RESUMEN
OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) primarily affects small vessels. Large-vessel involvement (LVI) is rare. We aimed to describe the characteristics of LVI, to identify associated risk factors, and to describe its therapeutic management. METHODS: This multicenter case-control (1:2) study included patients with AAV according to the ACR/EULAR classification and LVI as defined by the Chapel Hill nomenclature, together with controls matched for age, sex, and AAV type. RESULTS: We included 26 patients, 15 (58 %) of whom were men, with a mean age of 56.0 ± 17.1 years. The patients had granulomatosis with polyangiitis (n = 20), or microscopic polyangiitis (n = 6). The affected vessels included the aorta (n = 18; 69 %) supra-aortic trunks (n = 9; 35 %), lower-limb arteries (n = 5; 19 %), mesenteric arteries (n = 5; 19 %), renal arteries (n = 4; 15 %), and upper-limb arteries (n = 2; 8 %). Imaging showed wall thickening (n = 10; 38 %), perivascular inflammation (n = 8; 31 %), aneurysms (n = 5; 19 %), and stenosis (n = 4; 15 %). Comparisons with the control group revealed that LVI was significantly associated with neurological manifestations (OR=3.23 [95 % CI: 1.11-10.01, p = 0.03]), but not with cardiovascular risk factors (OR=0.70 [95 % CI: 0.23-2.21, p = 0.60]), or AAV relapse (OR=2.01 [95 % CI: 0.70-5.88, p = 0.16]). All patients received corticosteroids, in combination with an immunosuppressant in 24 (92 %), mostly cyclophosphamide (n = 10, 38 %) or rituximab (n = 9, 35 %). CONCLUSION: Regardless of distinctions based on vessel size, clinicians should consider LVI as a potential manifestation of AAV, with the aorta commonly affected. The risk of developing LVI appears to be greater for clinical phenotypes of AAV with neurological involvement. Standard AAV treatment can be used to manage LVI.
Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Humanos , Masculino , Femenino , Persona de Mediana Edad , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Estudios de Casos y Controles , Anciano , Adulto , Factores de Riesgo , Inmunosupresores/uso terapéuticoRESUMEN
INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare necrotizing vasculitis affecting small vessels and associated with severe asthma and eosinophilia. Monoclonal antibodies blocking the IL-5 signaling pathway, one example being benralizumab, decrease the proliferation of eosinophils and represent an effective treatment in severe eosinophilic asthma. They are a therapeutic option currently studied for EGPA. We report the paradoxical clinical case of EGPA appearing following the initiation of treatment with benralizumab (anti-IL-5R monoclonal antibody). CASE REPORT: We describe the case of a 66-year-old female patient with severe asthma. Following the initiation of benralizumab, she showed deterioration of her general condition, associated with severe peripheral neuropathy of the lower limbs. At the same time, she developed massive hypereosinophilia. After an extensive workup, the patient was eventually diagnosed with an EGPA with multisystem involvement. Benralizumab was stopped and a course of high dose corticosteroids and immunosuppressants was initiated, enabling slow clinical recovery. CONCLUSION: Anti-IL5 monoclonal antibodies, including benralizumab, are being investigated for new therapeutic indications, including EGPA. Paradoxically, a few rare cases of EGPA associated with these molecules have been reported in the literature. The causal link of this association remains hypothetical. Close monitoring of patients on benralizumab consequently seems indispensable.
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Asma , Síndrome de Churg-Strauss , Eosinofilia , Granulomatosis con Poliangitis , Anciano , Anticuerpos Monoclonales Humanizados/uso terapéutico , Asma/complicaciones , Síndrome de Churg-Strauss/complicaciones , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/tratamiento farmacológico , Femenino , Granulomatosis con Poliangitis/complicaciones , Granulomatosis con Poliangitis/diagnóstico , Granulomatosis con Poliangitis/tratamiento farmacológico , HumanosRESUMEN
We propose a new numerical model to describe thrombus formation in cerebral aneurysms. This model combines CFD simulations with a set of bio-mechanical processes identified as being the most important to describe the phenomena at a large space and time scales. The hypotheses of the model are based on in vitro experiments and clinical observations. We document that we can reproduce very well the shape and volume of patient specific thrombus segmented in giant aneurysms.
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Aneurisma Intracraneal/complicaciones , Aneurisma Intracraneal/patología , Modelos Biológicos , Análisis Espacio-Temporal , Trombosis/complicaciones , Trombosis/patología , Simulación por Computador , Hemorreología , Células Endoteliales de la Vena Umbilical Humana/metabolismo , Humanos , Aneurisma Intracraneal/fisiopatología , ARN Mensajero/genética , ARN Mensajero/metabolismo , Estrés Mecánico , Trombosis/fisiopatologíaRESUMEN
Since the advent of philosophy and rational thought, a question has been raised: Is medicine an art, a science, a technique, or the three of them? In this paper we discuss two monuments of Western thought that have approached this topic: Plato and Aristotle. For Plato, medicine is focused on what is transient and changing and therefore it is a subordinate science. However, he has a positive view because it presupposes knowledge of all and he takes medicine as a dialectical model. Aristotle places medicine in his classification of sciences. He emphasizes the idea that the purpose of medicine is based on what "happens most often" and insists on essential role of experimentation. It is remarkable to notice that these ideas developed 2.500 years ago are still relevant and remain the core of the epistemological conceptions of modern medicine.