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1.
Rev. méd. (La Paz) ; 27(2): 49-51, Jul. - Dic. 2021. Ilus
Artigo em Espanhol | LILACS | ID: biblio-1361289

RESUMO

Las Vasculitis Primarias (VP), a lo largo de su evolución pueden presentar diversas manifestaciones oculares. La afectación de la esclera, conocida como escleritis es un cuadro infrecuente. Presentamos el caso de una paciente con antecedente de Granulomatosis con Poliangeitis (GPA) que consulta por ojo rojo, aumento de volumen e intenso dolor en ojo derecho, siendo diagnosticada de escleritis anterior, cuya evolución fue favorable gracias al diagnóstico y tratamiento precoces


Assuntos
Granulomatose com Poliangiite
2.
Rev. cir. traumatol. buco-maxilo-fac ; 21(4): 20-23, out.-dez. 2021. ilus, tab
Artigo em Português | LILACS, BBO - Odontologia | ID: biblio-1391249

RESUMO

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)


Assuntos
Humanos , Masculino , Adulto , Doenças Autoimunes , Granulomatose com Poliangiite , Prótese Maxilofacial , Palato , Fonação , Vasos Sanguíneos , Mortalidade , Corticosteroides , Deglutição , Imunossupressores
3.
Arq. Asma, Alerg. Imunol ; 5(3): 298-301, jul.set.2021. ilus
Artigo em Inglês | LILACS | ID: biblio-1399398

RESUMO

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.


Assuntos
Humanos , Masculino , Adulto , Prednisolona , Granulomatose com Poliangiite , Corticosteroides , Anticorpos Monoclonais Humanizados , Sinais e Sintomas , Terapêutica , Sons Respiratórios , Rinite , Interleucina-5 , Tosse , Diagnóstico , Dispneia , Eosinofilia
4.
Medicina (B.Aires) ; 81(2): 198-207, June 2021. graf
Artigo em Inglês | LILACS | ID: biblio-1287271

RESUMO

Abstract ANCA-associated vasculitis is a heterogeneous group of rare autoimmune conditions of unknown cause. Clinical characteristics and prognostic factors were analyzed in 47 patients: 20 (42.5%) with granulomatosis with polyangiitis, 17 (36.2%) with microscopic polyangiitis, 6 (12.8%) with renal-limited vasculitis, and 4 (8.5%) with eosinophilic granulomatosis with polyangiitis. Mean age at diagnosis was 53.5 ± 16.5 years and the median of BVAS (Birmingham Vasculitis Activity Score) was 14 (4-42). The most frequent clinical manifesta tions were: general in 44 (93.6%), renal in 30 (63.8%) and respiratory in 28 (59.6%). All received corticosteroids at the beginning of treatment. Intravenous cyclophosphamide was associated in 20 (42.5%) and oral route in 14 (29.8%); azathioprine in 3 (6.4%) and rituximab in 2 (4.2%). At a median follow-up of 35.5 months (range 0.14- 234), 21 relapses were recorded in 14 patients. Overall mortality was 3.5 deaths per 100 patient-year in the whole group. Those over 55 years old, the presence of alveolar hemorrhage, those with FFS (Five Factor Score) of 2, and patients with MPA had poor prognosis. Renal involvement, ANCA pattern and BVAS were not associated to a poorer prognosis.


Resumen Las vasculitis asociadas a ANCA son un grupo heterogéneo de entidades autoinmunes, poco frecuentes, de etiología desconocida. Analizamos las características clínicas y factores pronóstico en 47 pacientes: 20 (42.5%) granulomatosis con poliangeítis, 17 (36.2%) poliangeítis microscópica, 6 (12.8%) vasculitis limitada al riñón y 4 (8.5%) granulomatosis eosinofílica con poliangeítis. La edad promedio al diagnóstico fue 53.5 ± 16.5 años y la mediana de BVAS (Birmingham Vasculitis Activity Score) 14 (4-42). Las manifestaciones clínicas más frecuentes fueron: generales en 44 (93.6%), renales 30 (63.8%) y respiratorias en 28 (59.6%). Todos recibieron corticoides al inicio del tratamiento. Se asoció ciclofosfamida endovenosa en 20 (42.5%) y oral en 14 (29.8%); azatioprina en 3 (6.4%) y rituximab en 2 (4.2%). En una mediana de seguimiento de 35.5 meses (rango 0.14-234), se registraron 21 recaídas en 14 pacientes. La mortalidad fue 3.5 por cien pacientes-año en todo el grupo. Los mayores de 55 años, con presencia de hemorragia alveolar, FFS (Five Factor Score) de 2, y los casos con poliangeítis microscópica tuvieron peor pronóstico. El compromiso renal, el patrón de ANCA y el BVAS no se asociaron a peor pronóstico.


