RESUMEN
Granulomatosis with polyangiitis (GPA) is primary necrotizing vasculitis, which predominantly affects small to medium vessels. Herein, we describe a case of a 60-year-old female with GPA who developed inflammatory wall thickening localized in the aortic arch, upper abdominal aorta, and pulmonary artery. The wall thickening in the large vessels and other GPA lesions such as lung nodules and orbital mass had failed to respond to high-dose glucocorticoids combined with cyclophosphamide; however, all were successfully treated with rituximab. Our literature review identified 24 cases of large-vessel involvement associated with GPA. Luminal stenosis, occlusion, or wall thickening were observed in 8, periaortitis in 11, and aneurysms in 5 cases. The most commonly affected vessel was the abdominal aorta (12 cases), followed by the thoracic aorta (6 cases), subclavian artery (4 cases), and internal carotid artery (4 cases). Glucocorticoids were used in 23 cases, 20 of which received combination therapy with cyclophosphamide. Surgical or endovascular therapies were performed in 10 cases with aneurysmal dilatation. This is the first case showing the potential efficacy of rituximab for refractory large-vessel involvement associated with GPA.
Asunto(s)
Granulomatosis con Poliangitis/tratamiento farmacológico , Rituximab/uso terapéutico , Enfermedades Vasculares/tratamiento farmacológico , Ciclofosfamida/uso terapéutico , Femenino , Glucocorticoides/uso terapéutico , Granulomatosis con Poliangitis/complicaciones , Humanos , Persona de Mediana Edad , Retratamiento , Resultado del Tratamiento , Enfermedades Vasculares/complicacionesAsunto(s)
Anticuerpos Antiidiotipos/sangre , Inmunoconjugados/uso terapéutico , Enfermedades Musculares/tratamiento farmacológico , Enfermedades Musculares/inmunología , Partícula de Reconocimiento de Señal/inmunología , Abatacept , Humanos , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Enfermedades Musculares/sangre , Resultado del TratamientoRESUMEN
We herein report the case of an 84-year-old man with steroid-dependent adult-onset Still's disease (AOSD) whose daily steroid dose was successfully tapered after etanercept treatment. The corticosteroids worked well initially, and the patient went into remission promptly; however, he suffered a relapse due to steroid tapering. Because treatment with cyclosporine and methotrexate was ineffective, reducing the steroid dose was difficult, and the corticosteroids induced myopathy and diabetes. However, steroid tapering was accomplished in combination with etanercept therapy, and the patient's steroid-induced side effects disappeared. Etanercept should therefore be considered as a steroid-sparing treatment option in patients with steroid-responsive, steroid-dependent AOSD.