Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/tratamiento farmacológico , Inhibidores de las Cinasas Janus/uso terapéutico , Piperidinas/uso terapéutico , Pirimidinas/uso terapéutico , Adulto , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/metabolismo , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/fisiopatología , Sedimentación Sanguínea , Proteína C-Reactiva/metabolismo , Síndrome de Churg-Strauss/tratamiento farmacológico , Síndrome de Churg-Strauss/metabolismo , Síndrome de Churg-Strauss/fisiopatología , Femenino , Granulomatosis con Poliangitis/tratamiento farmacológico , Granulomatosis con Poliangitis/metabolismo , Granulomatosis con Poliangitis/fisiopatología , Humanos , Masculino , Poliangitis Microscópica/tratamiento farmacológico , Poliangitis Microscópica/metabolismo , Poliangitis Microscópica/fisiopatología , Persona de Mediana Edad , Proyectos Piloto , Resultado del Tratamiento , Adulto JovenAsunto(s)
Alérgenos/inmunología , Asma/inmunología , Síndrome de Churg-Strauss/inmunología , Inmunoglobulina E/inmunología , Enfermedades Otorrinolaringológicas/inmunología , Adulto , Anciano , Asma/fisiopatología , Estudios de Casos y Controles , Síndrome de Churg-Strauss/fisiopatología , Femenino , Granulomatosis con Poliangitis/inmunología , Humanos , Masculino , Análisis por Micromatrices , Poliangitis Microscópica/inmunología , Persona de Mediana Edad , Enfermedades Otorrinolaringológicas/fisiopatologíaRESUMEN
The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). The SCMR web site ( https://www.scmr.org ) hosts a case series designed to present case reports demonstrating the unique attributes of CMR in the diagnosis or management of cardiovascular disease. Each clinical presentation is followed by a brief discussion of the disease and unique role of CMR in disease diagnosis or management guidance. By nature, some of these are somewhat esoteric, but all are instructive. In this publication, we provide a digital archive of the 2019 Case of the Week series as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.
Asunto(s)
Síndrome de Churg-Strauss/diagnóstico por imagen , Imagen por Resonancia Magnética , Trombosis/diagnóstico por imagen , Disfunción Ventricular Izquierda/diagnóstico por imagen , Antineoplásicos/efectos adversos , Cardiotoxicidad , Síndrome de Churg-Strauss/fisiopatología , Síndrome de Churg-Strauss/terapia , Diagnóstico Diferencial , Femenino , Neoplasias Cardíacas/diagnóstico por imagen , Humanos , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Trombosis/fisiopatología , Trombosis/terapia , Disfunción Ventricular Izquierda/inducido químicamente , Disfunción Ventricular Izquierda/fisiopatología , Disfunción Ventricular Izquierda/terapia , Función Ventricular Izquierda/efectos de los fármacos , Adulto JovenRESUMEN
Polyangiitis overlap syndrome (POS) is a diagnostic term coined by Leavitt and Fauci that characterises patients with overlapping features of more than one vasculitis. Prior case studies of antineutrophil cytoplasmic antibodies (ANCA)-associated POS have only been published in patients with eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis alongside proteinase-3/cytoplasmic (C)-ANCA positivity. We present a case of a 60-year-old woman with dyspnoea, hemoptysis, positive perinuclear-ANCA and renal biopsy demonstrating evidence of microscopic polyangiitis. In addition, our patient also had asthma, mononeuritis multiplex, eosinophilia and migratory pulmonary infiltrates, thus fulfilling the criteria for EGPA. This novel case report suggests that POS is not limited to C-ANCA positivity and has variable presentations.
