Pulmonary sarcoidosis with arterial involvement

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.

Actualización en el estudio de granulomatosis con poliangeitis (granulomatosis de Wegener) / Update in study of granulomatosis with polyangiitis (Wegener's granulomatosis)

Resumen:La granulomatosis con poliangeítis (GPA) es una vasculitis sistémica de pequeño vaso, que afecta más frecuentemente el tracto respiratorio y el riñón. Sus criterios diagnósticos se basan en la clínica, exámenes de laboratorio, imágenes e histología. El 90% son ANCA (anticuerpos anticitoplasma de neutrófilos) positivos. La histología muestra inflamación granulomatosa, necrosis y vasculitis. Los exámenes de imagen son de vital importancia en su estudio inicial y seguimiento, correspondiendo principalmente a técnicas tomográficas. La tomografía Computada (TC) es el método de elección para la evaluación de vía aérea superior y pulmón, con alta sensibilidad en afectación de cavidades nasal/paranasales, árbol bronquial y pulmón. La Resonancia Magnética está indicada en compromiso del sistema nervioso central y corazón. El PET/CT presenta alta sensibilidad en enfermedad tóraco-abdominal, es de utilidad en detectar lesiones no visibles con otras técnicas, y en control de tratamiento. El compromiso renal, de alta ocurrencia en GPA, presenta escasa traducción en las imágenes y es frecuentemente indetectable con imágenes, aunque el PET/CT puede ser positivo en casos de glomerulonefritis acentuada. La radiología simple no debe ser utilizada en el estudio de GPA dado su bajo rendimiento diagnóstico. El tratamiento se basa en terapia corticoidea e inmunosupresora. Las recaídas son frecuentes, por lo que estos pacientes requieren seguimiento a largo plazo.

Abstract:Granulomatosis with polyangiitis (GPA) is a systemic type of vasculitis that affects small vessels, most commonly involving the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, imaging and histology. Ninety percent are ANCA (anti-neutrophilic cytoplasmic antibodies) positive. Histology demonstrates granulomatous inflammation, necrosis and vasculitis. Imaging studies are vital for the initial work-up and follow-up. Computed Tomography (CT) is the method of choice for evaluation of the upper airway and lungs, because of its high sensitivity detecting anomalies of paranasal sinuses, bronchial tree and lungs. Magnetic Resonance is indicated for evaluation of the central nervous system and heart. PET/CT has high sensitivity for thoracic and abdominal disease, is useful at detecting lesions not seen with other imaging techniques, and for follow-up. Renal involvement, very frequent on GPA, is usually undetectable at imaging, but may be seen at PET/CT in cases of marked glomerulonephritis. Plain X-rays should not be used for evaluation of GPA because of their low diagnostic performance. Treatment is based on corticosteroid and immunosuppressive therapy. Relapses are frequent, so these patients require long-term follow-up.

Bilateral acute angle-closure glaucoma as a first presentation of granulomatosis with polyangiitis (Wegener's) / Glaucoma de ângulo fechado bilateral agudo como uma primeira apresentação da granulomatose com poliangeíte (de Wegener)

ABSTRACT We report a case of bilateral acute angle-closure glaucoma in a patient with undiagnosed granulomatosis with polyangiitis (Wegener's). A 59-year-old man presented with a severe headache, ocular pain, blurred vision, shortness of breath, and mild fever. Clinical examination revealed conjunctival chemosis, corneal edema, and shallow anterior chambers. Closed angles were observed bilaterally on gonioscopy. The patient was treated with intravenous mannitol, oral acetazolamide, and anti-glaucomatous eye drops. Over the following two days, his vision improved and intraocular pressures decreased. Subsequently, laser iridotomies were performed bilaterally and the patient attended consultations with our departments of respiratory medicine, nephrology, and rheumatology and was subsequently diagnosed with granulomatosis with polyangiitis. Bilateral acute angle-closure glaucoma is a very rare ocular manifestation of granulomatosis with polyangiitis. The association of this clinical entity with Wegener's granulomatosis remains unknown.

