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Vasculitis mainly affects the walls of the blood vessels, and is an uncommon disease in the pediatric population. In general, they are classified according to the EULAR / PreS consensus in children and in adults according to the Chapel-Hill consensus conference. ANCA-associated vasculitis (AAV) is part of small-vessel disease and is represented by granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA), and others. The representative renal histopathological findings are focal necrotizing glomerulonephritis with crescents, variable interstitial inflammation, absence of immune complexes, or small deposits of immunoglobulins. Clinically, AAV can manifest with hematuria, proteinuria, high blood pressure, and/or rapidly progressive glomerulonephritis. GPA can severely affect the kidney in 75% of cases. In MPA, renal involvement (75-90%) can be rapid and severe with the possibility of requiring renal replacement therapy in more than half of the patients. Furthermore, up to 25% of patients may have high blood pressure, and the mortality at one year can be up to 85%. In EGPA the renal involvement is usually mild. Three pediatric cases of AAV with different renal outcomes are presented, including the need for renal replacement therapy with the recovery of renal function, kidney transplantation, and death, followed in a fourth level of care institution in Colombia.

Las vasculitis, patologías cuyo hallazgo principal es la afectación de las paredes de los vasos sanguíneos, se presentan de forma infrecuente en la población pediátrica. En general, en niños se clasifican de acuerdo con el consenso de la EULAR/PReS, y en adultos, según la Conferencia de Consenso de Chapel-Hill. Las vasculitis asociadas con ANCA (VAA) hacen parte de las vasculitis de pequeños vasos y están representadas por la granulomatosis con poliangeítis (GPA), la granulomatosis eosinofílica con poliangeítis (EGPA) y la poliangeítis microscópica (PAM), entre otras. A nivel renal, los hallazgos histopatológicos representativos son la glomerulonefritis focal necrotizante con media luna, inflamación intersticial variable, ausencia de complejos inmunes o pequeños depósitos de inmunoglobulinas. Clínicamente, las VAA pueden manifestarse con hematuria, proteinuria, hipertensión arterial o glomerulonefritis rápidamente progresiva. La GPA puede afectar de forma severa el riñón en el 75% de los casos, mientras que, en la PAM, el compromiso renal (75-90%) puede ser rápido y severo con posibilidad de requerir terapia de reemplazo renal en más de la mitad de los pacientes. Además, hasta el 25% de los casos puede tener hipertensión arterial, con una mortalidad a un ario de 85%. En la EGPA, el compromiso renal suele ser leve. Se presentan 3 casos pediátricos de VAA con diferentes desenlaces renales, que incluyen necesidad de terapia de reemplazo renal con recuperación de función renal, trasplante renal y muerte, seguidas en una institución de IV nivel del suroccidente colombiano.

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Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss Syndrome, is a rare pathology that belongs to the group of diseases characterized by necrotizing vasculitis of small and medium-sized systemic blood vessels. Symptomatic cardiovascular involvement occurs in 27% to 47% of Churg-Strauss cases, being one of the most serious manifestations. The diagnosis is usually confirmed with tissue biopsy showing eosinophil infiltration, but in selected cases with the recent inclusion of cardiac MRI, we can dispense with it. Early diagnosis is important because early treatment is usually associated with improvement in the condition.

La granulomatosis eosinofílica con poliangeítis, anteriormente conocida como síndrome de Churg-Strauss, es una patología poco frecuente que pertenece al grupo de enfermedades caracterizadas por vasculitis necrotizante de vasos sanguíneos sistémicos de pequeño y mediano calibre. La afectación cardiovascular sintomática ocurre entre un 27% a un 47% de los casos de Churg-Strauss, siendo una de las manifestaciones más graves. El diagnóstico suele confirmarse con biopsia de tejido con infiltración de eosinófilos, pero con la reciente inclusión de la resonancia cardíaca, podemos prescindir de ella. El diagnóstico precoz es importante debido a que el tratamiento oportuno suele asociarse con mejoría del cuadro.