Asunto(s)
Síndrome de Churg-Strauss/diagnóstico , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Poliangitis Microscópica/diagnóstico , Mononeuropatías/fisiopatología , Insuficiencia Renal Crónica/fisiopatología , Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Azatioprina/uso terapéutico , Síndrome de Churg-Strauss/tratamiento farmacológico , Síndrome de Churg-Strauss/inmunología , Síndrome de Churg-Strauss/fisiopatología , Ciclofosfamida/uso terapéutico , Femenino , Humanos , Inmunosupresores/uso terapéutico , Poliangitis Microscópica/tratamiento farmacológico , Poliangitis Microscópica/inmunología , Poliangitis Microscópica/fisiopatología , Persona de Mediana Edad , Peroxidasa/inmunología , Prednisona/uso terapéutico , Insuficiencia Renal Crónica/patología , Tomografía Computarizada por Rayos XAsunto(s)
Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Síndrome de Churg-Strauss/inmunología , Interleucina-5/inmunología , Mononeuropatías/inmunología , Polineuropatías/inmunología , Anciano , Biopsia , Síndrome de Churg-Strauss/patología , Síndrome de Churg-Strauss/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Mononeuropatías/patología , Mononeuropatías/fisiopatología , Mieloblastina/inmunología , Fibras Nerviosas Mielínicas/patología , Peroxidasa/inmunología , Polineuropatías/patología , Polineuropatías/fisiopatología , Estudios Retrospectivos , Nervio Sural/patologíaRESUMEN
OBJECTIVE: To describe the efficacy and safety of biologics for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: A retrospective European collaborative study was conducted in patients with EGPA who received treatment with biologics for refractory and/or relapsing disease. RESULTS: Among the 147 patients with EGPA included in the study, 63 received rituximab (RTX), 51 received mepolizumab (MEPO), and 33 received omalizumab (OMA). At the time of inclusion, the median Birmingham Vasculitis Activity Score (BVAS) was 8.5 (interquartile range [IQR] 5-13) in the RTX group, while the median BVAS in the OMA group was 2 (IQR 1-4.5) and the median BVAS in the MEPO group was 2 (IQR 1-5). In patients receiving RTX, the median BVAS declined both at 6 months (median 1, IQR 0-4.5) and at 12 months (median 0, IQR 0-2), and the frequency of remission, partial response, treatment failure, and stopping treatment due to adverse events was 49%, 24%, 24%, and 3%, respectively. For the treatment of glucocorticoid (GC)-dependent asthma, patients who received MEPO had a much better GC-sparing effect and overall response than did patients who received OMA. The frequency of remission, partial response, treatment failure, and stopping treatment due to adverse events was 15%, 33%, 48%, and 4%, respectively, in the OMA group and 78%, 10%, 8%, and 4%, respectively, in the MEPO group. Remission rates at 12 months were 76% and 82% among patients receiving MEPO at a doses of 100 mg and 300 mg, respectively. CONCLUSION: These results suggest that RTX could be effective in treating relapses of EGPA vasculitis. MEPO is highly effective with a good safety profile in patients with GC-dependent asthma. Our data suggest that 100 mg MEPO monthly could be an acceptable dosage for first-line therapy in selected instances of EGPA, recognizing, however, that this has not been compared to the validated dosage of 300 mg monthly.
