Lupus eritematoso cutáneo y lupus eritematoso sistémico: revisión de una historia sin fin / Cutaneous lupus erythematosus and systemic lupus erythematosus: endless story review

l lupus eritematoso es una afección que se conoce desde antiguo, referida en especial al compromiso cutáneo, pero ha sido mejor definida desde principios del siglo XIX, constituyéndose como una enfermedad sistémica con una base autoinmune. Mecanismos patogénicos inmunogenéticos desempeñan un papel en la susceptibilidad a la enfermedad, sobre la que actuarían variaciones hormonales y factores ambientales. De esta interacción deriva la producción de múltiples anormalidades inmunológicas, cuya acción sobre los tejidos da origen a la expresión clínica de la enfermedad. La relación entre el compromiso cutáneo, en especial crónico discoide, y sistémico, ha sido un factor de polémica, importante de dilucidar por el significado terapéutico y de pronóstico para el enfermo. De esta revisión se puede concluir que las diferentes manifestaciones del lupus constituyen una misma enfermedad, pero probablemente factores etiopatogénicos genéticos, hormonales y ambientales marcan la diferencia.

Lupus erythematosus is a condition that has been known since ancient times, especially referring to skin involvement, but has been better defined since the beginning of the 19th century, constituting itself as a systemic disease with an autoimmune basis. Immunogenetic pathogenic mechanisms play a role in disease susceptibility, on which hormonal variations and environmental factors would act. From this interaction derives the production of multiple immunological abnormalities, whose action on the tissues gives rise to the clinical expression of the disease. The relationship between cutaneous involvement, especially chronic discoid, and systemic, has been a controversial factor, important to elucidate due to its therapeutic and prognostic significance for the patient. From this review it can be concluded that the different manifestations of lupus constitute the same disease, but genetics, hormonal and environmental etiopathogenic factors probably make the difference.

Más allá del diagnóstico: comunicación, lenguaje y empatía / Beyond diagnosis: communication, language and empathy

La medicina actual, inmersa en una sociedad fría e individualista, se ha alejado de la buena comunicación con el enfermo. Es fundamental que en la formación médica más allá de entender la enfermedad, se comprenda al ser humano que la padece, lo que es parte del humanismo médico. La buena comunicación, más aún si es de una mala noticia para el enfermo y su entorno cercano, debe acompañarse de un lenguaje adecuado y un grado de empatía, que trasmitan una esperanza que no se aparte de la veracidad y la realidad.

Medicine today, immersed in a cold and individualistic society, has moved away from good communication with the patient. It is essential in medical training more beyond to understand disease, comprise the human suffering it, which is part of medical humanism. Good communication, even if it is bad news for the sick and their close environment, must accompanied by a suitable language and a degree of empathy, that convey hope to not depart from the truth and reality, accompanied by a suitable language and a degree of empathy, that convey hope to not depart from the truth and reality.

Artritis reumatoidea de 20 años o más de evolución: experiencia preliminar / Rheumatoid arthritis 20 years and over of evolution: preliminary experience

Rheumatoid Arthritis (RA) is characterized by persistent synovitis, systemic inflammation and presence of autoantibodies, mainly Rheumatoid Factor and Anticitrullinated peptide antibody. This condition develop in genetically susceptible individuals, where environmental factors interact, although the pathogenesis is not yet fully known. Since the disease was described until now, there have been major diagnostic and therapeutic advances. In this paper was performed a retrospective and prospective evaluation of 28 patients with RA over twenty years of evolution, was held in treatment and periodic inspection at the Department of Rheumatology at Hospital San Juan de Dios...

La Artritis Reumatoide (AR) se caracteriza por presentar una sinovitis persistente, inflamación sistémica y presencia de autoanticuerpos, fundamentalmente factor reumatoideo y anticuerpo anti-péptido citrulinado. Esta patología se desarrolla en individuos genéticamente susceptibles, donde interactúan diversos factores ambientales, aunque la patogenia aún no se conoce en su totalidad. Desde que se describió la enfermedad hasta la actualidad, ha habido grandes avances diagnósticos y terapéuticos. En este trabajo se realizó una evaluación retrospectiva y prospectiva de 28 pacientes con AR sobre 20 años de evolución, en tratamiento y control periódico en el Departamento de Reumatología del Hospital San Juan de Dios...

Lupus eritematoso sistémico en niños en su transición a adultos: experiencia basada en 17 casos / Systemic lupus erythematosus in children during their transition into adulthood: experience based on 17 cases

