Caso clínico de enfermería: modelo de adaptación en adolescente con síndrome de Felty y Rhupus / Nursing Clinical Case: Model of Adaptation in Adolescent with Felty and Rhupus Syndrome / Relato de caso de enfermagem: modelo de adaptação em adolescente com síndrome de Felty e Rhupus

Introducción. El síndrome de Rhupus es la superposición de dos enfermedades autoinmunes, la artritis reumatoide o artritis idiopática juvenil y el lupus eritematoso sistémico, la prevalencia es de 7-401 por 100,000 niños. El síndrome de Felty se caracteriza por la tríada de artritis idiopática juvenil, esplenomegalia y neutropenia; padecer más de una patología reumática es un extraño fenómeno estimado entre el 0.01-2%. Objetivo. Describir el proceso de atención de enfermería integral en una adolescente con Rhupus y síndrome de Felty, bajo el modelo de adaptación de Callista Roy. Metodología. Caso clínico de enfermería en una paciente de 15 años seleccionada en hospitalización pediátrica, con previo consentimiento informado; intervenida a través del proceso de atención de enfermería estructurado según la taxonomía de la North American Nursing Diagnosis Association, la Clasificación de Resultados de Enfermería, y la Clasificación de Intervenciones de Enfermería, con intervenciones intrahospitalarias y seguimiento con tele-enfermería. Resultados. Mejoría de la ambulación y afrontamiento de problemas evidenciados por el aumento del bienestar de la paciente y la familia. Conclusiones. Ante una enfermedad desconocida, el proceso de atención de enfermería, con intervenciones directas y acompañamiento continuo, permite realizar una atención integral, a fin de lograr la adaptación de la paciente y su familia. Palabras clave: Adaptación Psicológica; Atención de Enfermería; Enfermería; Síndrome de Felty; Lupus Eritematoso Sistémico; Resiliencia Psicológica.

Introduction. Rhupus syndrome is the overlap of two autoimmune diseases, rheumatoid arthritis or juvenile idiopathic arthritis, and systemic lupus erythematosus, with a prevalence of 7-401 per 100,000 children. Felty's syndrome is characterized by the triad of juvenile idiopathic arthritis, splenomegaly, and neutropenia; experiencing more than one rheumatic pathology is a rare phenomenon estimated between 0.01-2%. Objective. Describe the comprehensive nursing care process in an adolescent with Rhupus and Felty's syndrome, under the adaptation model of Callista Roy. Methodology. Nursing case study of a 15-year-old patient selected in pediatric hospitalization, with prior informed consent; intervened through the structured nursing care process according to the taxonomy of the North American Nursing Diagnosis Association, the Nursing Outcomes Classification, and the Nursing Interventions Classification, with in-hospital interventions and follow-up through tele-nursing. Results. Improvement in ambulation and coping with problems evidenced by the increased well-being of the patient and the family. Conclusions. Faced with an unknown disease, the nursing care process, with direct interventions and continuous support, allows for comprehensive care to achieve the adaptation of the patient and her family. Keywords: Adaptation, Psychological; Nursing Care; Nursing; Felty Syndrome; Lupus Erythematosus, Systemic; Resilience, Psychological.

Introdução. A síndrome de Rhupus é a sobreposição de duas doenças autoimunes, artrite reumatoide ou artrite idiopática juvenil e lúpus eritematoso sistêmico, a prevalência é de 7-401 por 100,000 crianças. A síndrome de Felty é caracterizada pela tríade de artrite idiopática juvenil, esplenomegalia e neutropenia; sofrer de mais de uma patologia reumática é um fenômeno estranho estimado entre 0.01-2%. Objetivo. Descrever o processo de assistência integral de enfermagem em uma adolescente com Rhupus e síndrome de Felty, sob o modelo de adaptação de Callista Roy. Metodologia. Caso clínico de enfermagem em uma paciente de 15 anos selecionada em internação pediátrica, com prévio consentimento informado; ela teve intervenção por meio do processo de cuidado de enfermagem estruturado segundo a taxonomia da North American Nursing Diagnosis Association, a Classificação dos Resultados de Enfermagem e a Classificação das Intervenções de Enfermagem, com intervenções intra-hospitalares e acompanhamento com tele-enfermagem. Resultados. Melhora na deambulação e enfrentamento de problemas evidenciados pelo aumento do bem-estar do paciente e da família. Conclusões. Diante de uma doença desconhecida, o processo de assistência de enfermagem, com intervenções diretas e acompanhamento contínuo, permite um cuidado integral, de forma a alcançar a adaptação do paciente e de sua família. Palavras-chave: Adaptação Psicológica; Cuidados de Enfermagem; Enfermagem; Síndrome de Felty; Lúpus Eritematoso Sistêmico; Resiliência Psicológica.