Asunto(s)
Asma/tratamiento farmacológico , Productos Biológicos/uso terapéutico , Síndrome de Churg-Strauss/tratamiento farmacológico , Factores Inmunológicos/uso terapéutico , Adulto , Anciano , Anticuerpos Monoclonales Humanizados/uso terapéutico , Asma/fisiopatología , Síndrome de Churg-Strauss/fisiopatología , Femenino , Glucocorticoides/uso terapéutico , Humanos , Masculino , Persona de Mediana Edad , Omalizumab/uso terapéutico , Recurrencia , Estudios Retrospectivos , Rituximab/uso terapéutico , Insuficiencia del Tratamiento , Resultado del TratamientoRESUMEN
OBJECTIVES: ANCA-associated vasculitis (AAV) usually involves the renal and respiratory systems, but the paediatric literature on pulmonary manifestations and outcomes is limited. We aimed to describe pulmonary manifestations and outcomes after therapy in a cohort of paediatric AAV (pAAV) patients. METHODS: A retrospective chart review of all patients <19 years presenting to our institution with AAV between 1/2008 and 2/2018 was conducted. Patient demographics, clinical presentation, diagnostic testing, therapy and pulmonary outcomes over the first 3 years after presentation were evaluated. RESULTS: A total of 38 patients were included; all had ANCA positivity by immunofluorescence. A total of 23 had microscopic polyangiitis (MPA), 13 had granulomatosis with polyangiitis and 2 had eosinophilic granulomatosis with polyangiitis. A total of 30 (79%) had pulmonary manifestations, with cough (73%) and pulmonary haemorrhage (67%) being the most common. Abnormalities were noted in 82% of chest CT scans reviewed, with nodules and ground-glass opacities being the most common. At 6, 12 and 36 months follow-up, respectively, 61.8%, 39.4% and 29% of patients continued to show pulmonary manifestations. Five MPA patients with re-haemorrhage are described in detail. CONCLUSION: MPA was more common than granulomatosis with polyangiitis, with pulmonary involvement being common in both. MPA patients had more severe pulmonary manifestations. Chest CT revealed abnormal findings in a majority of cases. A subgroup of young MPA patients experienced repeat pulmonary haemorrhage. Treatment modality and response were comparable in different subtypes of AAV, except for this young MPA group. Additional prospective studies are needed to better understand the different phenotypes of pAAV.
Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/fisiopatología , Tos/fisiopatología , Hemoptisis/fisiopatología , Hemorragia/fisiopatología , Enfermedades Pulmonares/fisiopatología , Nódulos Pulmonares Múltiples/fisiopatología , Adolescente , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/inmunología , Autoanticuerpos/inmunología , Niño , Preescolar , Síndrome de Churg-Strauss/inmunología , Síndrome de Churg-Strauss/fisiopatología , Estudios de Cohortes , Progresión de la Enfermedad , Femenino , Granulomatosis con Poliangitis/inmunología , Granulomatosis con Poliangitis/fisiopatología , Hemoptisis/inmunología , Hemorragia/inmunología , Humanos , Lactante , Enfermedades Pulmonares/diagnóstico por imagen , Enfermedades Pulmonares/inmunología , Masculino , Poliangitis Microscópica/inmunología , Poliangitis Microscópica/fisiopatología , Nódulos Pulmonares Múltiples/diagnóstico por imagen , Mieloblastina/inmunología , Peroxidasa/inmunología , Recurrencia , Estudios Retrospectivos , Tomografía Computarizada por Rayos XRESUMEN
Patients with eosinophilic granulomatosis with polyangiitis (EGPA) most commonly die from cardiac causes, however, cardiac involvement remains poorly characterised and the relationship between cardiac and pulmonary disease is not known. This study aimed to characterise myocardial and pulmonary manifestations of EGPA, and their relationship. Prospective comprehensive cardiopulmonary investigation, including a novel combined cardiopulmonary magnetic resonance imaging (MRI) technology, was performed in 13 patients with stable EGPA. Comparison was made with 11 prospectively recruited matched healthy volunteers. Stable EGPA was associated with focal replacement and diffuse interstitial myocardial fibrosis (myocardial extracellular volume 26.9% vs. 24.7%; p = 0.034), which drove a borderline increase in left ventricular mass (56 ± 9 g/m2 vs. 49 ± 8 g/m2; p = 0.065). Corrected QT interval was significantly prolonged and was associated with the severity of myocardial fibrosis (r = 0.582, p = 0.037). Stable EGPA was not associated with increased myocardial capillary permeability or myocardial oedema. Pulmonary tissue perfusion and capillary permeability were normal and there was no evidence of pulmonary tissue oedema or fibrosis. Forced expiratory volume in one second showed a strong inverse relationship with myocardial fibrosis (r = -0.783, p = 0.038). In this exploratory study, stable EGPA was associated with focal replacement and diffuse interstitial myocardial fibrosis, but no evidence of myocardial or pulmonary inflammation or pulmonary fibrosis. Myocardial fibrosis was strongly associated with airway obstruction and abnormal cardiac repolarisation. Further investigation is required to determine the mechanisms underlying the association between heart and lung disease in EGPA and whether an immediate immunosuppressive strategy could prevent myocardial fibrosis formation.
