Raynaud's phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report.

BACKGROUND: POEMS syndrome is a rare paraneoplastic syndrome caused by plasma cell disorder almost always lambda restricted. Secondary Raynaud's phenomenon is an overlooked skin manifestation of the disease even though it is present in twenty percent of patients. On POEMS syndrome have not been described positive antinuclear antibodies (ANA) and this could lead to a misdiagnosis of autoimmune disease, mainly systemic sclerosis. CASE PRESENTATION: A 47-year-old man presented with color changes on fingertips consistent with biphasic Raynaud's phenomenon; an antinuclear antibody test was positive (at 1:320 titers in a speckled pattern) with normal nailfold capillaroscopy. Clinical features of systemic sclerosis were absent. Twenty-four months later, the patient presented symmetric sensorimotor demyelinating polyneuropathy, and he was diagnosed with Guillain-Barre syndrome; treatment with intravenous gammaglobulin had an incomplete response. Raynaud's phenomenon persisted associated with acrocyanosis, white nails, and positive ANA (1:1280 in a nucleolar pattern). POEMS syndrome was suspected, and serum protein electrophoresis (SPEP) was done. The SPEP revealed polyclonal gammopathy, and serum immunofixation showed monoclonal (M)-protein (IgG lambda). Serum vascular endothelial growth factor concentration showed increased levels. The patient was diagnosed with POEMS syndrome, and treatment with lenalidomide and dexamethasone improved the Raynaud's phenomenon, acrocyanosis, and white nails, but the neurological response was partial. CONCLUSIONS: POEMS syndrome may mimic clinical manifestations of systemic sclerosis v.g. Raynaud's phenomenon, skin thickening, telangiectasia, and positive ANA. Raynaud's phenomenon may precede other clinical manifestations of POEMS syndrome by several months. It is necessary to have a high index of suspicion for the diagnosis, especially in patients with peripheral polyneuropathy and monoclonal paraprotein. The significance of positive ANA in this condition is unknown and deserves further investigation.

Desenlaces obstétricos en pacientes con arteritis de Takayasu: serie de casos / Pregnancy outcomes in patients with Takayasu's arteritis: Case series

RESUMEN Objetivo: La actividad y el riesgo de recaída de la arteritis de Takayasu son bajos durante la gestación. Hasta el 40% de las pacientes puede tener desenlaces obstétricos desfavorables, por lo que es importante conocer su comportamiento clínico. Describimos las características clínicas y el desenlace obstétrico de gestantes con arteritis de Takayasu atendidas en un hospital de alta complejidad. Materiales y métodos: Evaluación retrospectiva de historias clínicas de gestantes con arteritis de Takayasu atendidas en el Hospital Universitario San Vicente Fundación de Medellín, Colombia, entre 2011 y 2018. Resultados: Se incluyó en el estudio a 6 pacientes con mediana de edad al diagnóstico de 17,5 arios (RI 9,25), al parto de 24 arios (RI 8,25) y con una duración de la enfermedad de 5,5 arios (RI 10,5). Del total, 3 pacientes tenían compromiso aórtico extenso; al parto, 3 pacientes esta ban activas y requirieron inmunosupresores, 5 tenían hipertensión arterial, una desarrolló preeclampsia en el segundo trimestre, una tenía insuficiencia mitral y tricuspídea grave con disminución de la fracción de eyección del ventrículo izquierdo; 2 tenían aneurismas (arte ria subclavia izquierda y aorta ascendente). Ningún embarazo resultó en aborto ni parto pretérmino; hubo 2 óbitos fetales, uno por restricción del crecimiento intrauterino e insuficiencia placentaria, y otro de etiología desconocida; ambas pacientes con actividad de la enfermedad, afección aórtica extensa e hipertensión arterial. Fueron por cesárea 5 par tos por indicación materna; no hubo disección aórtica, rotura aneurismática ni hemorragia cerebral. Conclusión: Las pacientes con enfermedad activa y afección aórtica extensa presentaron resultados obstétricos desfavorables, lo que indica que el inadecuado control de la vasculitis genera mayores complicaciones materno-fetales.

ABSTRACT Objective: The activity and risk of relapse of Takayasu's arteritis are low during pregnancy. Up to 40% of patients may have unfavorable obstetric outcomes therefore it is important to know their clinical behavior. We describe the clinical characteristics and obstetric outcome of pregnant women with Takayasu arteritis treated in a hospital of high complexity. Materials and methods: A retrospective evaluation of medical records of pregnant patients with Takayasu's arteritis treated at Hospital Universitario San Vicente Fundación in Medellin, Colombia between 2011 and 2018. Results: Six patients with a median age at diagnosis 17.5 (RI 9.25) years, at delivery 24 (RI 8.25) years, disease duration 5.5 (RI 10.5) years. Three patients had extensive aortic involvement; at delivery, 3 patients were active and required immunosuppressants, 5 had high blood pres sure, one developed preeclampsia in the second trimester, one had severe mitral and tricus pid insufficiency with a decreased ejection fraction of the left ventricle; 2 had aneurysms (left subclavian artery and ascending aorta). No pregnancy resulted in abortion or preterm birth; there were 2 fetal deaths, one due to intrauterine growth restriction and placental insuffi ciency and another of unknown etiology; both patients with disease activity, extensive aortic condition, and arterial hypertension. Five deliveries were by cesarean section by maternal indication; there was no aortic dissection, aneurysmal rupture or cerebral hemorrhage. Conclusion: Patients with active disease and extensive aortic compromise presented unfa vorable obstetric results, suggesting that inadequate control of vasculitis leads to greater maternal-fetal complications.