Síndrome de felty: reporte de caso / Felty syndrome: a case report

El síndrome de Felty (SF) es una manifestación extraarticular de la artritis reumatoide (AR), caracterizada por: AR, neutropenia y esplenomegalia es infrecuente aunque severo. Se desarrolla en personas de mediana edad, con una historia de AR. El diagnóstico es estrictamente clínico y el tratamiento está enfocado a disminuir el dolor articular, las infecciones y evitar las deformidades óseas. Presentamos el caso de una mujer de 46 años, que ingresa por el servicio de emergencias con dificultad respiratoria, fiebre y neutropenia siendo diagnosticada con neumonía y encontrándose en estudio. El trabajo de seguimiento mostró esplenomegalia, y fue diagnosticada con AR de varios años de evolución. Lo enunciado junto a otras evidencias ayudaron a que fuera diagnosticada con SF. El interés del caso es presentar una rara complicación de la AR, que en ocasiones puede ser sub-diagnosticada. Los pacientes con neutropenia continua, altos nivel de factor reumatoide y auto anticuerpos deben ser sospechosos de desarrollar el SF como una presentación inicial de AR o con una AR no diagnosticada como en este caso.

Felty's Sindrome (SF) is an extraarticular manifestation of rheumatoid arthritis (AR), characterized by: AR, neutropenia and splenomegaly which is infrequent but hursh. It is developed in people of middle age and with an history of AR. The diagnosis is strictly clinical and the treatment is focused on subside the joint pain, the infections and relieve osseus deformities. We report the case of a woman of 46 years old who entered by the service of emergency with respiratory difficult, fever and neutropenia who was diagnosed with pneumonia in study. The following work shows splenomegaly and was diagnosed with AR of many years of evolution. It stated along with another evidences supported the diagnosis of SF. The particular interest ofthis case is to presentan unusual complication of AR, which occasionally could be sub-diagnosticated. The patients with continuous neutropenia, high levels of rheumatoid factor and autoantibodies have to be suspects of develop SF as an initial presentation of AR or with an AR without diagnosis like on this case.

Treatment with Rituximab in a Patient with Refractory Felty Syndrome and Low Rheumatoid Arthritis Disease Activity: A Case Report

Felty syndrome (FS) is a rare manifestation in rheumatoid arthritis (RA) characterized by neutropenia and splenomegaly. Treatment for FS is not well established because there has been no randomized controlled study. A few recent reports found rituximab effective in patients with refractory FS. According to those reports, most patients with RA and FS had active arthritis. Here we report a case of a patient with glucocorticoid dependent and disease-modifying anti-rheumatic drugs (DMARDs) refractory FS and quiescent RA who was successfully treated with rituximab.

Embolización parcial del bazo en el síndrome de Felty: intervencionismo para un síndrome clínico / Partial splenic embolization in felty´s syndrome: interventionism in a clinic syndrome

El síndrome de Felty comprende la asociación de artritis reumatoidea crónica, neutropenia y esplenomegalia. Esta última, cuando se asocia a hiperesplenismo, se caracteriza por el desarrollo de trombocitopenia, leucopenia, anemia o de todas. La embolización parcial endovascular del bazo puede ser una opción válida para el tratamiento del hiperesplenismo, pero no está exenta de riesgos. Presentamos el caso de un paciente joven, con artritis reumatoidea, síndrome de Felty e hiperesplenismo, a quien se realizó embolización parcial endovascular del bazo.