Asunto(s)
Obstrucción de las Vías Aéreas/diagnóstico por imagen , Cardiomiopatías/diagnóstico por imagen , Síndrome de Churg-Strauss/diagnóstico por imagen , Enfermedades Pulmonares/diagnóstico por imagen , Pulmón/diagnóstico por imagen , Imagen por Resonancia Cinemagnética , Obstrucción de las Vías Aéreas/fisiopatología , Cardiomiopatías/patología , Cardiomiopatías/fisiopatología , Estudios de Casos y Controles , Síndrome de Churg-Strauss/patología , Síndrome de Churg-Strauss/fisiopatología , Femenino , Fibrosis , Humanos , Pulmón/fisiopatología , Enfermedades Pulmonares/fisiopatología , Masculino , Persona de Mediana Edad , Miocardio/patología , Valor Predictivo de las Pruebas , Pronóstico , Estudios ProspectivosAsunto(s)
Síndrome de Churg-Strauss/genética , Receptores de IgG/genética , Estudios de Casos y Controles , Síndrome de Churg-Strauss/fisiopatología , Supervivencia sin Enfermedad , Proteínas Ligadas a GPI/genética , Haplotipos , Humanos , Polimorfismo de Nucleótido Simple , Pronóstico , RecurrenciaRESUMEN
Eosinophilic granulomatosis with polyangitis (EGPA) is a systemic necrotizing small-vessel vasculitis that presents heterogeneously as a multi-organ disease. EGPA evolves through three phases: (1) prodromic phase with asthma, atopy and sinusitis, (2) eosinophilic phase characterized by peripheral eosinophilia and eosinophilic infiltration without necrosis, and (3) vasculitic phase involving organ damage. EGPA often presents with asthma, mononeuritis multiplex, lung infiltrates, sinusitis and constitutional symptoms. Although myalgias are common, EGPA rarely presents with true weakness with elevated creatinine kinase (CK). We describe a rare case of a patient presenting with eosinophilic myositis, who subsequently developed fulminant EGPA. The patient's diagnosis was supported by an initial clinical presentation of weakness and elevated CK, followed by fleeting pulmonary infiltrates and mononeuritis multiplex, peripheral eosinophilia, and strongly positive myeloperoxidase anti-cytoplasmic antibody (MPO-ANCA). Muscle biopsy revealed eosinophilic myositis. The patient responded well to high-dose glucocorticoids and cyclophosphamide with improved symptoms and biochemical markers. Based on our literature review, there are only seven similar cases reported of EGPA presenting with myositis and confirmatory muscle biopsies. There is significant heterogeneity in their clinical findings, histopathology and treatments that were used. Our case report and literature review highlights the importance of recognizing myositis as an initial presenting symptom of EGPA, providing an opportunity for early diagnosis and treatment to reduce risk of further disease progression and morbidity.