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This article reviews the pulmonary manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Its frequency in the different phenotypes of the disease, clinical manifestations and updated therapeutic recommendations are reviewed, aiming to alert the medical community about the existence of these diseases. We pretend to stimulate a timely suspicion, diagnostic precision, and the implementation of effective therapies, to reduce the eventual sequelae derived from a diagnostic omission or an inappropriate treatment for the different clinical scenarios in which these diseases appear.

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La granulomatosis eosinofílica con poliangitis (GEPA), es un trastorno poco frecuente en la edad pediátrica, representando menos de un 2% de las vasculitis en pediatría. Su etiología es desconocida y de difícil diagnóstico debido a la variabilidad en su cuadro clínico. Los pacientes presentan como rasgo característico antecedentes de asma, rinitis alérgica y eosinofilia en sangre periférica. A nivel microscópico es una vasculitis necrosante que afecta a vasos de pequeño a mediano calibre, presencia de granulomas y eosinofilia extravascular. La GEPA es una vasculitis asociada a la presencia de anticuerpos anticitoplasma de neutrófilo (ANCA), su positividad en niños sólo se encuentran en menos del 40% de los casos. Presentamos el caso de una escolar de 8 años con cuadros respiratorios recurrentes asociados a eosinofilia superior al 10%, manifestaciones cutáneas importantes, esofagitis eosinofílica y polineuropatía, finalmente diagnosticado con GEPA-ANCA-PR3 (ANCA específico para proteinasa 3).

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disorder in pediatric age, accounting for less than 2% of vasculitis in pediatrics. Its etiology is unknown and difficult to diagnose due to the variability in its clinical picture. Patients have a history of asthma, allergic rhinitis and eosinophilia in peripheral blood as a characteristic feature. Microscopically, necrotizing vasculitis that affects small to medium-caliber vessels, the presence of granulomas and extravascular eosinophilia. GEPA is a vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA), its positivity in children is only found in less than 40% of cases. We present the case of an 8-year-old schoolgirl with recurrent respiratory symptoms associated with eosinophilia greater than 10%, important skin manifestations, eosinophilic esophagitis and polyneuropathy, finally diagnosed with GEPA-ANCA-PR3 (specific ANCA for proteinase 3).

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Abstract Vasculitis is a group of several clinical conditions in which the main histopathological finding is fibrinoid necrosis in the walls of blood vessels. This article assesses the main dermatological aspects relevant to the clinical and laboratory diagnosis of small- and medium-vessel cutaneous and systemic vasculitis syndromes. The most important aspects of treatment are also discussed.

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Vasculitis is a group of several clinical conditions in which the main histopathological finding is fibrinoid necrosis in the walls of blood vessels. This article assesses the main dermatological aspects relevant to the clinical and laboratory diagnosis of small- and medium-vessel cutaneous and systemic vasculitis syndromes. The most important aspects of treatment are also discussed.

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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.

