Results:① Thirty-nine AAV patients were enrolled, including 36 GPA and 3 MPA. During the 20(3, 104) months follow-up, 59.0%(23/39) patients had suffered relapses. The time for first relapse was 11(3, 42) months after remission. ② There were no difference in the relapse rate [60.0%(18/30) vs 55.6%(5/9), χ2=0.06, P=1.000), the time of first relapse [15(3, 42) vs 10(9, 30), Z=0.45, P=0.678], CD19 + B [23.5 (5, 148) cell/μl vs 3(2, 15) cell/μl, Z=0.57, P=0.605] and serum IgG [7.09(5.13, 13.90) g/L vs 9.72(5.32, 12.0) g/L, Z=0.36, P=0.770] between standard dose and low-dose groups. The rate of major relapse-free was significantly less in patients treated with standard dose than patients with reduced dose of RTX {87.1%[95% CI(73.4%, 100.8R%)] vs 64.3%[95% CI(23.1%, 105.4%)], χ2=7.59, P=0.006}. ③ There were no difference in relapse rate [50.0%(3/6) vs 60.6%(20/33), χ2=0.24, P=0.674], time of first relapse [23(6, 25) vs 11(3, 42), Z=0.05, P=0.982], CD19 + B[35(15, 50) cell/μl vs 10(0, 148) cell/μl, Z=0.95, P=0.382] and serum IgG[6.70(5.91, 7.49) g/L vs 7.69(3.78, 13.90) g/L, Z=0.48, P=0.700] between the fixed interval dosage and the on-demand dosage groups. There was no difference in the rate of major relapse-free between the two groups (100% vs 77.8%, χ2=1.79, P=0.181). ④ The incidence of infusion reaction was 5.1%(2/39) and infection was 20.5%(8/39). Serum IgG level was 4.37(3.78, 13.4) g/L at infection. There was no difference in safety between the standard and low-dose groups or between fixed interval and on-demand dosage groups ( P>0.05). Conclusion:There is no significant difference in relapse rate bet-ween the standard RTX dose and low-dose RTX induction therapy group, but the major relapse rate is sign-ificantly reduced in the standard dose RTX therapy. The relapse rate of fixed intervals dosage group is similar to that of on-demand dosage group. The safety profile of the standard dose and low-dose induction therapy groups or fixed intervals and on-demand dosage groups is similiar.