Asunto(s)
Síndrome de Churg-Strauss/fisiopatología , Mononeuropatías/fisiopatología , Miositis/fisiopatología , Anciano de 80 o más Años , Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Antirreumáticos/uso terapéutico , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/tratamiento farmacológico , Síndrome de Churg-Strauss/inmunología , Ciclofosfamida/uso terapéutico , Eosinofilia/tratamiento farmacológico , Eosinofilia/inmunología , Femenino , Glucocorticoides/uso terapéutico , Humanos , Mononeuropatías/tratamiento farmacológico , Mononeuropatías/inmunología , Miositis/tratamiento farmacológico , Miositis/inmunología , Peroxidasa/inmunología , Resultado del TratamientoRESUMEN
OBJECTIVE: To investigate the clinicopathologic features of eosinophilic granulomatosis with polyangiitis (EGPA)-associated neuropathy with a focus on the presence or absence of anti-neutrophil cytoplasmic antibodies (ANCAs). METHODS: We examined the clinical features and pathologic findings of sural nerve biopsy specimens from 82 patients with EGPA-associated neuropathy. Of these patients, 32.9% were myeloperoxidase (MPO)-ANCA positive, and 67.1% were MPO-ANCA negative. PR3-ANCA was negative in all of 78 examined patients. RESULTS: Upper limb symptoms were more frequently reported as initial neuropathic manifestations in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (44.4% vs 14.6%, p < 0.01). The serum levels of C-reactive protein were significantly higher in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (p < 0.05). Sural nerve biopsy specimens showed findings suggestive of vasculitis (i.e., destruction of vascular structures) in epineurial vessels; these results were seen more frequently in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (p < 0.0001). Conversely, the numbers of eosinophils in the lumen of the epineurial vessels (p < 0.01) and epineurial vessels occluded by intraluminal eosinophils (p < 0.05) were higher in the MPO-ANCA-negative group than in the MPO-ANCA-positive group. Furthermore, the incidence of eosinophil infiltration in the endoneurium was higher in the MPO-ANCA-negative group than in the MPO-ANCA-positive group (p < 0.01). CONCLUSIONS: This study suggests that the pathogenesis of EGPA comprises at least 2 distinct mechanisms: ANCA-associated vasculitis resulting in ischemic effects and inflammation, which is prominent in MPO-ANCA-positive patients, and eosinophil-associated vascular occlusion leading to ischemia and eosinophil-associated tissue damage, which is conspicuous in MPO-ANCA-negative patients.
Asunto(s)
Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Síndrome de Churg-Strauss/fisiopatología , Debilidad Muscular/fisiopatología , Nervios Periféricos/irrigación sanguínea , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Trastornos Somatosensoriales/fisiopatología , Anciano , Asma/etiología , Síndrome de Churg-Strauss/complicaciones , Síndrome de Churg-Strauss/inmunología , Electrodiagnóstico , Femenino , Humanos , Enfermedades Renales/etiología , Extremidad Inferior/inervación , Enfermedades Pulmonares/etiología , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Debilidad Muscular/etiología , Mieloblastina/inmunología , Conducción Nerviosa , Enfermedades Otorrinolaringológicas/genética , Nervios Periféricos/patología , Enfermedades del Sistema Nervioso Periférico/etiología , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/patología , Peroxidasa/inmunología , Enfermedades Cutáneas Vasculares/etiología , Trastornos Somatosensoriales/etiología , Nervio Sural/patología , Tomografía Computarizada por Rayos X , Extremidad Superior/inervaciónAsunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/tratamiento farmacológico , Antirreumáticos/uso terapéutico , Rituximab/uso terapéutico , Agammaglobulinemia/inducido químicamente , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/fisiopatología , Síndrome de Churg-Strauss/tratamiento farmacológico , Síndrome de Churg-Strauss/fisiopatología , Técnica Delphi , Duración de la Terapia , Granulomatosis con Poliangitis/tratamiento farmacológico , Granulomatosis con Poliangitis/fisiopatología , Humanos , Huésped Inmunocomprometido , Vacunas contra la Influenza/uso terapéutico , Gripe Humana/inmunología , Gripe Humana/prevención & control , Quimioterapia de Mantención , Poliangitis Microscópica/tratamiento farmacológico , Poliangitis Microscópica/fisiopatología , Neutropenia/inducido químicamente , Infecciones Neumocócicas/inmunología , Infecciones Neumocócicas/prevención & control , Vacunas Neumococicas/uso terapéutico , Neumonía por Pneumocystis/inmunología , Neumonía por Pneumocystis/prevención & control , Guías de Práctica Clínica como Asunto , Recurrencia , Combinación Trimetoprim y Sulfametoxazol/uso terapéutico , Reino UnidoRESUMEN