RESUMO Relatamos um caso glaucoma bilateral agudo de ângulo fechado em um paciente sem diagnóstico prévio de granulomatose com poliangeíte (Wegener). Um homem de 59 anos apresentou-se com uma forte dor de cabeça, dor nos olhos, visão turva, dificuldade em respirar e febre baixa. Observamos quemose conjuntival, edema da córnea e câmara anterior rasa. A gonioscopia demonstrou ângulos fechados bilateralmente. Ele foi tratado com manitol intravenoso, acetazolamida oral, olho e colírios antiglaucomatosos. Durante os dois dias seguintes a sua visão melhorou e as pressões intra-oculares diminuíram. A seguir, foram realizadas iridotomias a laser bilateralmente e ele foi referido para os departamentos de doenças pulmonares, nefrologia e reumatologia. Ele foi diagnosticado com poliangeíte granulomatosa. Glaucoma bilateral agudo de ângulo fechado é uma entidade clínica muito rara e sua associação com a granulomatose de Wegener é desconhecida e deve acrescentar-se à lista de manifestações oculares de granulomatose com poliangeíte.

Granulomatosis with polyangiitis, a new nomenclature for Wegener's Granulomatosis - Case report

The granulomatosis with polyangiitis, initially known as Wegener's granulomatosis, is a small and medium vessels vasculitis. It's classic form presents a triad: necrotizing granuloma of respiratory tract, necrotizing cutaneous vasculitis and glomerulonephritis. This vasculitis has cytoplasmic antineutrophil antibodies as signal. This work illustrates a case, of multisystemic rare disease, in which the segment and treatment were considered satisfactory for symptoms remission.

Granulomatosis eosinofílica con poliangeítis: reporte de caso yrevisión de la literatura / Eosinophilic granulomatosis with polyangiitis: case report and review of the literature

La granulomatosis eosinofílica con poliangeítis (Síndrome de Churg-Strauss) es una enfermedad vasculítica primaria poco frecuente. El diagnóstico actualmente se define a partir de la presencia de al menos cuatro de seis criterios propuestos por la Sociedad Americana de Reumatología, los cuales incluyen: asma bronquial, eosinofilia mayor que 10 por ciento, sinusitis paranasal, infiltración pulmonar, evidencia histológica de vasculitis y compromiso neurológico ya sea mono o polineuropático. En el presente artículo se reporta el caso de un paciente de 56 años con antecedentes de asma bronquial, rinitis alérgica y poliposis nasal operada, derivado a nuestro centro por cuadro de aumento de volumen doloroso en ambas extremidades inferiores, baja de peso, parestesias y debilidad muscular. Asociado a esto desarrolló lesiones purpúricas palpables cuya biopsia resultó compatible con granulomatosis eosinofílica con poliangeítis. El paciente posteriormente recibió tratamiento inmunosupresor con prednisona y un pulso de ciclofosfamida con buena respuesta clínica. Se presenta una revisión bibliográfica a propósito del caso.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome) is an uncommon primary vasculitis. The diagnosis is currently defined by the presence of at least four of six criteria proposed by the American College of Rheumatology, which include: asthma, eosinophilia less than 10 percent, paranasal sinusitis, pulmonary infiltration, histologic evidence of vasculitis and neurologic compromise as mono or polyneuropathy. In the present article, we report the case of a 56 year-old man with history of asthma, allergic rhinitis and operated nasal polyposis, referred to our center with painful bulking in both lower extremities, weight loss, paresthesias and muscle weakness. It also developed palpable purpura. Biopsy of skin lesions was compatible with eosinophilic granulomatosis with polyangiitis. The patient subsequently received immunosuppressive therapy with prednisone and a cyclophosphamide bolus with good clinical response. A review on the subject is also presented.

Hemorragia alveolar na granulomatose com poliangeíte: um desafio favorável / Alveolar hemorrhage in granulomatosis with polyangiitis: a positive challenge

A granulomatose com poliangeíte (GPA) é uma vasculite necrosante multissistêmica de etiologia desconhecida que atinge vasos de pequeno e médio calibre, principalmente das vias aéreas e dos rins, acometendo, em sua maioria, indivíduos entre 30 e 50 anos, cuja evolução pode ser fatal se a terapêutica adequada não for instituída em tempo hábil...

Pulmonary renal syndrome in a case of wegener´s granulomatosis.

We report a case of a 42-year-old patient who presented with Wegener’s granulomatosis complicated by pulmonary renal syndrome, i.e., diffuse alveolar haemorrhage and rapidly progressive crescentic glomerulonephritis. The patient was treated with plasmapheresis and immunosuppressive drugs — intravenous cyclophosphamide and methyl prednisolone. The clinical, haematological and biochemical parameters improved substantially and remission is achieved.