Felty's syndrome is a rare association of rheumatoid arthritis, neutropeniaand splenomegaly. When associated with hypersplenism ischaracterized by increased pooling or destruction of the corpuscularelements of the blood by the spleen and thrombocytopenia, leukopenia,or anemia may be present.Partial splenic embolization has shown to be effective. However, theprocedure may increase the risk of complications.We described a case of a young patient with Felty's syndrome, rheumatoidarthritis and hypersplenism who underwent partial splenicendovascular embolization.

Neumonía organizante criptogénica asociada asíndrome de Felty / Cryptogenic organizing pneumonia associated with Felty's syndrome

El síndrome de Felty es la tríada de artritis, esplenomegalia y neutropenia. Adicionalmente, sepueden encontrar en este síndrome múltiples factores propios de la artritis reumatoide, entreellos la presencia de nódulos pulmonares. Presentamos el caso de un paciente con hallazgosclínicos y paraclínicos que evidencian la presencia del síndrome de Felty, presentando, además,nódulos pulmonares en los estudios radiológicos e histológicos que documentaron granulomascrónicos, bronquiolitis obliterante y neumonía organizante.

A Case of Bowen's Disease During Adalimumab Treatment for Felty's Syndrome / 대한내과학회지

Felty's syndrome is an uncommon condition characterized by the combination of rheumatoid arthritis (RA), splenomegaly, and neutropenia. Disease-modifying anti-rheumatic drugs and biologic agents have been used to treat early RA to achieve remission. Anti-tumor necrosis factor (TNF)-alpha agents are effective for controlling the disease; however, some studies have suggested the possibility of adverse effects such as increased frequency of infection or malignancy. Bowen's disease, known as squamous cell carcinoma in situ, is a skin cancer that can be caused by sun exposure, arsenic ingestion, human papilloma virus, skin damage, and prolonged immunosuppression. We report the case of a 64-year-old female diagnosed with Bowen's disease during treatment with an anti-TNF agent for Felty's syndrome with a review of the literature.

Síndrome de Felty e Calazar: um desafio para o reumatologista / Felty's syndrome and Kala-azar: a challenge for the rheumatologist

Os autores relatam o caso de uma paciente com artrite reumatoide que evoluiu com grave neutropenia e esplenomegalia, sendo firmado o diagnóstico de Síndrome de Felty, que posteriormente desenvolveu Calazar. Ambas têm apresentação clínica e laboratorial semelhantes, tornando o diagnóstico diferencial difícil. O relato deste caso objetiva chamar a atenção para o reconhecimento da infecção por leishmaniose visceral em pacientes portadores de doenças reumáticas, assim como a possibilidade de um paciente com Calazar mimetizar um quadro de doença reumática sistêmica.

Case report of a patient with rheumatoid arthritis who developed severe neutropenia, splenomegaly and was diagnosed with Felty's syndrome. The patient later developed Kala-azar. Both diseases have similar clinical and laboratory presentation, making the differential diagnosis difficult. The present case report aims at drawing attention to the identification of visceral Leishmaniasis infection in patients with rheumatic diseases, as well as possibility of a patient with Kala-azar mimicking a set of symptoms of systemic rheumatic disease.

Rituximabe na síndrome de Felty refratária / Rituximab in the refractory Felty's syndrome

Os autores relatam o caso de uma paciente de 29 anos com diagnóstico de artrite reumatoide soropositiva que com seis meses de evolução desenvolveu granulocitopenia severa e esplenomegalia, embora mantivesse em remissão o quadro articular. Não apresentou resposta à corticoterapia oral e em forma de pulsos, além do metotrexato e leflunomida, tendo apresentado reação adversa ao uso do infliximabe e falta de resposta ao adalimumabe. Diante das infecções de repetição, apesar dos vários esquemas de antibióticos e uso crônico do G-CSF, dos altos títulos de fator reumatoide, dos níveis elevados da VHS e da PCR, utilizou-se o rituximabe no esquema clássico de tratamento da artrite reumatoide. Houve resposta clínica completa com aumento crescente do número de neutrófilos e normalização dos mesmos além da queda dos títulos de fator reumatoide, da VHS e da PCR. Atualmente, a paciente encontra-se em remissão clínica e laboratorial, em uso de prednisona 5 mg/dia e metotrexato 10 mg/semana.

