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.

Pituitary dysfunction in granulomatosis with polyangiitis.

PURPOSE: Granulomatosis with polyangiitis (GPA) is a multisystem disease, characterized by necrotizing small-vessel vasculitis, which mainly affects the respiratory tract and the kidneys. Pituitary involvement in GPA is rare, present in about 1% of all cases of GPA. To date, only case reports or small case series have been published. Herein we report clinical features, imaging findings, treatment and outcomes in three patients with GPA-related pituitary dysfunction (PD). METHODS: A retrospective analysis of three cases of GPA-related PD was conducted, followed by systematic review of the English medical literature using PubMed. RESULTS: The three cases include three women aged between 32 and 37 years. PD was the presenting feature in one and two developed PD in the course of the disease. All patients had a pituitary lesion on MRI. Conventional treatment with high doses of glucocorticoids and cyclophosphamide led to resolution or improvement of the MRI abnormalities, whereas it was not effective in restoring PD. A systematic review identified 51 additional patients, showing that GPA can lead to partial or global PD, either at onset or, during the course of the disease. Secondary hypogonadism is the predominant manifestation, followed by diabetes insipidus (DI). Sellar mass with central cystic lesion is the most frequent radiological finding. CONCLUSION: GPA should be carefully considered in patients with a sellar mass and unusual clinical presentation with DI and systemic disease. Although conventional induction-remission treatment improves systemic symptoms and radiological pituitary abnormalities, hormonal deficiencies persist in most of the patients. Therefore, follow-up should include both imaging and pituitary function assessment.

Granulomatose de Wegener / Wegener?s granulomatosis

A granulomatose de Wegener é uma doença relativamente incomum e caracterizada por inflamação granulomatosa necrosante e vasculite de pequenos e médios vasos. Múltiplos órgãos são frequentemente envolvidos, com predileção para o trato respiratório, os olhos e os rins. Homens e mulheres de todas as idades podem ser afetados. A apresentação clínica das lesões dermatológicas é altamente variável, incluindo púrpura palpável, nódulos subcutâneos dolorosos, pápulas, pústulas e hiperplasia gengival. A realização de biópsia e análise histopatológica é essencial para a confirmação diagnóstica e diagnóstico diferencial, assim como outros exames, tais como radiografia e tomografia computadorizada de tórax. A doença possui diferentes graus de gravidade e cada caso deve ser analisado de forma específica para uma melhor opção terapêutica, objetivando evitar complicações precoces ou tardias...

Granulomatose de Wegener: relato de casos / Wegener's granulomatosis: cases report

JUSTIFICATIVA E OBJETIVOS: A granulomatose de Wegener (GW) é uma vasculite sistêmica de etiologia idiopática. É caracterizada por uma inflamação granulomatosa necrotizante, vasculite pauci-imune dos vasos de pequeno e médio calibres com compromisso predominantemente das vias respiratórias e dos rins. Está associada à presença de anticorpos anticitoplasma dos neutrófilos (ANCAs). O objetivo deste estudo foi a descrição de 2 casos de GW, salientando a importância do diagnóstico e terapêutica precoces para o prognóstico. RELATO DOS CASOS: Caso 1: Paciente do sexo feminino, 69 anos, com insuficiência renal rapidamente progressiva, glomerulonefrite pauci-imune com granulomas na biópsia renal e com positividade para o ANCA antimieloperoxidase (MPO). Caso 2:Paciente do sexo feminino, 26 anos, com otite refratária aos antibióticos, nódulos pulmonares e ANCA antiproteinase 3 (PR3)positivo. Foram tratadas com imunossupressores, mas em ambashouve lesão irreversível de órgãos (insuficiência renal terminal esurdez grave, respectivamente). CONCLUSÃO: A GW é uma doença pouco frequente, cujo diagnóstico permanece um desafio clínico. Este assenta na presença de sintomas constitucionais, compromisso das vias aéreas e renal, inflamação granulomatosa necrotizante e vasculite na histologia e presença de ANCAs. O tratamento baseia-se na imunossupressão. Salienta-se que a brevidade na determinação do diagnóstico e consequentemente no inicio da terapêutica é vital para o desfecho dos casos, reduzindo as sequelas irreversíveis e a morte.