The different sets of criteria for diagnosis or classification of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) lead to numerous overlapping and reclassified diagnoses in clinical practice. We designed this study to assess the difficulties in classifying patients with AAV. As a secondary objective, different variables were tested to predict prognosis. We conducted a retrospective chart review in a Western Spain multicentre survey. A total of 115 adult patients diagnosed with AAV from 2002 to 2013 and followed for at least 3 years were included. They were classified according to (1) Chapel Hill Consensus Conference (CHCC), (2) European Medicines Agency algorithm and (3) French Vasculitis Study Group/European Vasculitis Society phenotypes. Fifty-three patients (46%) had neither distinctive histopathological data of a single AAV definition nor any surrogate markers for granulomatous inflammation and thus did not fulfill any diagnostic criteria. Ocular, ear, nose, throat, skin, and lung involvement were more frequent with proteinase 3 (PR3) antibodies, whereas peripheral neuropathy was more frequent with myeloperoxidase (MPO) antibodies. When the disease was severe at diagnosis, the HR for mortality was 10.44. When induction treatment was not given in accordance with the guidelines, the HR for mortality was 4.00. For maintenance treatment, the HR was 5.49 for mortality and 2.48 for relapse. AAV classification is difficult because many patients had neither specific clinical data nor distinctive histological features of a single CHCC definition. A structured clinical assessment of patient severity is the best tool to guide the management of AAV.
Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/clasificación , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/patología , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/fisiopatología , Mortalidad , Anciano , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/inmunología , Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Síndrome de Churg-Strauss/clasificación , Síndrome de Churg-Strauss/inmunología , Síndrome de Churg-Strauss/patología , Síndrome de Churg-Strauss/fisiopatología , Epistaxis/inmunología , Epistaxis/patología , Epistaxis/fisiopatología , Oftalmopatías/inmunología , Oftalmopatías/patología , Oftalmopatías/fisiopatología , Femenino , Enfermedades Gastrointestinales/inmunología , Enfermedades Gastrointestinales/patología , Enfermedades Gastrointestinales/fisiopatología , Granulomatosis con Poliangitis/clasificación , Granulomatosis con Poliangitis/inmunología , Granulomatosis con Poliangitis/patología , Granulomatosis con Poliangitis/fisiopatología , Humanos , Hipertensión/inmunología , Hipertensión/patología , Hipertensión/fisiopatología , Enfermedades Renales/inmunología , Enfermedades Renales/patología , Enfermedades Renales/fisiopatología , Fallo Renal Crónico/fisiopatología , Enfermedades Pulmonares/inmunología , Enfermedades Pulmonares/patología , Enfermedades Pulmonares/fisiopatología , Masculino , Poliangitis Microscópica/clasificación , Poliangitis Microscópica/inmunología , Poliangitis Microscópica/patología , Poliangitis Microscópica/fisiopatología , Persona de Mediana Edad , Mieloblastina/inmunología , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/patología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Peroxidasa/inmunología , Prevención Primaria , Pronóstico , Modelos de Riesgos Proporcionales , Recurrencia , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Sinusitis/inmunologíaRESUMEN
Eosinophilic granulomatosis with polyangiitis (EGPA), or Churg-Strauss syndrome, is a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia. Clinically, it presents in various ways and progresses in three phases: prodromic (asthma and rhino-sinusitis), eosinophilic (peripheral eosinophilia and organ involvement), and vasculitic (clinical manifestations due to small vessel vasculitis). The differential diagnosis of EGPA principally includes eosinophilic and vasculitic disorders, early treatment is needed to avoid a fatal outcome. We present the case report of a 38-year-old male with a history of asthma. After a month-and-a-half of progressive weakness, no fever, diarrhea, vomiting and abdominal pain associated with weight loss, he was diagnosed of intestinal parasitosis. He later died of a massive myocardial necrosis due to EGPA with multiple organs affected.