Assuntos
Humanos , Pessoa de Meia-Idade , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/tratamento farmacológico , Síndrome de Churg-Strauss/epidemiologia , Granulomatose com Poliangiite , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Prognóstico , Anticorpos Anticitoplasma de Neutrófilos , Poliangiite Microscópica
5.
Rev. argent. reumatolg. (En línea) ; 32(1): 28-30, mar. 2021. ilus, tab
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1279756

RESUMO

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.


Assuntos
Granulomatose com Poliangiite , Insuficiência Respiratória , Terapêutica , Espectroscopia de Ressonância Magnética
6.
Autops. Case Rep ; 11: e2021294, 2021. graf
Artigo em Inglês | LILACS | ID: biblio-1285393

RESUMO

Necrotizing sarcoid granulomatosis (NSG) is a rare and under-recognized cause of granulomatous disease, described as a variant of typical nodular sarcoidosis. It can be asymptomatic when the patient has a single pulmonary nodule or may be accompanied by cough, fever, and dyspnea, or even symptoms due to the involvement of other organs such as the eyes, liver, and central nervous system. The histopathological analysis is essential for the differential diagnosis of other infectious and non-infectious causes of granuloma and to determine the appropriate treatment. NSG is characterized by the presence of a granuloma with extensive coagulative necrosis associated with the occurrence of vasculitis. We present the case of a patient diagnosed with NSG who had an unusual outcome with recurrent pulmonary thromboembolisms followed by hemodynamic instability and death.


Assuntos
Humanos , Feminino , Adulto , Sarcoidose Pulmonar/patologia , Granuloma , Granulomatose com Poliangiite/patologia , Diagnóstico Diferencial
7.
Artigo em Português | LILACS, CONASS, Coleciona SUS, SES-GO | ID: biblio-1355052

RESUMO

Objetivo: descrever as evidências disponíveis na literatura científica sobre eficácia e segurança do rituximabe comparado a diferentes tratamentos. Materiais e métodos: é uma revisão rápida de evidências científicas para tomada de decisão informada por evidências em políticas e práticas de saúde. Conclusão: o Rituximabe tem eficácia e segurança similares à da Ciclofosfamida, para terapia de indução de remissão e para manutenção da remissão e, para pacientes com doença recidivante, o Rituximabe é mais eficaz que a Ciclofosfamida para manter a remissão. Para terapia de manutenção, Rituximabe é mais eficaz que Azatioprina, com perfil de segurança similar. Diferentes regimes de dosagem do Rituximabe tem eficácia e segurança similar para terapia de manutenção. O Infliximabe parece ser superior ao Rituximabe nos desfechos de eficácia (indução e manutenção da remissão).


Objective: to describe the evidence available in the scientific literature on the efficacy and safety of rituximab compared to different treatments. Materials and Methods: is a rapid review of scientific evidence for evidence-informed decision making in health policy and practice. Conclusion: Rituximab has similar efficacy and safety to Cyclophosphamide, for remission induction therapy and for maintenance of remission, and for patients with relapsing disease, Rituximab is more effective than Cyclophosphamide in maintaining remission. For maintenance therapy, Rituximab is more effective than Azathioprine, with a similar safety profile. Different dosing regimens of Rituximab have similar efficacy and safety for maintenance therapy. Infliximab appears to be superior to Rituximab in efficacy outcomes (induction and maintenance of remission).


Assuntos
Humanos , Granulomatose com Poliangiite/tratamento farmacológico , Vasculite Sistêmica/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Poliangiite Microscópica/tratamento farmacológico , Rituximab/efeitos dos fármacos , Azatioprina , Ciclofosfamida , Infliximab , Glucocorticoides
8.
Rev. colomb. reumatol ; 27(4): 291-297, oct.-dic. 2020. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1289333

RESUMO

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.