BACKGROUND AND OBJECTIVES: Wegener's granulomatosis (WG) is a systemic vasculitis of unknown etiology. Characterized by necrotizing granulomatous inflammation and pauciimmune vasculitis of the small and medium-sized blood vessels affecting mainly the upper and lower respiratory tract and the kidneys. It's associated with antineutrophil cytoplasmic antibodies (ANCAs). The purpose of this study is to describe two clinical cases of WG emphasizing the importance of an early diagnosis and treatment in the prognosis.CASE REPORTS: Case 1: Female patient of 69 year-old, with rapidly progressive renal failure, pauci-immune glomerulonephritiswith granulomas in the renal biopsy and ANCA antimyeloperoxidase (MPO-ANCA). Case 2: Female patient of 26year-old with otitis media non responsive to antibiotics, pulmonary nodules, and ANCA directed against proteinase 3 (PR3).The patients were treated with immunosuppressive therapy, bothof them irreversible organ damage (renal failure and deafness). CONCLUSION: A WG it's an uncommon disease, which remains a clinical challenge. The diagnosis is based in the presence of constitutional symptoms, upper and lower airways and kidney involvement with necrotizing granulomatous inflammation and vasculitis, and associated with ANCAs. Therapy is based in immunossupression. We emphasize that early diagnosis and treatment is important to the outcome, minimizing irreversible organs damage and death.

Otomastoiditis como manifestación clínica inicial de granulomatosis de Wegener / Otomastoiditis as an initial clinical manifestation of Wegener's granulomatosis

La granulomatosis de Wegener (GW) forma parte del grupo de vasculitis primarias, de vasos pequeños y medianos, asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA). Esta enfermedad puede afectar cualquier órgano, especialmente vías aéreas superiores, inferiores y el riñón. Muy raramente la primera y única manifestación clínica de GW generalizada es la otomastoiditis. Presentamos el caso de un paciente que inició su enfermedad con una inflamación del oído medio, sin respuesta al tratamiento habitual. Posteriormente agregó compromiso nasal, pulmonar y renal. La determinación de anticuerpos anticitoplasma de neutrófilos y la biopsia transbronquial confirmaron el diagnóstico de GW. Comunicamos este caso para referir que la otomastoiditis del adulto, refractaria al tratamiento habitual, puede raramente ser la primera y única manifestación clínica de la GW.

Wegener's granulomatosis (WG) forms part of a group of primary vasculitis of the small and medium-sized blood vessels, associated with antineutrophil cytoplasmic antibodies (ANCA). This disease may affect any body organ, especially the upper and lower airways and the kidneys. Hardly ever is otomastoiditis the first and only clinical manifestation of generalized Wegener's granulomatosis. We present the case of a patient whose disease started with the inflammation of the middle ear, which was unresponsive to the usual treatment. Later he developed nasal, pulmonary and renal compromise. The determination of ANCA and a transbronchial biopsy confirmed the diagnosis of WG. We report this case to express the view that otomastoiditis in adults, which is refractory to the usual treatment, may seldom be the first and only clinical manifestation of WG.

Prevalência das manifestações clínicas iniciais da granulomatose de Wegener no Brasil: relato de seis casos e revisão da literatura / Wegener's granulomatosis: prevalence of the initial clinical manifestations: report of six cases and review of the literature