Presentación de un caso de vasculitis de Wegener / Presentation of a case of Wegener’s vasculitis

Se presenta una paciente con granulomatosis de Wegener en su forma localizada y se efectúa una actualización de las manifestaciones clínicas, el diagnóstico y el tratamiento.

Trombose em artérias pulmonares pequenas e médias em granulomatose de Wegener: um estudo com microscopia confocal por varredura a laser / Thrombosis in small and medium-sized pulmonary arteries in Wegener's granulomatosis: a confocal laser scanning microscopy study

OBJETIVO: A granulomatose de Wegener (GW) pode causar dano nas células endoteliais e fenômenos tromboembólicos. Entretanto, poucos estudos analisaram a microcirculação pulmonar - artérias pulmonares de pequeno/médio calibre (APPMC) - em pacientes com GW. O objetivo deste estudo foi quantificar trombos de fibrina em amostras de APPMC de pacientes com GW. MÉTODOS: Analisamos 24 APPMC de seis pacientes com GW e 16 APPMC de quatro pacientes controles sem WG. Utilizamos CD34 para a marcação do endotélio em todas as amostras e microscopia confocal a laser para detectar trombos de fibrina intravasculares. Calculamos a área total do vaso, a área livre do lúmen e a área trombótica. RESULTADOS: A média da área total do vaso foi similar no grupo GW e no grupo controle (32.604 µm² vs. 32.970 µm², p = 0,8793). Trombos foram identificados em 22 das 24 APPMC (91,67 por cento) no grupo GW, e em nenhuma do grupo controle (p < 0,0001; OR = 297 (IC95 por cento: 13,34-6.612). A média da área trombótica foi maior no grupo GW do que no grupo controle (10.068 µm² vs. 0.000 µm², p < 0,0001). Em contraste, a média da área livre do lúmen foi menor no grupo GW que no grupo controle (6.116 µm² vs. 24.707 µm², p < 0,0001). CONCLUSÕES: A microscopia confocal a laser mostrou uma associação significante entre trombose microvascular pulmonar e GW. Isso sugere um possível papel da trombose microvascular na fisiopatologia da GW pulmonar, evocando o potencial benefício da anticoagulação na GW pulmonar. Entretanto, novos estudos são necessários para confirmar nossos achados, assim como um ensaio clínico randomizado a fim de testar o papel da anticoagulação no tratamento de pacientes com GW pulmonar.

OBJECTIVE: Wegener's granulomatosis (WG) can cause endothelial cell damage and thromboembolic events. Nevertheless, there have been few studies on the pulmonary microcirculation-small and medium-sized pulmonary arteries (SMSPA)-in patients with WG. The objective of this study was to quantify fibrin thrombi in the SMSPA of patients with WG. METHODS: We analyzed 24 SMSPA samples collected from six patients with WG and 16 SMSPA samples collected from four patients without WG. In all samples, we used the endothelial cell marker CD34 and confocal laser scanning microscopy in order to detect intravascular fibrin thrombi. We calculated the total vessel area, the free lumen area, and the thrombotic area. RESULTS: The mean total vessel area was similar in the WG and control groups (32,604 µm² vs. 32,970 µm², p = 0.8793). Thrombi were present in 22 (91.67 percent) of the 24 WG group samples and in none of the control group samples (p < 0.0001; OR = 297; 95 percent CI: 13.34-6,612). The mean thrombotic area was greater in the WG group samples than in the control group samples (10,068 µm² vs. 0.000 µm²; p < 0.0001). In contrast, the mean free lumen area was smaller in the WG group samples than in the control group samples (6,116 µm² vs. 24,707 µm²; p < 0.0001). CONCLUSIONS: Confocal laser scanning microscopy revealed a significant association between pulmonary microvascular thrombosis and WG. This suggests a possible role of microvascular thrombosis in the pathophysiology of pulmonary WG, evoking the potential benefits of anticoagulation therapy in pulmonary WG. However, further studies are needed in order to confirm our findings, and randomized clinical trials should be conducted in order to test the role of anticoagulation therapy in the treatment of patients with pulmonary WG.

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.