Bullous lupus as the first manifestation of systemic lupus erythematosus in the pediatric population: A diagnostic challenge in daily practice.

INTRODUCTION: In the GLADEL cohort, the bullous lupus (BSLE) prevalence was 0.41%. However, literature on pediatric BSLE is scarce. This study described the clinical, histological, and immunological characteristics and the treatment response in a series of children with BSLE as the first clinical manifestation of pediatric SLE. METHODS: The clinical, histological, and immunological characteristics of a series of 5 cases of BSLE between 2010-2019 from two reference centers in Colombia were analyzed. RESULTS: All cases had bullous lesions that resolved with residual hypopigmentation. One had a focal seizure, and another arthritis with thrombocytopenia. Two had transient proteinuria with normal urinalysis. Anti-nuclear antibody titers ranged from 1:160 to 1:2560, and four were anti-dsDNA (+). Five patients had anti-RNP antibodies, and four anti-Sm antibodies. All had low C3, and 80% low C4 counts; 80% had erythrocyte sedimentation rate (ESR) ≥20 mm/hour and 60% had C-reactive protein (CRP) ≥0.5 mg/dL. All patients responded to glucocorticoids and dapsone. Histology reports and direct immunofluorescence (DIF) test showed subepidermal blisters with neutrophils in the papillary dermis and linear deposits of Igs/complement proteins in 80% of the skin biopsies. IgG/IgM was present in 5 samples. IgA was positive in 60% and C3 in 80%. CONCLUSIONS: In this pediatric series, BSLE tends to have a monophasic behavior associated with neuropsychiatric, skeletal, and hematological involvement in 40% of the patients, and with good prognosis.

Uso de desfibriladores implantables y terapia de resincronización cardiaca en ancianos mayores de 70 a 80 años: controversias y evidencia / Use of implantable defibrillators and cardiac resynchronisation therapy in patients more than 70 to 80 years old: arguments and evidence

Resumen Prevenir la muerte súbita y aumentar la supervivencia y la calidad de vida en los pacientes con falla cardiaca son las principales indicaciones para el uso de los desfibriladores implantables y la terapia de resincronización cardiaca respectivamente. Hay certeza de la eficacia clínica de estos dispositivos para la población general; sin embargo, no es tan clara para los pacientes mayores de 70 años dado que esta población no está lo suficientemente representada en los diseños de los estudios clínicos. La evidencia disponible hasta el momento precisa que en este grupo etario existe beneficio de la terapia de resincronización cardiaca, pero es cuestionable el uso de cardiodesfibriladores. Así mismo, es indispensable tener en cuenta no sólo el riesgo de mortalidad de los pacientes sino también su funcionalidad y fragilidad para definir si se benefician o no de estos dispositivos.

Abstract The prevention of sudden death, to increase the survival, and quality of life in patients with heart failure, are the main indications for the use of implantable defibrillators and cardiac resynchronisation therapy, respectively. There is confidence in the clinical efficacy of these devices for the general population, but it is not so clear in patients over 70 years-old, given that this population is not sufficiently represented in clinical study designs. Although the evidence available up until now shows that there is a benefit in this age group for the use cardiac resynchronisation therapy, the use of cardiac defibrillators is questionable. It is also indispensable to not only to take into account the mortality risk in these patients, but also their functionality and frailty in order to determine whether or not they would benefit from these devices.

Nódulo pulmonar cavitado en artritis reumatóide: reporte de un caso / Cavitating pulmonary nodule in rheumatoid arthritis: A case report

RESUMEN Los nódulos reumatoides pulmonares son una manifestación extra-articular infrecuente de la artritis reumatoide (AR). Su estudio es importante porque su presentación clínica e imagenológica puede confundirse con tuberculosis, infecciones fúngicas y neoplasias pulmonares. Producen pocos síntomas y pueden conducir a complicaciones fatales como neumotórax, fístula broncopleural y sobreinfección con formación de abscesos. La confirmación diagnóstica fundamental descansa en el estudio histopatológico, dado que es un diagnóstico de exclusión. El tratamiento precisa seguimiento clínico ya que no hay una terapia dirigida utilizándose en casos severos rituximab y tocilizumab. En general, su aparición tiende a empobrecer el pronóstico de los pacientes con AR.

ABSTRACT Pulmonary rheumatoid nodules are an uncommon extra-articular manifestation of rheumatoid arthritis (RA). Its study is important because the clinical and imaging presentation could be confused with tuberculosis, fungal infections, and/or lung neoplasms. Pulmonary nodules produce few symptoms and can lead to fatal complications such as pneumothorax, bronchopleural fistula, and a superinfection with abscess formation. The fundamental diagnostic confirmation is the histopathological study because it is an exclusion diagnosis. The treatment needs clinical follow-up as there is no targeted therapy, with Rituximab and Tocilizumab being used in severe cases. In general, their appearance tends to lead to a poorer prognosis of patients with RA.