OBJETIVOS: Descrever as manifestações clínicas iniciais da Granulomatose de Wegener (GW) diagnosticada no Brasil. PACIENTES E MÉTODOS: Análise retrospectiva de seis prontuários do Serviço de Reumatologia do Hospital Geral de Fortaleza (HGF), assim como a realização de um levantamento bibliográfico dos casos de GW descritos no Brasil obtidos dos bancos de dados LILACS, SciELO e MEDLINE. RESULTADOS: O estudo identificou 49 pacientes; 15 (31 por cento) do sexo masculino e 34 (69 por cento) do sexo feminino. A forma sistêmica ocorreu em 35 pacientes (73 por cento): 28 adultos, cinco crianças e dois adolescentes. A doença limitada ocorreu em 13 adultos e uma criança. A média da idade adulta no início da doença foi de 42,2 anos (18 a 65 anos). O quadro clínico agudo, com sintomas há menos de três meses do diagnóstico, ocorreu em 41 por cento (20/49) da casuística e a forma insidiosa, em 59 por cento (29/49) dos pacientes. A prevalência das manifestações clínicas iniciais nos adultos com doença sistêmica (n = 28) foi 64 por cento (18/28) das vias aéreas superiores (VAS), 36 por cento (10/28) pulmonares, 18 por cento (5/28) renais, 25 por cento (7/28) oculares, 11 por cento (3/28) cutâneas, 25 por cento (7/28) musculoesqueléticas e 7 por cento (2/28) neurológicas. Na forma limitada do adulto (n = 13), os sintomas prevalentes foram 84 por cento (11/13) VAS, 23 por cento (3/13) oculares e 15 por cento (2/13) pulmonares. CONCLUSÃO: No Brasil, a prevalência das manifestações clínicas iniciais da GW foi semelhante aos resultados da literatura. A falta de especificidade dos sintomas pode retardar o diagnóstico na forma insidiosa da doença e aumentar a morbimortalidade das formas agudas.

OBJECTIVES: To describe the initial clinical manifestations of Wegener's Granulomatosis (WG) in Brazil. PATIENTS AND METHODS: Retrospective analysis of six medical records of WG patients followed-up at the Rheumatology Department of Hospital Geral of Fortaleza (HGF), as well as a bibliographic survey of cases of WG in Brazil on LILACS, SciELO, and MEDLINE databases. RESULTS: The study identified 49 patients, 15 (31 percent) males and 34 (69 percent) females. Systemic disease was observed in 35 patients (73 percent): 28 adults, 5 children, and 2 teenagers. Limited disease was observed in 13 adults and 1 child. The average age of onset in adults was 42.2 years (18 to 65 years). Acute clinical manifestations, with the onset of symptoms less than three months before the diagnosis, were observed in 41 percent (20/49) of the patients, and the insidious presentation in 59 percent (29/49) of the patients. The prevalence of the initial clinical manifestations in adults with systemic disease (n = 28) was 64 percent (18/28), upper airways, 36 percent (10/28), lungs, 18 percent (5/28), kidneys, 25 percent (7/28), eyes, 11 percent (3/28) skin, 25 percent (7/28), musculoskeletal, and 7 percent (2/28), neurological. In adults (n = 13) with limited disease, prevalent symptoms included: upper airway, 84 percent (10/13), eyes, 23 percent (3/13), and lungs, 15 percent (2/13). CONCLUSION: The prevalence of the initial clinical manifestations of WG in Brazil was similar to that reported in the literature. The lack of specific symptoms may delay diagnosis cases with insidious presentation of the disease and increase the morbidity and mortality in acute disease.

Granulomatosis de wegener refractaria: rol terapéutico de rituximab: revisión de tres casos / Refractoy wegener's granulomatosis: therapeutic rol of rituximab

El tratamiento convencional para la granulomatosis de Wegener, que incluye esteroides y ciclofosfamida, no es suficiente para el control de la actividad de la enfermedad en algunos pacientes considerados refractarios y conlleva cierta toxicidad relacionada al mismo. La depleción de células B con rituximab ha demostrado ser efectivo en el tratamiento de varias enfermedades autoinmunes, incluyendo las vasculitis sistémicas asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA). Reportamos tres casos de granulomatosis de Wegener refractarios tratados exitosamente con rituximab.

Conventional therapy for Wegener's granulomatosis, steroid and cyclophosphamide, fails to control disease activity in same refractorypatients and has treatment-related toxicity. B cell depletion therapyusing rituximab has been shown to be effective for certain autoimmunediseases, including antineutrophil cytoplasmic antibody (ANCA) associated vasculitis. We report three refractory cases of Wegener’s granulomatosis successfully treated with rituximab.