Estudo da camada elástica das arteríolas pulmonares em portadores de granulomatose de Wegener / Study of the elastic fibers of the pulmonary arteriolas of patients with Wegener granulomatosis

A Granulomatose de Wegener é uma síndrome clínico-patológica caracterizada por vasculite granulomatosa necrozante que acomete o trato respiratório superior, pulmões e rins. A vasculite granulomatosa afeta os vasos pulmonares com destruição das camadas elásticas das artérias.. O objetivo do presente estudo foi quantificar o sistema elástico da parede das artérias pulmonares de pacientes portadores de Granulomatose de Wegener comparado com o de pacientes normais. Foram estudadas 36 artérias de pequeno calibre ( diâmetro < 62 micra) e 36 artérias de médio calibre ( diâmetro entre 62 e 213 micra) obtidas de tecido pulmonar de 6 pacientes portadores de Granulomatose de Wegener comparadas com 36 artérias de pequeno e 36 artérias de médio calibre obtidas de 6 pacientes normais...

Wegener Granulomatosis is a clinico-pathologic syndrome characterized by necrotizing granulomatous vasculitis that affects the upper respiratory tract, the lungs and the kidneys. The granulomatous vasculitis affects the pulmonary vessels with destruction of the elstic system fibers of the vessels walls that can be visualized with the Verhoff staining technique . The objective of the present study was to quantify the area of the elastic system fibers of the walls of the pulmonary arteries of patients with the diagnosis of Wegener Granulomatosis compared to the area of the elastic system fibers of normal patients. We studied 36 small size pulmonary arteries ( diameter < 62 µ) and 36 medium size pulmonary arteries ( diameter between 62 and 213µ) taken from lung tissue of 6 patients with the diagnosis of Wegener Granulomatosis compared to 36 small size and 36 medium size pulmonary arteries taken from lung tissue of normal patients. The elastic system fibers of the pulmonary arteries walls was atained by resorcine-fucsine technique e the área of the elastic system was quantified through a special softer for área calculation of the Image analyser FOX pro of the Faculty of Medicine of the University of São Paulo. The medium area of the elastic system fibers of the small size pulmonary arteries of Wegener Granulomatosis was 73,6 ± 20,9 µ² ( 29,76 to 109,05 µ²) compared to 680,8 ± 256,1 µ² ( 263,34 to 1380,75 µ²) of the normal patients ( p< 0,0001- ANOVA -2 factors)...

Endoscopic Laser Surgery for Subglottic Stenosis in Wegener's Granulomatosis

PURPOSE: Wegener's granulomatosis (WG) is a rare multisystem inflammatory disease, which infrequently involves the subglottic area and trachea. Treatment usually involves the use of immunosuppressive agents with corticosteroids. Some patients, however, continue to have symptoms of airway obstruction after clinical remission following the standard therapeutic regimen. Objective: To investigate laser treatment for subglottic stenosis in five patients suffering from WG. MATERIALS AND METHODS: We endoscopically treated 5 patients with subglottic stenosis due to WG and airway obstruction by Nd:YAG and CO2 lasers. One of the patients had preoperative tracheostomy and after treatment was decannulated and could not breathe without dyspnea. Another patient required stenting of the subglottic area. RESULTS: All five patients were able to breathe without dyspnea after the treatment. Three patients were treated with an Nd:YAG laser but needed repeated laser treatment every four to six months, whenever they complained of dyspnea. The other two patients were treated with a CO2 laser; one of these patients had preoperative tracheostomy and was treated twice by CO2 laser and decannulated, with no further difficulty in breathing. The follow-up period was 1-5 years. CONCLUSIONS: Nd:YAG and CO2 lasers are recommended in the treatment of subglottic stenosis (SS) due to WG, particularly when the stenosis is in continuity or close proximity to the vocal cords.

Manifestaciones clínicas de la granulomatosis de Wegener y la poliangeítis microscópica en Santiago-Chile, 1990-2001 / Clinical features of Wegener granulomatosis and microscopic polyangiitis in Chilean patients