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Granulomatose com Poliangiite , Meningite , Sistema Nervoso Central , Diagnóstico , Cefaleia
9.
Rev. argent. reumatolg. (En línea) ; 31(2): 37-40, jun. 2020. ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1143930

RESUMO

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.


Assuntos
Humanos , Granulomatose com Poliangiite , Reumatologia , Vasculite , Linfoma
10.
Rev. colomb. reumatol ; 27(2): 123-129, ene.-jun. 2020. tab, graf
Artigo em Espanhol | LILACS | ID: biblio-1251646

RESUMO

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.


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Granulomatose com Poliangiite , Exoftalmia , Otolaringologia , Sistema Respiratório , Vasculite , Olho
11.
Rev. chil. neuro-psiquiatr ; 58(2): 191-197, jun. 2020. ilus
Artigo em Espanhol | LILACS | ID: biblio-1115484

RESUMO

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.


Assuntos
Humanos , Feminino , Adulto , Sistema Nervoso Central , Granulomatose com Poliangiite , Glomerulonefrite , Neurocirurgia
12.
Rev. méd. Chile ; 148(6): 755-761, jun. 2020. tab, graf
Artigo em Espanhol | LILACS | ID: biblio-1139368

RESUMO

Background: Cumulative survival in patients with anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (VAA) is 88 and 78% at 1 and 5 years, respectively. Despite this, mortality continues to be 2.7 times higher than the general population. Differences in the clinical profile of VAA in different ethnicities have been observed. Aim: To identify factors at the time of diagnosis, associated with mortality at one year of follow-up and to describe the clinical characteristics of these patients. Material and Methods: We identified in local databases and reviewed clinical records of patients with VAA with at least one year of follow up in a clinical hospital. Demographic and laboratory parameters and clinical activity scores were analyzed. Results: Of 103 patients with VAA identified, 65 met the inclusion criteria and were analyzed. Their age ranged from 45 to 63 years and 56% were women. Thirty-five patients (54%) were diagnosed as granulomatosis with Polyangiitis (GPA) and 30 patients (46%) with Microscopic Polyangiitis (MPA). The frequency of renal disease was 53% and pulmonary involvement occurred in 72%. At one year of follow-up 11 patients died resulting in a mortality of 17%. Seven patients died within three months after diagnosis. MPO ANCA were more common than PR3 ANCA. In the multivariate analysis, the presence of ophthalmological involvement, lung kidney syndrome and a Five Factor Score (FFS) of 1 or more were independent factors associated with mortality at one year. Conclusions: In these patients, pulmonary manifestations predominate. Lung kidney syndrome, ophthalmological involvement and a FFS score ≥ 1 were associated with mortality.


Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Granulomatose com Poliangiite , Estudos Retrospectivos , Peroxidase , Anticorpos Anticitoplasma de Neutrófilos , Mieloblastina
13.
Adv Rheumatol ; 60: 04, 2020. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1088643

RESUMO

Abstract Background: Granulomatosis with polyangiitis (GPA) is a granulomatous necrotizing vasculitis with high morbidity and mortality. Anti-neutrophil cytoplasmic antibody is a valuable diagnostic marker, however its titer lacks predictive value for the severity of organ involvement. Platelet to lymphocyte ratio (PLR) and mean platelet volume (MPV) has been regarded as a potential marker in assessing systemic inflammation. We aimed to explore the value of PLR and MPV in the assessment of disease activity and manifestations of disease in GPA. Methods: 56 newly diagnosed GPA patients and 53 age-sex matched healthy controls were included in this retrospective and cross-sectional study with comparative group. Complete blood count was performed with Backman Coulter automatic analyzer, erythrocyte sedimentation rate (ESR) with Westergen method and C-reactive protein (CRP) levels with nephelometry. The PLR was calculated as the ratio of platelet and lymphocyte counts. Result: Compared to control group, ESR, CRP and PLR were significantly higher and MPV significantly lower in GPA patients. In patients group, PLR was positively correlated with ESR and CRP (r = 0.39, p = 0.005 and r = 0.51, p < 0.001, respectively). MPV was negatively correlated with ESR and CRP (r = - 0.31, p = 0.028 and r = - 0.34 p = 0.014, respectively). Patients with renal involvement had significantly higher PLR than patients without renal involvement (median:265.98, IQR:208.79 vs median:180.34 IQR:129.37, p = 0.02). PLR was negatively correlated with glomerular filtration rate (r = - 0.27, p = 0.009). A cut-off level of 204 for PLR had 65.6% sensitivity and 62.5 specificity to predict renal involvement. Conclusion: PLR exhibit favorable diagnostic performance in predicting renal involvement in patients with GPA.(AU)


