Linfoma y granulomatosis eosinofílica con poliangeitis (síndrome de Churg-Strauss): reporte de un caso / Lymphoma and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): case report

Diversas etiologías pueden desencadenar a las vasculitis ANCA (anticuerpos anticitoplasma de neutrófilo). Entre ellas se encuentran las neoplasias hematológicas, como los linfomas no Hodgkin, que pueden asociarse con diferentes autoanticuerpos y manifestaciones reumatológicas. Es esencial sospechar estas causas secundarias si la enfermedad tiene un curso crónico con respuesta tórpida al tratamiento. En el presente artículo se reporta un caso inusual de asociación entre granulomatosis eosinofílica con poliangeitis y linfoma no Hodgkin de bajo grado de agresividad.

Diverse etiologies can trigger ANCA (antineutrophil cytoplasmic antibodies) vasculitis. These include hematological neoplasms, such as non-Hodgkin lymphomas, which can be associated with different autoantibodies and rheumatological manifestations. These secondary causes are essential to suspect if the disease has a chronic course with a poor response to treatment. In this article, we report an unusual association between eosinophilic granulomatosis with polyangiitis and low-grade non-Hodgkin lymphoma.

Small vessel vasculitis, different renal outcomes in pediatric patients. Case reports

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.

Clinical characteristics and prognostic factors in an argentinian cohort with ANCA-associated vasculitis / Características clínicas y factores pronósticos en una cohorte argentina de vasculitis asociada a ANCA

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

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

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

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.

Manifestaciones imagenológicas pulmonares y abdominales de las vasculitis primarias / Imaging of Pulmonary and Abdominal Manifestations of Primary Vasculitis

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

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

Eosinophilic Annular Erythema in a Patient with Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) / 대한피부과학회지

No abstract available.

Rare Manifestations of Churg-Strauss Syndrome with Mediastinal and Hilar Lymphadenopathies: Report of an Autopsy Case

No abstract available.

Eosinophilic Granulomatosis with Polyangiitis: Experiences in Korean Patients

Eosinophilic granulomatosis with polyangiitis (EGPA) is one form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Identical to what has been called Churg-Strauss syndrome, EGPA exhibits both allergic and vasculitis features. EGPA was first described as a syndrome consisting of asthma, fever, eosinophilia, and organ involvement including heart failure, neuropathy, and kidney damage, by Churg and Strauss in 1951. On the basis of the 2012 Chapel Hill Consensus Conferences Nomenclature of Vasculitis, EGPA comprises three typical allergic components, including asthma, peripheral eosinophilia, and eosinophil-rich granuloma of the respiratory tracts. EGPA has three clinical and histological stages. The first is an allergic stage composed of asthma and sinusitis, and the second is an eosinophilic stage characterised by peripheral hypereosinophilia and intra-organ infiltration of eosinophils. The last is a vasculitic stage, including necrotising inflammation of small vessels and end-organ damage. In this review, we describe the classification criteria for EGPA and recommendations for the evaluation and management of EGPA with conventional and newly suggested drugs for EGPA. Also, we discuss a variety of clinical aspects such as predictive values for prognosis and associations with other Th2-mediated diseases and hepatitis B virus.

A pediatric case of eosinophilic granulomatosis with polyangiitis accompanied by heart failure mimicking an asthma attack

Eosinophilic granulomatosis with polyangiitis (EGPA, also known as the Churg-Strauss syndrome) is a disorder characterized by asthma, peripheral eosinophilia and systemic vasculitis. It rarely occurs in children, so that physicians may frequently mistake it for a simple uncontrolled asthma. Since a subsequent cardiac involvement is critical for the prognosis, it is important to suspect EGPA in children with severe, uncontrolled asthma. The cardiac manifestations in EGPA are variable from asymptomatic electrocardiogram abnormalities to pericarditis with pericardial effusion, myocarditis with cardiomyopathy, heart failure, and sudden cardiac death. Although delayed treatment may lead to fatal cardiac complications in EGPA, adequate immune suppression can reverse cardiac impairment. We report a 14-year-old girl with persistent asthma refractory to steroids who was eventually diagnosed with an anti-neutrophil cytoplasmic antibody-negative EGPA.

Síndrome de Churg Strauss como diagnóstico diferencial de poliposis nasosinusal, a propósito de un caso / Churg Strauss Syndrome as a differential diagnosis of nasosinusal poliposis, case report

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.

Isolated Focal Cortical Infarction in a Patient with Churg-Strauss Syndrome

No abstract available.

Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis

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.

A Case of Churg-Strauss Syndrome Presenting Umbilicated Ulcerative Papules / 대한피부과학회지

No abstract available.

Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions.