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BACKGROUND: In a recent phase III trial (NCT02020889) 53% of mepolizumab-treated versus 19% of placebo-treated patients with eosinophilic granulomatosis with polyangiitis (EGPA) achieved protocol-defined remission. OBJECTIVE: We sought to investigate post hoc the clinical benefit of mepolizumab in patients with EGPA using a comprehensive definition of benefit encompassing remission, oral glucocorticoid (OGC) dose reduction, and EGPA relapses. METHODS: The randomized, placebo-controlled, double-blind, parallel-group trial recruited patients with relapsing/refractory EGPA receiving stable OGCs (prednisolone/prednisone, ≥7.5-50 mg/d) for 4 or more weeks. Patients received 300 mg of subcutaneous mepolizumab or placebo every 4 weeks for 52 weeks. Clinical benefit was defined post hoc as follows: remission at any time (2 definitions used), 50% or greater OGC dose reduction during weeks 48 to 52, or no EGPA relapses. The 2 remission definitions were Birmingham Vasculitis Activity Score of 0 plus OGC dose of 4 mg/d or less (remission 1/clinical benefit 1) or 7.5 mg/d or less (remission 2/clinical benefit 2). Clinical benefit was assessed in all patients and among subgroups with a baseline blood eosinophil count of less than 150 cells/µL, baseline OGC dosage of greater than 20 mg/d, or weight of greater than 85 kg. RESULTS: With mepolizumab versus placebo, 78% versus 32% of patients experienced clinical benefit 1, and 87% versus 53% of patients experienced clinical benefit 2 (both P < .001). Significantly more patients experienced clinical benefit 1 with mepolizumab versus placebo in the blood eosinophil count less than 150 cells/µL subgroup (72% vs 43%, P = .033) and weight greater than 85 kg subgroup (68% vs 23%, P = .005); in the OGC greater than 20 mg/d subgroup, results were not significant but favored mepolizumab (60% vs 36%, P = .395). CONCLUSION: When a comprehensive definition of clinical benefit was applied to data from a randomized controlled trial, 78% to 87% of patients with EGPA experienced benefit with mepolizumab.

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The authors present a classic case of Churg-Strauss syndrome with an exuberant clinical picture in a 34-year-old woman. She showed the following diagnostic criteria: asthma, polyneuropathy, rhinopathy, marked eosinophilia, positive p-ANCA with a perinuclear pattern, and skin histopathology results suggestive of vasculitis with eosinophils. There was a good response to prednisone, dexamethasone pulse therapy, and cyclophosphamide.

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RESUMEN El síndrome de Churg-Strauss (SCS) es una vasculitis necrotizante sistémica de etiología desconocida, que afecta vasos sanguíneos de pequeño y mediano tamaño. Característicamente se definen tres estadios: prodrómico, hipereosinofílico y una etapa de vasculitis sistémica. Dentro del prodrómico se encuentran manifestaciones del área otorrinolaringológica, que suelen preceder por años a las manifestaciones propias de las vasculitis. Entre ellas destacan la otitis media crónica, rinitis alérgica y rinosinusitis crónica poliposa. Reportamos el caso de un paciente de sexo masculino de 64 años con poliposis nasosinusal bilateral, con biopsia compatible con SCS, encontrándose en estado prodrómico de la enfermedad. Además, presentamos una revisión de la literatura sobre esta patología.

ABSTRACT Churg-Strauss syndrome (CSS) is a necrotizing systemic vasculitis of unknown etiology, which involves small and medium-sized vessels. It is characterized by three stages: prodromic, hypereosinophilic and systemic vasculitis. In the prodromal stage we can find otolaryngological manifestations (including chronic otitis media, allergic rhinitis, chronic rhinosinusitis with polyps) that usually precede the proper manifestations of the vasculitis by years. Here, we report a 64-year old male patient with sinonasal polyps and biopsy compatible with CSS, being in the prodromal stage of the illness. Also, we present a review of this disease.

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Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab. Rituximab is associated with a good response in the treatment of vasculitis, but a variable response for the control of allergic symptoms. Here, we report a 16-year-old female patient with severe EGPA (gastrointestinal and cutaneous vasculitis, rhinitis and asthma) refractory to conventional treatment. She was treated with rituximab, which enabled rapid control of the vasculitis component of the disease, but there was no response to rhinitis and asthma. Additionally, she developed severe bronchospasm during rituximab infusion. Sequential rituximab and omalizumab were initiated, leading to remission of all manifestations of vasculitis, rhinitis, and asthma, in addition to bronchospasm related to rituximab infusion.

