RESUMEN
A man in his 80s had a 4-week history of progressive weakness and fatigue, with associated development of purple bruiselike lesions on his head. What is your diagnosis?
Asunto(s)
Cuero Cabelludo , Neoplasias Cutáneas , Masculino , Humanos , Neoplasias Cutáneas/diagnóstico , Diagnóstico DiferencialAsunto(s)
Enfermedad Injerto contra Huésped , Trasplante de Células Madre Hematopoyéticas , Linfoma Cutáneo de Células T , Neoplasias Cutáneas , Enfermedad Injerto contra Huésped/etiología , Humanos , Linfoma Cutáneo de Células T/terapia , Neoplasias Cutáneas/terapia , Acondicionamiento Pretrasplante , Trasplante HomólogoRESUMEN
CASE: A 67-year-old female presented with an exceedingly rare cardiac neoplasm - papillary fibroelastoma. This is made rarer still as it occurred on the pulmonary valve. The patient complained of a prolonged history of chest discomfort. Magnetic resonance imaging and echocardiography revealed a pulmonary valve papillary fibroelastoma. Surgical excision proved curative and the patient remains asymptomatic to date. DISCUSSION: The literature surrounding papillary fibroelastomas is discussed. Primary cardiac tumours are uncommon. Papillary fibroelastomas occurring the right side of the heart comprise less than 0.05% of these. They have a characteristic macroscopic appearance which allow them to be easily identified with echocardiography and at surgical excision. They can present in a variety of ways including classical cardiac symptoms, embolic complications or as an incidental finding. Surgical excision is the definitive treatment.
Asunto(s)
Fibroelastoma Papilar Cardíaco/cirugía , Neoplasias Cardíacas/cirugía , Válvula Pulmonar/cirugía , Anciano , Fibroelastoma Papilar Cardíaco/diagnóstico por imagen , Ecocardiografía , Femenino , Neoplasias Cardíacas/diagnóstico por imagen , Humanos , Imagen por Resonancia Magnética , Válvula Pulmonar/diagnóstico por imagen , Enfermedades Raras , Resultado del TratamientoRESUMEN
BACKGROUND: The aim was to study changes in immunohistochemical expression markers of synovial and skin inflammation, clinical outcomes and magnetic resonance imaging (MRI) scores with abatacept treatment in patients with psoriatic arthritis (PsA). METHODS: Biological-treatment-naïve PsA patients with active disease including synovitis of a knee were enrolled in this single-centre, crossover study. Patients were randomised to receive intravenous abatacept 3 mg/kg of body weight or placebo infusion on day 1, 15 and 29; thereafter abatacept 10 mg/kg of body weight was administered every 28 days for 5 months. Clinical data were collected at each visit. Synovial biopsy of the involved knee was obtained at baseline and 2 and 6 months. MRI of the same knee and skin biopsy was performed prior to arthroscopy. RESULTS: Fifteen patients were recruited. Significant improvements in the joint-related measures were observed; 90% were European League Against Rheumatism criteria responders and 30% achieved psoriasis area severity index (PASI)50 at 6 months. Reduction in synovitis (P = 0.016) and vascularity (P = 0.039) macroscopic scores consistent with decrease in total MRI score (P = 0.016) were noticed. Abatacept decreased the immunohistological expression of FOXP3+ cells (P = 0.027), specifically the expression of CD4+FOXP3+ regulatory T cells (Tregs) (P = 0.008) in the synovium over 6 months. There was no significant clinical or immunohistological change in any of the skin measures. CONCLUSION: This is the first study assessing synovial and psoriatic skin immunpathological changes following abatacept treatment in PsA. Reduction in Treg expression in the synovium but not in the psoriatic lesion suggests abnormal Treg function in PsA with differential suppressive capacity in the synovium compared to the lesional skin. The results of this study demonstrate that abatacept 10 mg/kg of body weight might be an effective treatment option for joint disease in patients with PsA. TRIAL REGISTRATION: Irish Health Products Regulatory Authority. TRIAL REGISTRATION NUMBER: CT 900/489/1 - Abatacept (case number: 2077284, EudraCT Number: 2009-017525-19, Protocol number: 77777). Registered on 12 March 2010.