Diagnosis of Churg-Strauss Syndrome Presented With Neuroendocrine Carcinoma: A Case Report

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis that affect small and medium-sized blood vessels and is accompanied by asthma, eosinophilia, and peripheral neuropathy. This report describes a case of a 52-year-old man who had a history of sinusitis, asthma, and thymus cancer and who had complained of bilateral lower extremity paresthesia and weakness for a month. Peripheral neuropathy was detected by electrodiagnostic studies. Resection of a mediastinal mass, which was diagnosed as thymic neuroendocrine carcinoma, was performed five months before his visit. After thymectomy, peripheral blood tests revealed a gradual increase in eosinophils. Two months after surgery, he was admitted to the hospital for dyspnea, and nodules of focal consolidation were found in his chest X-ray. One month later, pyoderma occurred in the right shin, and the skin biopsy showed extravascular eosinophilic infiltration. He was diagnosed with CSS after thymectomy, and we report a very rare case of CSS presented with thymic neuroendocrine carcinoma.

Eosinophilic Annular Erythema Associated with Churg-Strauss Syndrome

No abstract available.

Afectación cerebral en la granulomatosis de Wegener. Reporte de caso / Brain involvement in Wegener's granulomatosis. Case report

La granulomatosis con poliangeítis (GPA) ­antes denominada granulomatosis de Wegener­ es una enfermedad autoinmunitaria multisistémica poco frecuente, de etiología desconocida. En esta enfermedad el compromiso del sistema nervioso central es muy raro aunque tiene mucha importancia porque se relaciona con períodos refractarios. El diagnóstico diferencial de las manifestaciones cerebrales de la GPA es difícil porque no hay signos radiográficos excluyentes de otras lesiones intra o extra-axiales. Este artículo presenta el caso de un paciente con granulomatosis de Wegener de larga evolución, que debuta con crisis convulsivas y focalidad neurológica secundarias a una lesión intraaxial parietal izquierda. Las características sintomáticas, histopatológicas y radiográficas se describen cuidadosamente y se discute el papel de la cirugía para esclarecer el diagnóstico en este paciente. (AU)

The granulomatosis with polyangiitis (GPA), known before as Wegener's granulomatosis, is an autoimmune multisystemic disease, not so common, of unknown etiology. The compromise of this particular disease to the neurological central system is very rare although it's very important because of its relationship with refractory disease periods. The differential diagnosis of the manifestations of the GPA at cerebral level it's not easy to see because there are not that allows the exclusion of other lesions intra or extra axials. We present the case of the patient with Wegener granulomatosis of long evolution that begins with seizures and motor deficit secondary to a left parietal intra axial mass. The clinical characteristics histopathological and radiological are careful described and the role of surgery in management is discussed. (AU)

Churg-Strauss syndrome that presented with mediastinal lymphadenopathy and calculous cholecystitis

No abstract available.

Eosinophilic granulomatosis with polyangiitis accompanied by rapidly progressive glomerulonephritis

Eosinophilic granulomatosis with polyangitis (EGPA) should be considered in asthmatic patients who present with severe systemic symptoms and eosinophilia. Progressive renal insufficiency can occur during the acute phase of EGPA accompanied by renovascular involvement. A 58-year-old man visited local clinic with complaints of malaise, weight loss, fever, and dyspnea. Eosinophilia was revealed in peripheral blood. Pulmonary function tests were carried out, which yielded decreased lung function with positive bronchodilator response. Kidney and skin biopsies were performed, and histological examination showed acute necrotizing crescentic glomerulonephritis and leukoclastic vasculitis in the skin, which led to a diagnosis of EGPA (Churg-Strauss syndrome) associated with rapidly progressive glomerulonephritis. The patient received pulse steroid therapy with parenteral methylprednisolone, followed by oral prednisolone. Clinical and laboratory findings improved dramatically, and remission was attained rapidly. The patient continued to be in remission for 5 months. Prompt and aggressive treatment with systemic corticosteroids is mandatory to control disease activity and to achieve remission.

Native T1 Mapping Demonstrating Apical Thrombi in Eosinophilic Myocarditis Associated with Churg-Strauss Syndrome

Eosinophilic myocarditis is a disease characterized by eosinophilic infiltration of the myocardium, consisting of acute necrotic stage, thrombotic stage, and fibrotic stage. Although T1 mapping has been increasingly used in various cardiac pathologies, there has been no report of T1 mapping in eosinophilic myocarditis. We report a case of 75-year-old female with eosinophilic myocarditis, whose cardiac magnetic resonance imaging included native T1 mapping, in which apical thrombi were distinctly seen as areas with decreased T1 values, next to areas of inflammation seen as increased T1 value in subendocardium.