Assuntos
Humanos , Linfócitos/química , Granulomatose com Poliangiite/fisiopatologia , Volume Plaquetário Médio/métodos , Contagem de Plaquetas/métodos , Estudos Transversais , Estudos Retrospectivos , Contagem de Linfócitos/métodos
14.
Rev. colomb. radiol ; 31(3): 5389-5385, sept. 2020. ilus, graf
Artigo em Inglês, Espanhol | LILACS | ID: biblio-1343649

RESUMO

Las vasculitis son un grupo de enfermedades que consisten en la inflamación dentro y alrededor de la pared de los vasos sanguíneos, lo que produce necrosis, y su extensión depende del tamaño y ubicación del vaso afectado. Se pueden manifestar en cualquier órgano, por lo que se consideran enfermedades sistémicas. Las vasculitis primarias son patologías poco comunes y difíciles de diagnosticar, porque sus signos y síntomas son inespecíficos y pueden confundirse con muchas otras patologías ­como enfermedades infecciosas, neoplásicas o del tejido conjuntivo­. Por esta razón, para su diagnóstico es importante combinar los hallazgos clínicos, radiológicos, de estudios histopatológicos y de laboratorio. Esta revisión se enfocará en el papel de los hallazgos imagenológicos en el diagnóstico y monitoreo de las manifestaciones pulmonares y abdominales de las vasculitis más frecuentes.


Vasculitis are a group of diseases that are characterized by the presence of inflammation in and around the wall of the blood vessels. This produces necrosis and inflammation where the extension of the injury depends on the size and location of the affected vessel. They can have manifestations in any organ, so they are considered systemic diseases. Primary vasculitis are uncommon pathologies and difficult to diagnose because its signs and symptoms are nonspecific and are easily confused with many other disease processes such as infectious, neoplastic or connective tissue. Because of this, it is important to combine clinical, radiological, histopathological and laboratory findings to achieve a correct diagnosis. In this review, we will focus on the role of imaging findings on the diagnosis and monitoring for pulmonary and abdominal manifestations of the most frequent vasculitis.


Assuntos
Vasculite , Poliarterite Nodosa , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Síndrome de Behçet , Arterite de Takayasu , Poliangiite Microscópica
15.
Rev Assoc Med Bras (1992) ; 66(7): 904-907, 2020. tab, graf
Artigo em Inglês | Sec. Est. Saúde SP, LILACS | ID: biblio-1136302

RESUMO

SUMMARY Churg-Strauss syndrome, Eosinophilic granulomatosis with polyangiitis (EGPA), is a systemic vasculitis that affects small- to medium-sized vessels. It is rare and part of the Anti-neutrophil cytoplasm antibody-associated vasculitis (ANCA) group. We present a 37-year-old man, with a previous history of asthma, that was sent to the ED due to 2 weeks of productive cough, occasional dyspnea on exertion, fever (one week), asthenia, and anorexia. Upon physical examination, he was subfebrile and tachycardic. He had leukocytosis (17.00 x10^9/L) and eosinophilia of 20.0 % (3.4 X10^9/L), creatinine level of 1.5 mg/dL, subtle elevation on liver function tests and CRP of 10.82mg/dL. On Chest X-Ray, there was infiltrate on the right pulmonary base. Due to a strong suspicion of EGPA, he was started on 80mg of prednisolone from admission. ANCA MPO was positive, with the remaining auto-immune study negative. He underwent Thorax CT (under corticotherapy) without relevant changes, as well as bronchoalveolar lavage, without macroscopic signs of alveolar hemorrhage. Because of active urinary sediment, nephrotic proteinuria (6.5g/24h), and acute renal failure he underwent a renal biopsy, which revealed pauci-immune crescentic glomerulonephritis, with predominantly acute findings (in the context of ANCA-MPO Vasculitis - EGPA). After the biopsy, he received three 1g methylprednisolone pulses and was started on Cyclophosphamide. He remained asymptomatic and renal function was restored. This case highlights the importance of integrating all findings in one clinical scenario to prevent a more complex disease diagnosis, with a specific treatment, from being missed.