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BACKGROUND: The anti-neutrophil cytoplasmic antibody-associated (ANCA) vasculitides includes granulomatosis with polyangiitis (Wegener's) (GPW), Chrug-Strauss syndrome (CSS) and microscopic polyangiitis (MPA). Since it has low incidence in our field, there are a few published papers. The aim of this study was to report the clinical characteristics, activity and damage of these vasculitides in Mexico City's metropolitan eastern area. METHODS: We studied 28 cases with ANCA-associated vasculitis in the aforementioned area. The classification was made according to the ACR 1990 criteria, the Chapel Hill 2012 consensus, and the EULAR 2007 recommendations. We collected demographic data, as well as data of clinical manifestations, laboratory, management and outcomes. The activity was established using the Birmingham Vasculitis Activity Score (BVAS) and the damage using the Vasculitis Damage Index (VDI). We used paired t test and 95% confidence intervals (95% CI). RESULTS: 15 females and 13 males were included, 20 with GPW and 8 with CCS. The mean age was 50.1 ± 12.7 years, and the evolution time was 69 ± 49.6 months. We didn't find cases of MAP. 23 patients (82.1%) had general symptoms. The initial and final BVAS means were 10.5 and 1.5 (p < 0.001) and the global VDI was 2.4. CONCLUSIONS: The presence of ANCA-associated vasculitides is rare in the eastern Metropolitan area of Mexico City. GPW was predominant over CCS, and MPA was absent. They are multi-system vasculitides with high scores of initial activity and moderate damage.

Introducción: las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilo (ANCA) incluyen la granulomatosis con poliangeítis (GPW), el síndrome de Churg-Strauss (SChS) y la poliangeítis microscópica (PAM). Como son de baja incidencia, los informes son escasos. El objetivo fue reportar las características clínicas, la actividad y el daño de casos con estas vasculitis en la zona oriente de la Ciudad de México. Métodos: Se incluyeron 28 casos. La clasificación de las vasculitis se hizo según los criterios ACR 1990, Chapel Hill 2012 y EULAR 2007. Se recabaron datos demográficos, manifestaciones clínicas, de laboratorio, tratamientos y evolución clínica. Se determinó el puntaje de actividad de Birmingham (BVAS) y el daño de la vasculitis (VDI). Se utilizó la prueba t pareada e intervalos de confianza al 95% (IC 95%). Resultados: 15 mujeres y 13 hombres fueron incluidos, 20 con GPW y 8 con SChS, con edad de 50.1 ± 12.7 años y evolución de 69 ± 49.6 meses. No hubo casos con PAM. Presentaron ataque al estado general 23 (82.1%). Los promedios BVAS inicial y final fueron: 10.5 y 1.5 (p < 0.001) y el VDI global fue de 2.4. Conclusiones: las vasculitis asociadas a ANCA son raras en esta zona de la Ciudad de México. Predominaron los casos de GPW sobre SChS y fueron nulos para PAM. Tienen presentación multisistémica, altos puntajes de actividad inicial y daño moderado.

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Abstract Churg-Strauss syndrome is a rare systemic vasculitis characterized by asthma and other allergy symptoms as well as eosinophilia and necrotizing vasculitis involving small and medium-sized vessels. Its prevalence in the general population ranges from 1-3 cases per million a year, varying according to the population studied. The authors describe a case of a female patient affected by the disease with important systemic manifestations and not very florid skin lesions.

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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.

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BACKGROUND: Churg-Strauss is a rare, idiopathic, hypereosinophilic disease characterized by blood, tissue, and systemic vasculitis in patients with a history of asthma or allergic rhinitis. Gastrointestinal manifestations of Churg Strauss appear in a 31-45% of the patients according to some series of studies, abdominal pain being the most frequent symptom followed by diarrhea and bleeding. CLINICAL CASE: Male patient with a history of asthma who presents abdominal pain apparently due to acute appendicitis. During the hospital stay the study protocol is complemented, confirmating the diagnosis of Churg Strauss syndrome with intestinal manifestations. CONCLUSION: Churg Strauss syndrome is a rare vasculitis that may present with intestinal manifestations so it is important to take into consideration the differential diagnosis. There are few cases in the literature associated with this syndrome and acute abdomen, all of them on the poor prognosis of this association.