Background: Systemic vasculitis are a group of heterogeneous diseases characterized by inflammation and necrosis of blood vessel walls. The etiology is not known, but geographic and environmental factors are implicated. Aim: To describe the clinical features of microscopic polyangiitis (MPA) and Wegener's granulomatosis (WG) in a Chilean cohort of patients. Patients and methods: Retrospective review of the medical records of 123 patients with the diagnosis of systemic vasculitis (65 MPA and 58 WG), seen from 1990 to 2001. The diagnosis were made based on the American College of Rheumatology and Chapel Hill criteria. Results: The mean follow-up for MPA was 15 months (1-120) and for WG, 20 months (1-120). The median age (years) at diagnosis for MPA was 61 (19-82) and WG 50 (20-82). Gender distribution was similar in both groups (male: 68percent and 57percent respectively).The main clinical features in the MPA group were renal involvement (68percent), peripheral nervous system involvement (57percent), pulmonary hemorrhage (28percent), and skin disease (32percent). In the WG group were alveolar hemorrhage (62percent), renal involvement (78percent), paranasal sinus involvement (57percent), and ocular disease (26percent). In both, creatinine levels above 2.0 mg/dl were associated with a higher mortality (p< 0.01). ANCA by immunofluorescence was performed in 56 MPA patients (75percent had pANCA, 4percent had cANCA and 21percent were ANCA negative) and in 55 WG patients (17percent had pANCA, 79percent had cANCA and 4percent were ANCA negative). Global mortality was 18percent and 17percent respectively, and the most common causes of death were infections. Conclusions: The clinical features of our patients are similar to other published data. In our WG and MPA patients the main predictor for death was a serum creatinine above 2 mg/dl.

Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl

CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpasture´s syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM). Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA), microscopic polyangiitis and Wegener´s granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14 percent, platelets = 260,000, white blood cells = 8300, 74 percent segmented, 4 percent eosinophils, 19 percent lymphocytes and 3 percent monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA) test with antigen specificity for myeloperoxidase (anti-MPO) was positive and the circulating anti-GBM showed an indeterminate result

Clinicopathologic study of Wegener's granulomatosis with special emphasis on early lesions in 10 Korean patients

We reviewed ten cases of Wegener's granulomatosis with special emphasis on the characteristics of the early stage of Wegener's granulomatosis. All patients presented with nonspecific symptoms and signs, so that Wegener's granulomatosis was not initially considered. However, half of the patients had clinical or radiologic disease in the nose/or paranasal sinuses as the primary presenting problems and showed neutrophil microabscess surrounded by palisading epithelioid cells and irregularly arranged giant cells in the nasal biopsy as the most characteristic feature. Five of ten patients were believed to have a protracted superficial phenomenon before involvement of other organs, specifically the lung or kidney. Four of ten patients showed nonreactivity to ANCA test at the time of presentation. Although the number of cases reviewed in this study was small, the rate of nonreactivity to ANCA was higher than those of the larger series. The importance of early diagnosis of Wegener's granulomatosis can not be overemphasized in view of the fact that cases unrecognized clinicopathologically finally progress to full-blown systemic form of Wegener's granulomatosis with poor prognosis. The diagnosis of Wegener's granulomatosis should be based on a thorough and meticulous examination of its characteristic histologic changes in biopsied tissue particularly extravascular foci.

Granulomatosis de Wegener: a propósito de un caso / Wegener's granulomatosis: case report

La granulomatosis de Wegener se caracteriza por la presencia de lesiones granulomatosas necrosantes de vías áereas, glomerulonefritis y con frecuencia, vasculitis que afecta a otros órganos. Presentamos una paciente con granulomatosis de Wegener y comentamos la clínica, patogenia, diagnóstico y tratamiento de dicha enfermedad

Granulomatosis de Wagener con afectación coroidea como diagnóstico diferencial de melanoma uveal. Reporte de un caso / Wegener's granulomatosis with choroidal mass as differential diagnosis of choroid melanoma. Case report

Presentamos una paciente de 52 años de edad con tumoración coroidea como única manifestación de granulomatosis de Wegener cuyo diagnóstico fue propuesto ante el resultado de biopsia intralesional y de esclera, y conformada por la respuesta al tratamiento específico. Discutimos las similitudes y diferencias que esta entidad comparte con el melanoma coroideo, así como la historia natural y el manejo de esta enfermedad

Granulomatosis de Wegener / Wegener's granulomatosis

La granulomatosis de wegener es una vasculitis generalizada con manifestaciones en diversos órganos, entre ellos la piel. El tracto respiratorio es el más afectado. El pronóstico es grave, pero ha mejorado con el tratamiento mediante ciclofosfamida y prednisona. Se presenta un caso con afección cutánea tratado con este régimen

Granulomatosis de wegener: presentación de dos casos

Le presentan dos casos de Granulomatosis de Wegener (WG), de ocurrencia contemporánea en dos Hospitales Universitarios de Bogotá; uno, con desenlace fatal debido a severo compromiso gastrointestinal y otro con manifestación inicial de compromiso ocular y aceptable respuesta al tratamiento. Se discuten las manifestaciones clínicas, la evolución y las imágenes de los principlaes hallazgos, realizándose un comentario final sobre esta entidad de rara ocurrencia.