RESUMO A vasculite de Churg-Strauss, granulomatose eosinofílica com poliangeíte (EGPA), é uma vasculite sistêmica que afeta vasos de pequeno e médio calibre. É rara e pertence ao grupo de vasculites associadas a anticorpos anticitoplasma de neutrófilos (Anca). Apresenta-se um homem de 37 anos, com antecedentes de asma, que recorre ao SU por tosse produtiva com dois meses de evolução, dispneia ocasional em esforço, febre (uma semana de evolução), astenia e anorexia. Ao exame objetivo apresentava-se subfebril e taquicárdico. Analiticamente com leucocitose (17,00 x10^9/L) e eosinofilia de 20,0% (3,4 X10^9/L), creatinina de 1,5 mg/dL, discreta alteração das provas de função hepática e PCR de 10,82 mg/dL. Na radiografia de tórax objetivava-se infiltrado na base pulmonar direita. Por elevada suspeita de EGPA, iniciou prednisolona 80 mg desde a admissão. Anca MPO+, com restante estudo de autoimunidade negativo. Realizou TC tórax (sob corticoterapia) sem alterações de relevo, bem como lavado bronco-alveolar, sem sinais macroscópicos de hemorragia alveolar. Por sedimento urinário ativo, proteinúria na faixa nefrótica (6,56 g/24h) e lesão renal aguda, realizou biópsia renal que revelou glomerulonefrite crescêntica pauci-imune, com achados predominantemente agudos (no contexto de Vasculite Anca-MPO - EGPA). Após biópsia, realizou três pulsos de 1 g de metilprednisolona e iniciou ainda ciclofosfamida. Ficou assintomático e com recuperação da função renal. Este caso realça a importância de integração de todos os achados num só cenário a fim de evitar que escape o diagnóstico de uma doença mais complexa e com um tratamento específico.


Assuntos
Humanos , Masculino , Adulto , Asma , Síndrome de Churg-Strauss/diagnóstico , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Eosinofilia , Metilprednisolona/uso terapêutico , Síndrome de Churg-Strauss/tratamento farmacológico
16.
Rev. colomb. reumatol ; 26(4): 268-270, oct.-dic. 2019. graf
Artigo em Inglês | LILACS | ID: biblio-1138819

RESUMO

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.


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Granulomatose com Poliangiite , Vasculite Sistêmica , Miosite , Inflamação
17.
Rev. cuba. reumatol ; 21(3): e100, sept.-dic. 2019. graf
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1093842

RESUMO

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


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


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Preparações Farmacêuticas , Granulomatose com Poliangiite/diagnóstico , Vasculite Sistêmica/complicações , Sons Respiratórios , Granulomatose com Poliangiite/diagnóstico por imagem
18.
Rev. colomb. reumatol ; 26(3): 216-219, jul.-set. 2019. tab, graf
Artigo em Espanhol | LILACS | ID: biblio-1126339

RESUMO

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.


Assuntos
Humanos , Feminino , Adolescente , Granulomatose com Poliangiite , Perda Auditiva , Manifestações Cutâneas , Vasculite Leucocitoclástica Cutânea , Diagnóstico Diferencial
19.
Rev. colomb. nefrol. (En línea) ; 6(1): 57-62, ene.-jun. 2019. graf
Artigo em Espanhol | LILACS, COLNAL | ID: biblio-1093026