Asunto(s)
Dolor Abdominal/etiología , Síndrome de Churg-Strauss/diagnóstico , Parasitosis Intestinales/diagnóstico , Adulto , Síndrome de Churg-Strauss/fisiopatología , Diagnóstico Diferencial , Resultado Fatal , Humanos , Masculino , Miocardio/patología , Necrosis/etiología , Pérdida de PesoAsunto(s)
Síndrome de Churg-Strauss , Ciclofosfamida/administración & dosificación , Duramadre , Imagen por Resonancia Magnética/métodos , Meningitis , Prednisolona/administración & dosificación , Adulto , Anticuerpos Anticitoplasma de Neutrófilos/análisis , Síndrome de Churg-Strauss/complicaciones , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/inmunología , Síndrome de Churg-Strauss/fisiopatología , Duramadre/diagnóstico por imagen , Duramadre/patología , Humanos , Hipertrofia , Aumento de la Imagen/métodos , Inmunosupresores/administración & dosificación , Masculino , Meningitis/diagnóstico , Meningitis/tratamiento farmacológico , Meningitis/etiología , Inducción de RemisiónRESUMEN
CASE PRESENTATION: A 56-year-old man was admitted to the ICU with chest pain, cough, hemoptysis, increasing dyspnea, and orthopnea for 1 week. The patient reported an 8-kg weight loss over the last month and recurrent wheezing episodes for approximately 1 year. He had a history of tobacco smoking and excessive alcohol consumption, both of which he stopped 15 years ago. His medical history included high BP treated with amlodipine and an episode of drug-induced angioedema 8 years ago. He had no history of recent travel.
Asunto(s)
Taponamiento Cardíaco , Síndrome de Churg-Strauss , Eosinofilia , Metilprednisolona/administración & dosificación , Pericardio/patología , Biopsia/métodos , Taponamiento Cardíaco/diagnóstico , Taponamiento Cardíaco/etiología , Taponamiento Cardíaco/fisiopatología , Taponamiento Cardíaco/terapia , Síndrome de Churg-Strauss/complicaciones , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/fisiopatología , Disnea/diagnóstico , Disnea/etiología , Electrocardiografía/métodos , Eosinofilia/diagnóstico , Eosinofilia/etiología , Glucocorticoides/administración & dosificación , Hemoptisis/diagnóstico , Hemoptisis/etiología , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Tomografía Computarizada por Rayos X/métodos , Resultado del TratamientoRESUMEN
Ischemic heart disease can be caused by multiple factors. However, epidemiological studies have evidenced an association between hypereosinophilia and acute coronary syndrome, most frequently observed in the Kounis and Churg-Strauss syndromes. We here report the case of a 37-year-old man, who was admitted to our hospital for acute coronary syndrome, complicated by hypokinetic cardiac arrest with severe hypereosinophilia.
Asunto(s)
Síndrome Coronario Agudo/etiología , Síndrome de Churg-Strauss/diagnóstico , Paro Cardíaco/etiología , Síndrome de Kounis/diagnóstico , Adulto , Síndrome de Churg-Strauss/fisiopatología , Eosinofilia/etiología , Humanos , Síndrome de Kounis/fisiopatología , Masculino , Isquemia Miocárdica/etiologíaRESUMEN
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is a systemic necrotizing vasculitis of small- and medium-size vessels, characterized by asthma and blood eosinophilia. EGPA typically occurs in patients with preexisting asthma, and involves the skin, lungs, and peripheral nerves. Poor-prognosis factors (i.e., involvement(s) of the gastrointestinal tract, heart, and/or kidney) have been assessed with the Five-Factor Score (FFS). One-third of the patients have anti-myeloperoxidase antineutrophil cytoplasm antibodies, and their presence seems to differentiate between two phenotypes, with different clinical characteristics and prognoses. Overall survival has improved markedly since the use of glucocorticoids and immunosuppressants, but relapse rates remain high. All patients require glucocorticoids, and for those with severe/refractory disease and FFS-defined poor prognoses, immunosuppressants should be used (cyclophosphamide for induction and azathioprine for maintenance therapy). Recent advances in EGPA management, including several novel immunomodulatory drugs and targeted biotherapies, were or are being evaluated to improve EGPA patients' prognoses.