The authors report a case of a 29 year old woman who has seropositive rheumatoid arthritis for six months, and developed severe granulocytopenia and important splenomegaly, however she didnït show any joint inflammation. She did not respond either to pulse or oral steroids, or to oral methotrexate and leflunomide. She also developed an adverse reaction to the use of infliximab and did not respond well to adalimumab. Although she has had repeated infections, despite the various forms of antibiotics and long-term use of G-CSF, with high titers of rheumatoid factor, and high levels of ESR and CRP, the classic Rituximab method for treating rheumatoid arthritis was used. There was a good clinical response with an increase in the number of neutrophils following normalization of them, together with the reduction of rheumatoid factor titers, ESR and CRP. At the moment, the patient is in remission, according to both clinical and laboratory criteria and taking 5mg of prednisone per day and 10mg of methotrexate per week.

Extra-articular manifestations of rheumatoid arthritis: a hospital-based study

The frequency of extra-articular manifestations in rheumatoid arthritis [ExRA] differs from one country to another, so we investigated ExRA frequency in a well-defined hospital patient population with rheumatoid arthritis [RA] in Saudi Arabia. We also examined possible predictors of the development ExRA. A retrospective analysis was conducted of all patients diagnosed with RA at a university hospital during a 4-year period. Cases were classified according to the 1987 American College of Rheumatology criteria for RA, and the frequency of ExRA was recorded. Of 140 patients who fulfilled the criteria for the diagnosis of RA, 98 [70%] developed ExRA features. Anemia occurred in 61%, thrombocytosis in 16%, pulmonary involvement in 10%, and renal amyloidosis, vasculitis and Felty syndrome were present in 6%, 2% and 1%, respectively. The mortality rate was high [16%] in patients with ExRA. The predictors for mortality were lung involvement, age over 50 years and kidney amyloidosis. ExRA were present in a substantial proportion of our patients, which lead to a worse disease outcome. Anemia, thrombocytosis and respiratory system involvement were the commonest. Early recognition and treatment are important to decrease mortality

Síndrome de Felty: relato de caso e revisão da terapêutica / Felty's syndrome: case report and review of therapeutics

Os autores relatam um caso de artrite reumatóide, com 20 anos de evolução, que desenvolveu neutropenia e esplenomegalia em uso de 20 mg de metotrexato, que persistiu mesmo com sua substituição pela ciclosporina A. Apresentou infecções de orofaringe, pele e trato urinário. Após afastar doenças hematológicas, o diagnóstico foi de síndrome de Felty. Realizou-se o tratamento com antibioticoterapia, fator estimulador de colônia de granulócitos e macrófagos, pulsoterapia com metilprednisolona e leflunomida. Evoluiu inicialmente com melhora clínica e laboratorial. Após 2 meses de uso do leflunomida, foi admitida em pronto-socorro com quadro de sepse, ocorrendo o óbito em poucas horas.

The authors describe the case of a 42 years-old female patient with rheumatoid arthritis with a 20 years of follow-up. The patient was using methotrexate (20 mg/week); she developed neutropenia and splenomegaly that persisted despite changing medication for cyclosporine A. She then developed oropharyngeal, skin and urinary tract infections. After excluding for hematological affections, she was diagnosed as presenting Felty's syndrome. She was started on antibiotics while receiving also granulocyte-macrophage colony stimulating factor, pulsed methylprednisolone, and leflunomide. Two moths after the initiation of leflunomide, she was admitted to an emergency hospital unit with septic shock that resulted in her death in a few hours.