RESUMO

Resumen Actualmente vivimos un proceso de transición en la pirámide poblacional. Por lo tanto, se hacen más frecuentes las enfermedades renales en los ancianos. Así, las vasculitis primarias en adultos mayores son enfermedades raras, causadas por la inflamación de los vasos sanguíneos y muy poco diagnosticadas. La literatura se limita a escasos casos clínicos. La vasculitis por anticuerpo citoplasmático anti neutrófilo (ANCA) positivo (VAA) es más frecuente en personas mayores de 50 años. La detección de ANCA es de peor pronóstico en esta población. También, es importante la alta sospecha y diagnóstico oportuno, ya que así se puede brindar un tratamiento óptimo y disminuir las complicaciones propias de la enfermedad y aquellas asociadas a la inmunosupresión. Los adultos mayores con VAA tienen mayor compromiso renal, hipertensión, dislipidemia y mortalidad incrementada. Por lo que a continuación se detallan dos casos clínicos desde la fecha del diagnóstico, edad, valores de laboratorio de la presentación inicial y la escala de actividad de vasculitis de Birmingham confirmados con biopsia renal (ANCA positivo) identificados en un hospital de especialidades de Ecuador.


Abstract Currently we live a process of transition in the population pyramid. Therefore, kidney diseases become more frequent in the elderly. Thus, primary vasculitis in older adults are rare diseases, caused by the inflammation of blood vessels and poorly diagnosed. The literature is limited to few clinical cases. Cytoplasmic anti-neutrophil antibody (ANCA) positive vasculitis (AAV) is more common in people older than 50 years. The detection of ANCA gives a very poor prognosis in this population. Also, high suspicion and timely diagnosis is important, as this can provide optimal treatment and reduce the complications of the disease and those associated with immunosuppression. Older adults with AAV have greater renal involvement, hypertension, dyslipidemia and increased mortality. Therefore, two clinical cases from the date of diagnosis, age, laboratory values of the initial presentation and the scale of activity of Birmingham vasculitis confirmed with renal biopsy (ANCA positive) identified in a specialty hospital of Ecuador are detailed below.


Assuntos
Humanos , Masculino , Feminino , Idoso , Vasculite , Granulomatose com Poliangiite , Equador , Injúria Renal Aguda
20.
Rev. med. Rosario ; 85(1): 17-19, ene.-abr. 2019. graf
Artigo em Espanhol | LILACS | ID: biblio-1052376

RESUMO

El síndrome pulmón-riñón es una entidad infrecuente, que comprende un gran espectro de patologías, como las vasculitis asociadas a ANCA y la enfermedad por anticuerpos antimembrana basal glomerular entre otras. Se describen en esta serie 12 casos donde las entidades más prevalentes fueron las antes mencionadas, observándose además un caso de lupus y uno de granulomatosis con poliangeítis, que se encuentran dentro de las causas menos frecuentes. La forma de presentación clínica inicial fue simultánea renal y pulmonar en 5/12 pacientes y renal en 7/12 de los mismos. El diagnóstico temprano de dichas patologías basándose en criterios clínicos, radiológicos, de laboratorio e histológicos, permite instaurar terapéuticas tempranas como la inmunosupresión y plasmaféresis, pudiendo prevenir complicaciones tales como las infecciones y la insuficiencia renal crónica terminal, siendo las primeras la principal causa de muerte (AU)


Pulmonary-renal syndrome is an infrequent condition. It includes a wide variety of conditions such as ANCA (antineutro-phil cytoplasmic autoantibody) associated with systemic vasculitis and anti-GBM (anti-glomerular basement membrane) disease among others. In this series we describe twelve cases, in which the most prevalent diseases were the above mentioned as well as one case of lupus and one of granulomatosis with polyangiitis (these being less frequent causes). The clinical presentation was both renal and pulmonary simultaneously in five of twelve patients and renal in seven of twelve patients. Early diagnosis of this condition on the basis of clinical, radiological, histological and analytic criteria allows early treatments such as immunosuppression and plasma exchange, thus avoiding complications such as infections (the main cause of death) and terminal chronic renal failure (AU)


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Glomerulonefrite/diagnóstico , Glomerulonefrite/terapia , Granulomatose com Poliangiite , Terapia de Imunossupressão , Plasmaferese , Doença Antimembrana Basal Glomerular/diagnóstico , Insuficiência Renal Crônica , Lúpus Eritematoso Sistêmico
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