Asunto(s)
Asma/complicaciones , Síndrome de Churg-Strauss/tratamiento farmacológico , Síndrome de Churg-Strauss/fisiopatología , Inmunosupresores/uso terapéutico , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Síndrome de Churg-Strauss/mortalidad , Ciclofosfamida/uso terapéutico , Glucocorticoides/uso terapéutico , Humanos , Pronóstico , Ensayos Clínicos Controlados Aleatorios como Asunto , RecurrenciaRESUMEN
Objective: To improve the clinical recognition of eosinophilic granulomatosis with polyangiitis(EGPA) in clinical manifestations, diagnosis and treatment. Methods: The clinical manifestations, pathological characteristic, imaging manifestations, diagnosis and the therapy of three patients with EGPA were presented. Results: These 3 patients had asthma-like symptoms and extrapulmonary manifestations of systemic vasculitis. They were 20, 40 and 44 years old. All of them were female.They denied exposure or contact. Chest radiographic examination showed that the most common features were nodule shadow and tree-in-bud in the lung. The pathological manifestation was characterized by hypereosinophilia, high total IgE(over 300 KU/L) and high CRP(over 14.1mg/L). The FeNO of 2 patients was over 100ppb. The ANCA of these 3 patients was negative. The pulmonary pathology was observed had eosinophil infiltration in the alveolar, interstitial and vessel for 3 cases. The clinical manifestations were nonspecific. All patients were treated by glucocorticoid and immune-inhibitor(alkylating agents or purine synthesis inhibitors) therapy. Because patients were complicated with other organs involved, they needed long-time treatment. Conclusions: This disease is diverse and complex, with a lack of pathognomonic symptoms. We should highly suspect eosinophilic granulomatosis with polyangiitis, when the patients present severe asthma and eosinophilia. Early detection, early treatment, and the prognosis could be better.
Asunto(s)
Síndrome de Churg-Strauss/fisiopatología , Eosinofilia/diagnóstico , Granulomatosis con Poliangitis/fisiopatología , Pulmón/patología , Adulto , Asma/etiología , Síndrome de Churg-Strauss/complicaciones , Eosinofilia/sangre , Femenino , Granulomatosis con Poliangitis/complicaciones , Humanos , PronósticoRESUMEN
A 65-year-old lady and a 69-year-old gentleman, both with a background history of adult-onset asthma, presented with clinical features of heart failure (HF). High-sensitivity cardiac troponin T and eosinophils were significantly raised, along with poor left ventricular (LV) systolic function on cardiac imaging. Endocardial and skin biopsy (in cases 1 and 2, respectively) showed eosinophilic infiltration. This in combination with the clinical features confirmed the diagnosis of eosinophilic myocarditis (EM) secondary to eosinophilic granulomatosis with polyangiitis in case 1. Both cases were managed with high-dose intravenous corticosteroids and conventional HF medication. Case 1 successfully responded clinically with improvement in LV systolic function. Case 2 required further immunosuppressive therapy (rituximab) and cardiac resynchronisation therapy, but eventually died of septic shock secondary to immunosuppressives. Our cases highlight the importance of early diagnosis and treatment of EM and ongoing monitoring of patients on immunosuppressive therapy.