Síndrome de Felty / Felty's syndrome

La artritis reumatoidea es una patología multisistémica, generalmente crónica y progresiva, caracterizada por un compromiso articular inflamatorio destructivo, en especial de pequeñas articulaciones, y múltiples manifestaciones extraarticulares, entre las que se destaca la afectación cutánea. El síndrome de Felty es una rara complicación de la artritis reumatoidea asociada a leucopenia con neutropenia y esplenomegalia. Este síndrome puede acompañarse de lesiones cutáneas como úlceras, que afectan principalmente los miembros inferiores. Se presenta el caso de dos pacientes con síndrome de Felty

Nodular regenerative hyperplasia of the liver: report of a case

Nodular regenerative hyperplasia of the liver [NRHL] is characterized by hepatocellular nodules without fibrous septa between the nodules, and has been described in association with certain diseases. The NHRL should be considered in a liver mass and coexisting portal hypertension. We described the case of a 33-year-old Iranian man with NRHL in association with essential thrombocythemia

Síndrome de Felty: caso de recidiva após suspensão da medicação de base / Felty's syndrome: relapse after discontinuing the medication

Os autores descrevem o caso de uma paciente de 49 anos de idade, com história de 30 anos de artrite reumatóide. Em 1993 desenvolveu neutropenia persistente e esplenomegalia. O diagnóstico foi síndrome de Felty (SF). O tratamento foi realizado com fator estimulador de colônia de granulócitos e macrófagos (GM-CSF), prednisona e metotrexato (MTX). Após melhora clínica e laboratorial, com valores normais de células sangüíneas, apenas o MTX foi mantido obtendo estabilização da doença. Em fevereiro de 2001, a paciente foi considerada em remissão e o MTX foi suspenso. Após dois meses, a paciente apresentou recidiva. Esse caso demonstra a importância da manutenção do tratamento com metotrexato, mesmo em baixas doses, para controlar a atividade da doença, considerando o aumento da morbidade e mortalidade associado às complicações de neutropenia grave

Síndrome febril prolongado con linfadenopatías: actualización y revisión del tema / Prolonged febrile syndrome with lymphadenopathy: update and review of this process

Presentamos una serie de 5 pacientes con síndrome febril prolongado con linfadenopatías (S.F.P.LA.), 2 de sexo masculino y 3 de sexo femenino, entre 16 y 55 años de edad. La etiología fue: lupus eritematoso sistémico (n=1), sarcoidosis (n=1), síndrome de Felty (n=1), linfadenopatía angioinmunoblástica (n=1) y mononucleosis infecciosa (n=1). Todos los casos fueron diagnosticados entre 1997 y 2002. Realizamos además una revisión y actualización del tema.

Surgical Treatment of Rheumatoid Arthritis / 대한정형외과학회잡지

Rheumatoid arthritis is a chronic inflammatory systemic disease of young or middle aged adults, characterized by destructive and proliferative changes in the synovial membrane, periarticular structures, skeletal muscle and perineural sheath. Eventually, the joints are destroyed, ankylosed and deformed. Therefore, the aim of treatment is to keep the inflammatory process at a minimum, thereby preserving joint motion, maintaining the health of muscle supplying motor power about the joint, and preventing secondary joint stiffness and deformity. Surgical treatment in rheumatoid arthritis has progressed and there have been advances in the relief of pain and increase in the range of motion. For the period of 15 years from January 1973 to December 1987, 55 cases of rheumatoid arthritis who received surgical treatment were reviewed and the results of clinical observation were as follows : 1. Among 977 patients of rheumatoid arthritis, 76 sites in 55 patients were operated on. 2. Sites of operation were the knee in 33 cases, hip in 20 cases, wrist and finger in 12 cases, ankle in 5 cases, elbow in 5 cases, and cervial spine in 1 case. 3. Operative methods were 40 cases of joint replacement, 27 cases of synovectomy, 4 cases of fusion, and 2 cases of tenotomy and capsulectomy. 4. Postoperative results were good in 19 patients, satisfactory in 16 patients, unsatisfactory in 5 patients, and poor in 2 patients. 5. The clinical stage at the time of joint replacement was not related to the end results of the operations. 6. Causes of unsatisfactory and poor results were preoperative flexion contracture and Felty's syndrome.