Antecedentes: la enfermedad de Churg-Strauss es poco común, idiopática, caracterizada por hipereosinofilia en sangre y tejidos, aunada a vasculitis sistémica en pacientes con antecedentes de asma o rinitis alérgica. Las manifestaciones gastrointestinales del síndrome de Churg Strauss se caracterizan por dolor abdominal, seguido de diarrea y hemorragia en 31-45% de los casos. Caso clínico: paciente masculino con antecedente de asma que acudió a consulta por abdomen agudo con probable apendicitis aguda; durante el protocolo de estudio se diagnosticó síndrome de Churg Strauss, con manifestaciones intestinales. Conclusión: el síndrome de Churg Strauss es una vasculitis poco frecuente que puede manifestarse con síntomas intestinales, como en este caso; es importante tenerlo en mente a la hora de los diagnósticos diferenciales. Existen pocos reportes con este síndrome asociado con abdomen agudo, todos ellos con mal pronóstico.

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A granulomatose eosinofílica com poliangiite é uma vasculite primária rara, caracterizada por hipereosinofilia, asma de surgimento tardio e granulomas eosinofílicos extravasculares. Relatamos um caso apresentado inicialmente com infarto do miocárdio e que, ulteriormente, teve prosseguimento apenas com asma, manifestações cutâneas e neuropatia periférica. Os parâmetros laboratoriais revelaram hipereosinofilia com um padrão perinuclear negativo de autoanticorpos citoplásmicos antineutrófilos (p-ANCA). A biópsia de pele demonstrou vasculite leucocitoclástica com infiltração eosinofílica, diante de uma angiografia coronária normal. Os sintomas do paciente melhoraram com metilprednisolona IV, pulsoterapia com ciclofosfamida e azatioprina.

Eosinophilic granulomatosis with polyangiitis is a rare primary vasculitic disease characterized by hypereosinophilia, late onset asthma and extravascular eosinophil granulomas. We report a case presented initially with acute myocardial infarction which later only proceed with asthma, skin manifestations and peripheral neuropathy. Laboratory parameters showed hypereosinohpilia with negative perinuclear pattern of antineutrophil cytoplasmic autoantibodies (p-ANCA). Skin biopsy showed leucocytoclastic vasculitis with eosinophilic infiltration while coronary angiography was normal. The patient's symptoms improved with IV methylprednisolone, pulse cyclophosphamide and azathioprine.

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Propósito de la revisión: revisar la literatura actual sobre el síndrome hipereosinofílico y establecercuáles son las diferencias y semejanzas con el síndrome de Churg-Strauss. Fuente de datos: revisión estructurada de la literatura médica, en las bases de datos Pubmed, Medline y Cochrane. Selección de estudios: se limitó la búsqueda a los artículos de revisión, guías de manejo y meta-análisis, sin límites en idioma ni fecha de publicación, utilizando los términos MESH: hypereosinophilic síndrome, eosinophilic, Churg-Strauss sindrome. Se acoplaron a términos como: update, review, associations. Extracción de datos: se hizo la lectura del resumen de 232 trabajos de los cuales se seleccionaron 111 que eran representativos para el tema de esta revisión, después de una clasificación y ordenamiento, se procedió a la redacción del texto. Conclusiones: el síndrome hipereosinofílico comprende un grupo heterogéneo de enfermedades con presentaciones clínicas muy variadas, para algunos autores el síndrome de Churg-Strauss o más recientemente conocido como granulomatosis eosinofílica con poliangiítis hace parte de este gran síndrome pero para otros, deben considerarse como entidades aparte. (Acta Med Colomb 2014; 39: 174-184).