Granulomatosis de Wegener y tuberculosis: Una mala combinación / Wegener's granulomatosis and tuberculosis: A bad combination

La enfermedad de Wegener es una vasculitis de pequeños y medianos vasos asociada a anticuerpos anticitoplasma del neutrófilo (ANCA). Dentro de los órganos blancos, el pulmón se encuentra comprometido en el 85% de los casos. Numerosas entidades deben ser consideradas en el diagnóstico diferencial, entre ellas, la tuberculosis pulmonar. Presentamos el caso de un hombre de 54 años de edad, con diagnóstico en el año 1996 de enfermedad de Wegener, que comienza en agosto de 2007 con expectoración hemoptoica, disnea de esfuerzo progresiva y esputo con baciloscopia BAAR (+), por lo que inicia tratamiento antituberculoso. La baciloscopia no se constata en dos lavados broncoalveolares contemporáneos. Evoluciona con deterioro de la función renal, púrpura palpable y anticuerpos anticitoplasma del neutrófilo patrón citoplasmático (ANCA-c) positivo. Se interpreta reactivación de su enfermedad de base. Inicia tratamiento inmunosupresor y hemodiálisis y suspende tratamiento antituberculoso. Un mes después del alta se reinterna con cuadro similar al previo, con esputo seriado positivo para BAAR.

Wegener's disease is a vasculitis of small and medium-sized vessels associated with anti-neutrophil cytoplasm antibodies (ANCA). Within their target organs the lungs are involved in 85% of cases. Many entities are part of the differential diagnosis, including pulmonary tuberculosis. We present the case of a 54 years old man, diagnosed as Wegener's disease in 1996 which begins in August 2007 with cough, hemoptysis, progressive dyspnea on effort and serial sputum positive for AFB and then starts TB treatment. Subsequent bronchoalveolar fluids resulted negative for AFB. The patient evolved with impaired renal function, palpable purpura and positive anti-neutrophil cytoplasm antibodies cytoplasmic pattern (c-ANCA), interpreted as Wegener's disease relapse. He started hemodialysis and immunosuppressive therapy and tuberculosis treatment was stopped. One month after discharge was readmitted with a similar picture with serial sputum positive for AFB.

Diagnóstico de granulomatosis de Wegener en pacientes con enfermedades oculares inflamatorias / Diagnosis of Wegener's granulomatosis in patients with ocular inflamatory disease

Objetivo. Informar las manifestaciones oftalmológicas en pacientes con granulomatosis de Wegener (GW). Método. Se revisó la base de datos del Instituto de Oftalmología Conde de Valenciana. Se recolectó la exploración oftalmológica completa, los exámenes de laboratorio y de gabinete, el tratamiento y la evolución. Resultados. Se incluyeron 11 pacientes con GW (18 ojos). Siete pacientes masculinos y 4 femeninos con edad promedio de 43.7 años (28-55). Tres habían tenido diagnóstico previo de GW y los 8 restantes se diagnosticaron en nuestro departamento. Siete presentaron cuadros bilaterales y 4 unilaterales. Las formas de presentación clínica fueron escleritis necrosante con queratitis ulcerativa periférica (QUP) (7/18), escleritis difusa (3/18), escleritis nodular (1/18), uveítis anterior no granulomatosa (1/18), neuropatía óptica isquémica (1/18), neuropatía óptica retrobulbar (1/18), desprendimiento de retina seroso (2/18) y dacriocistitis (2/18). De los 18 ojos, la capacidad visual final fue mejor o igual a 20/40 en 13, 20/400 en 3, cuenta dedos a 30cm o no-percepción de luz en 1. Actualmente 7 pacientes se encuentran en fase inactiva. Conclusiones. Las manifestaciones oftalmológicas más frecuentes en pacientes con GW fueron: escleritis necrosante y QUP. En la mayoría, la GW se diagnosticó después de las manifestaciones oftalmológicas, sin embargo, todos presentaron síntomas sistémicos u oftalmológicos previos.

