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

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

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

Association between Wegener´s granulomatosis and severe lumbar pain

Introducción: La granulomatosis de Wegener (GW) es una enfermedad autoinmune sistémica caracterizada por vasculitis granulomatosa necrotizante que afecta principalmente a las vías respiratorias superiores, pulmones y riñones. Sin embargo, con menos frecuencia puede afectar a los músculos, las articulaciones, la piel, los ojos, el sistema cardiovascular y el sistema nervioso. La presencia de dolor lumbar consiste en una manifestación clínica inusual debido a la afectación del sistema nervioso. Objetivo: El objetivo de este estudio es alertar a los profesionales de la salud acerca de la posibilidad de cortar el dolor lumbar estar relacionado con la granulomatosis de Wegener, su impacto en la vida diaria del paciente, así como los signos clínicos y las formas de diagnóstico. Materiales y Métodos: Revisión de la literatura utilizando PubMed, MEDLINE, Google Scholar, SciELO, EBSCO. Los trabajos seleccionados entre 1995 y 2013 por un total de 48 obras de las cuales se seleccionaron 21 de acuerdo con sus informes de afectación neurológica, diagnóstico y tratamiento. Discusión: Los síntomas neurológicos pueden ocurrir en 22-50 por ciento de los pacientes durante el curso de la GW. sistema nervioso (SNC) central es poco frecuente (sólo 2-8 por ciento de los pacientes) dolor lumbar .Severe es una manifestación clínica poco frecuente y puede estar asociada con la participación de sistema nervioso central y periférico. CNS debido a la compresión de la médula espinal a nivel lumbar. sistema nervioso periférico debido a la compresión de las raíces nerviosas. Conclusión: La granulomatosis de Wegener es ser una enfermedad sistémica puede presentar diferentes manifestaciones clínicas De acuerdo con el sitio involucrado. Es asociaciones con el dolor lumbar es rara y la refleja la afectación neurológica. Por lo tanto, en pacientes con dolor lumbar grave sin diagnóstico confirmado, granulomatosis de Wegener no debería ser considerado.

Introduction: Wegener’s granulomatosis (WG) is a systemic autoimmune disease characterized by necrotizing granulomatousvasculitis which primarily affects upper respiratory tract, lungs and kidneys. However, less frequently can affect muscles,joints, skin, eyes, cardiovascular system and nervous system. The presence of lumbar pain consists in an unusual clinicalmanifestation due to the involvement of the nervous system. Objective: The objective of this study is to alert health professionalsabout the possibility that severe lumbar pain be related to Wegener’s granulomatosis, it’s impact on the patient’s dailylife as well as clinical signs and diagnosis forms. Materials and Methods: Literature review using PubMed, MEDLINE, GoogleScholar, SciELO, EBSCO. Selected works from 1995 to 2013 totaling 48 works of which 21 were selected according to theirreports of neurological involvement, diagnosis and treatment. Discussion: Neurological symptoms may occur in 22-50 percent of patients during the course of WG. Central nervous system (CNS) involvement is uncommon (only 2-8 percent of patients).Severelumbar pain is a rare clinic manifestation and it can be associated with the involvement of central and peripheral nervous system.CNS due to compression of the spinal cord at the lumbar level. Peripheral nervous system due to compression of nerveroots. Conclusion: Wegener’s granulomatosis for being a systemic disease can present different clinical manifestations accordingto the involved site. It’s associations with lumbar pain is rare and reflects it’s neurological involvement. Therefore, inpatients with severe lumbar pain without confirmed diagnosis, Wegener’s granulomatosis should be considered.

Granulomatosis with Polyangiitis – A case report

Granulomatosis with Polyangiitis (GPA) is a rare multisystem autoimmune disease. It is characterized histopathologically by necrotizing granulomatous vasculitis. The classical clinical triad consists of upper airway involvement (characterized by sinusitis, otitis, nasal mucosa ulcers, bone deformities, and subglottic stenosis), lower respiratory tract involvement (cough, chest pain, hemoptysis) and glomerulonephritis. We reported here a case of Wegener’s granulomatosis presenting as a thick walled cavity.

Isolated necrotizing granulomatous vasculitis of the uterine cervix associated with human papillomavirus infection / Vasculite granulomatosa necrosante isolada de colo uterino associada à infecção por papilomavírus humano

Vasculites de órgão único, ou isoladas, já foram descritas em diversos órgãos e seu achado pode ser acidental. Relatamos um caso de vasculite granulomatosa necrosante isolada de colo uterino em uma paciente de meia-idade, previamente hígida, sexualmente ativa, e cuja pesquisa de DNA de papilomavírus humano (Human Papiloma Virus - HPV) por captura híbrida foi positiva. Não foi identificado comprometimento sistêmico e, como houve excisão completa da lesão, optou-se pelo acompanhamento clínico. Há poucos relatos, na literatura, de acometimento do trato genital feminino de forma isolada, alguns com presença simultânea de lesões que podem ser causadas pelo HPV, postulando-se uma associação patogênica.

Single organ vasculitis (SOV), or isolated vasculitis, has been described in several organs and it can be an accidental finding. We report a case of isolated necrotizing granulomatous vasculitis of the uterine cervix in a middle-aged woman, previously healthy, and sexually active, and whose human papillomavirus (HPV) DNA hybrid capture assay was positive. Systemic involvement was not detected and, since the lesion was completely removed, we opted for a clinical follow-up. The literature has very few reports on the isolated involvement of the female genital tract, and some had concomitant lesions that could be caused by the HPV, indicati.

Primary Necrotizing Granulomatous Vasculitis of the Stomach

A 55-year-old woman suffered from upper abdominal pain for two months and remained refractile against the anti-ulcer regimen. The palliative gastrectomy specimen revealed multiple shallow ulcerations on the thickened mucosal folds mainly in the antrum and body along the greater curvature where multiple, whitish nodules were found in the submucosa. Microscopically, individual submucosal nodules clearly corresponded to the necrotizing granulomatous vasculitis which were featured with diffuse fibrinoid necrosis of arterial walls accompanying granuloma formation and heavy infiltration of neutrophils, eosinophils, histiocytes and giant cells. Similar vasculitic lesions involved venules and arterioles. There were scattered vasculitic changes in the liver biopsy specimens and omentum. There were no clinical presentations or serological support of systemic involvement including systemic lupus erythematosus, Henoch-Schoenlein purpura, cryoglobulinemia or Churg-Strauss granulomatous vasculitis. We conclude that this is a hitherto undescribed primary necrotizing granulomatous vasculitis predominantly involving the stomach.