Purpose of review: to review the current literature on the hypereosinophilic syndrome and establish which are the differences and similarities with Churg-Strauss syndrome. Source of data: structured review of the medical literature in databases Pubmed, Medline and Cochrane. Study selection: the search was limited to review articles, management guidelines and meta-analysis with no limits on language or publication date, using the MESH terms: hypereosinophilic syndrome, eosinophilic, Churg-Strauss syndrome. These were coupled to terms like:update, review, association. Data extraction: reading of the summary of 232 studies was made. Of these, 111 that were representative for the subject of this review, were selected, and after an ordered classification system, the wording was performed. Conclusions: The hypereosinophilic syndrome comprises a heterogeneous group of diseases with varied clinical presentations; for some authors, Churg-Strauss, more recently known as eosinophilic granulomatosis with polyangiitis, is part of this great syndrome, but for others these should be considered as separate entities. (Acta Med Colomb 2014; 39: 174-184).

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Eosinophilic granulomatosis with polyangiitis is a rare primary vasculitic disease characterized by hypereosinophilia, late onset asthma and extravascular eosinophil granulomas. We report a case presented initially with acute myocardial infarction which later only proceed with asthma, skin manifestations and peripheral neuropathy. Laboratory parameters showed hypereosinohpilia with negative perinuclear pattern of antineutrophil cytoplasmic autoantibodies (p-ANCA). Skin biopsy showed leucocytoclastic vasculitis with eosinophilic infiltration while coronary angiography was normal. The patient's symptoms improved with IV methylprednisolone, pulse cyclophosphamide and azathioprine.

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As vasculites são um conjunto de entidades clínico-patológicas sistêmicas caracterizadas pela destruição da parede de vasos sanguíneos. O parênquima pulmonar é consistentemente mais afetado por três vasculites em particular: granulomatose de Wegener (ou granulomatose com poliangite), síndrome de Churg-Strauss e poliangite microscópica. Demograficamente, estas doenças inflamatórias afetam adultos de meia idade, e apresentam-se com sinais e sintomas respiratórios, além de sintomas sistêmicos. O diagnósico destas entidades recais na correlação da apresentação clínica e correlação desta com achados radiológicos e morfológicos. O diagnóstico diferencial das três vasculites pulmonares mais comuns é principalmente com infecções granulomatosas, como micobacterioses e infecções fúngicas, linfomas e outras vasculites. Este artigo explora as principais características histopatológicas e o diagnóstico diferencial destas entidades

Vasculitides are a group of systemic diseases characterized by the destruction of the blood vessels walls. The pulmonary parenchyma is consistently affected by three vasculidites in particular, namely, Wegener granulomatosis (or granulomatosis with polyangeitis), Churg-Strauss syndrome and microscopic polyangitis. Demographically toms. The diagnosis of this group of diseases is based on the correlation of the clinical picture with radiologic and morphologic findings. Differential diagnosis is mostly done with granulomatous infectious, such as mycobacteria and fungus, lymphomas and other vasculidites. This article explores the main histopathologic characteristics of these vasculidites as well as their main differential diagnoses

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ANCA (anti-neutrophil cytoplasmic antibodies) associated vasculitis or AAVare a group of diseases with predominant inflammation of small vessels and the presence of detectable ANCA in serum. Due to these common features, it is considered that AAV share pathogenic mechanisms. Consequently, a similar therapeutic approach has been developed. A new nomenclature has been recently proposed, with AAV including "granulomatosis with polyangiitis" (GPA, formerly Wegener's granulomatosis), "microscopic polyangiitis" (MPA), "eosinophilic granulomatosis with polyangiitis" (EGPA, formerly Churg-Strauss disease) and renal limited vasculitis. Research on AAV has shown significant advances in the last two decades, including advances in the knowledge of pathogenic mechanisms such as the pro-inflammatory role of ANCA, AAV murine models and genetic links. Evidence on previous immunosuppressive therapies has improved significantly and new, promissory drugs have been introduced, Rituximab being the most important. We review the advances of pathogenic mechanisms and treatment for these diseases.

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