OBJECTIVE: Report the ophthalmologic manifestations among patients with Wegener 's Granulomatosis (WG). METHOD: We reviewed the database of the Instituto de Oftalmologia Fundación Conde de Valenciana in order to collect information regarding complete ophthalmic examination, laboratory and cabinet tests, treatment, and disease progression. RESULTS: We included 11 patients with WG (18 eyes). Seven men and four women, mean age 43.7 years (range = 28-55). Three patients had a prior diagnosis of WG and the remaining eight patients were diagnosed by our study team. Seven subjects developed a bilateral affection and four had unilateral involvement. The clinical presentation was necrotizing scleritis with peripheral ulcerative keratitis (PUK) (7/18), diffuse scleritis (3/18), nodular scleritis (1/18), non-granulomatous uveitis (1/18), optic ischemic neuropathy (1/18), retrobulbar neuritis (1/18), serous retinal detachment (2/18), and dacryocystitis (2/18). Final visual acuity was better or equal to 20/40 (13/18), 20/400 (3/18), finger-counting or no-perception of light in 1/18. Currently, seven patients are symptom free. CONCLUSIONS: The most frequent ophthalmic manifestations among our patients with WG were: necrotizing scleritis and PUK. In most cases, WG was diagnosed after ophthalmic manifestations; however, all patients displayed prior systemic or ocular symptoms.

A Case of Wegener's Granulomatosis in the Orbital Area

Wegener`s granulomatosis(WG) is uncommon disease characterized by necrotizing granulomatous lesions in the both upper and lower respiratory tracts, generalized focal necrotizing vasculitis, and focal necrotizing glomerulonephritis. Other organs are also involved frequently. Ocular complications may occur secondary to contiguous granulomatous sinusitis or as a result of focal vasculitis and are encountered in 40-50% of cases of generalized form. We experienced a case of WG developed progressive paranasal sinusitis and rhinitis combined with orbital and ocular involvement. The diagnosis was confirmed by clinical findings and histopathologic features of tissue biopsy. The histopathology from periorbital area had typical findings of WG with necrotizing vasculitis and granuloma formation.

Wegener`s Granulomatosis of the Ureter Mimicking Ureteral Tumor / 대한비뇨기과학회지

Wegener`s granulomatosis (WG) is a multisystem disorder of unknown origin characterized by necrotizing granulomatous vasculitis. WG usually affects the upper respiratory tract, lungs, and kidneys with focal glomerulonephritis, but almost any organ can be affected. The ureter is primarily an unusual location for lesions of WG. A 30-year-old woman presented with intermittent right flank pain and hematuria. A renal ultrasound demonstrated unilateral hydronephrosis and a retrograde pyelography revealed a filling defect at right mid ureter and a computed tomography displayed marked concentric thickening of the right ureteral wall which was mimicking ureteral tumor. At nephroureterectomy, the right ureter was found to be obstructed by dense, intramural fibroinflammatory reaction. There was a necrotizing granulomatous vasculitis in the muscle layer of the ureter. Our case represents the rare occurrence of WG presenting ureteral bstruction.

A Case of Protracted Wegener`s Granulomatosis Which is Progress to Generalized Form / 대한내과학회지

Wegener's granulomatosis is a necrotizing and granulomatous vasculitis which involves upper and lower respiratory tract, kidney and skin. The patient who have the protracted Wegener's granulomatosis live a long peroid without major organ imvolvement, In mild cases, the progression is slow and may not need systemic cyclophosphamide treatment. Some cases, however, demonstrate renal involvement and may result in renal failure and death if adequate treatment is not provided. We experienced a case of protracted Wegener's granulomatosis that was a new concept and has not been reported !n Korea. The diagnosis was confirmed by clinical finding and histopathologic features of tiasue biopsy. This case represents a protracted Wegener's granulomatosis with paranasal sinusitis, rhinitis and skin purpura and progress to a gener alized form with kidney involvement after 6years. Treatment with oral cyclophosphamide, steroid and sulfamethoxazole-trimethoprime result improvement of skin, nasal symptom and labratory parameters.

Wegener' s Granulomatosis / 대한피부과학회지

Wegener's granulomatosis is a systemic necrotizing vasculitis of unknown cause. The disease is characterized by the involvement of the upper airway, the lung, and the kidney. Skin lesions are frequent and the most common lesion is purpura distributed on the limbs and trunk. A 34-year-old female showed recurrent purpuric macules on the both lower extremities and buttocks. The patient. showed nasal septal perforation with saddle nose deformity and C-ANCA positivity. Histopathologic findings of purpuric lesion revealed the features of necrotizing vasculitis. The histologic specimen from the nasal cavity showed chronic inflammation with granuloma formation and kidney showed focal necrotizing glomerulonephritis. Therefore, we treated the patient with prednisolone and cyclophosphamide having diagnosed Wegener's granulomatosis.