Foreword: Systemic Lupus Erythematosus (SLE) is an inflammatory, autoimmune and multisystemic disease featured by the presence of multiple antibodies, and which appears in both genders and at any age, including children, albeit rare under 5 years of age. Objective: To assess its clinical and in-lab manifestations, and the evolution of pediatrics patients and of those who transition into adulthood. Results: Seventeen (17) patients with infantile inception were assessed; they met the SLICC/ACR diagnose criteria over a time span running between years 2001 and 2013. They recorded an 88 percent of musculoskeletal compromise, 88 percent of renal compromise, 47 percent of alopecia, 47 percent of photosensitivity, and 42 percent of neurological compromise. All (100 percent) of them tested positive for antinuclear antibodies (ANA), 88 percent for anti-DNA, and 53 percent were Sm-positive. Anaemia was found in 42 percent of them, Leukopenia in 12 percent, and Lymphopenia in 35 percent. As regards the treatment, all of them (100 percent) received hydroxychloroquine, 94 percent were administered prednisone, 82 percent mycophenolate, and 71 percent cyclophosphamide. Fifteen of them (88 percent) have transitioned to the adults’polyclinic, and from them 6 (35 percent) have evidenced SLE reactivations associated to the lack of adherence to treatment. In this series the survival rate is of 100 percent of patients during the follow-up period. Conclusion: Infantile-inception Lupus, although less frequent, is more severe than that with an inception during adulthood, with a high prevalence of nephropathy, above half of the Classes III or IV. Early treatment with corticoids, immunosuppressants and hydroxychloroquine enables a favorable evolution and an appropriate transition into adulthood, with a survival rate of 100 percent. It is necessary to strengthen a good doctor-patient relationship during this transition, including a proper communication between the pediatric...

Introducción: El Lupus Eritematoso Sistémico (LES) es una enfermedad inflamatoria, autoinmune y multisistémica, caracterizada por la presencia de múltiples autoanticuerpos y que se presenta en ambos sexos y a cualquier edad, incluido en niños, siendo raro bajo los 5 años de edad. Objetivo: Evaluar sus manifestaciones clínicas, de laboratorio y evolución en pacientes pediátricos y en los que pasan a adultos. Resultados: Se analizó a 17 pacientes de inicio infantil que cumplieron los criterios diagnósticos SLICC y ACR en un periodo comprendido entre los años 2001 y 2013. Presentaron compromiso musculoesquelético 88 por ciento, renal 88 por ciento, alopecia 47 por ciento, fotosensibilidad 47 por ciento y neurológico 42 por ciento. Un 100 por ciento tuvieron anticuerpos antinucleares (AAN) positivos, 88 por ciento anti-DNA y 53 por ciento Sm positivos. Se encontró anemia en 42 por ciento, leucopenia en 12 por ciento y linfopenia en 35 por ciento. En relación al tratamiento, el 100 por ciento recibió hidroxicloroquina, prednisona 94 por ciento, micofenolato 82 por ciento y ciclofosfamida 71 por ciento. Quince (88 por ciento) han hecho la transición al Policlínico de Adultos, de los cuales seis (35 por ciento) han presentado reactivaciones del LES asociadas a falta de adherencia al tratamiento. En esta serie la sobrevida es del 100 por ciento de los pacientes durante el periodo de seguimiento. Conclusión: El Lupus de inicio infantil, si bien menos frecuente, es más severo que el de inicio adulto, con alta prevalencia de nefropatía, sobre la mitad de clase III o IV. El tratamiento precoz con corticoides, inmunosupresores e hidroxicloroquina permite una evolución favorable y un adecuado paso a la adultez, con 100 por ciento de sobrevida. Es necesario reforzar una buena relación médico-paciente en esta transición, incluida una comunicación adecuada entre el reumatólogo infantil y de adulto, que asegure mantener una adherencia y control óptimo de la enfermedad.

Crónica de una historia vivida / Chronicle of an experienced story

La Artritis Reumatoídea (AR) ha constituido durante mucho tiempo uno de los temas centrales de la reumatología, tanto por los misterios de su etiopatogenia como por el significado que tiene para quien la padece, que ve amenazadas su calidad de vida y su capacidad de participación en la sociedad. Los estudios en relación a su etiopatogenia han pasado por diferentes etapas. El desarrollo de la inmunología a partir de la década de los 70 permitió abrir nuevas perspectivas en relación a los mecanismos patogénicos que contribuían a la expresión clínica de la enfermedad. Una de las contribuciones iniciales para la mejor comprensión de la patogénesis de la AR fue el hallazgo de leucocitos que contenían inclusiones (célula AR) en el líquido sinovial de pacientes con AR y que estas células podían activar sus lisosomas y producir destrucción articular. El analizar el pasado permite comprender mejor el presente y lo que se esbozaba ayer es una realidad hoy, y actualmente la AR se considera una enfermedad autoinmune con expresión no sóloarticular, sino sistémica. Desde la presencia de la célula AR, se ha saltado a una gran familia celular, que participa a través de complejos mecanismos inmunológicos en la patogénesis de una enfermedad, que ha encontrado un diagnóstico cada vez más precoz y un tratamiento cada vez más eficaz, permitiendo, a su vez, una mejor calidad de vida a quienes la presentan.

Rheumatoid Arthritis (RA) has been for a long time a main subject of research in Rheumatology, not only for its largely unknown pathogenic mechanisms, but also for the great deal of discapacity that can produce in the patients that endure this disease and their capacity for participation on society. In relation to its pathogenesis, studies have gone through differents stages. The development of immunology since the 1970s allowed the opening of new perspectives in relation to the pathogenic mechanism that have contributed to the clinical expression of the disease. One of the earliest contributions to the understanding of the pathogenesis of RA was the finding of leucocytes containing inclusions (the RA cell) in the synovial fluid of patients wih RA and that this cells could activate their lysosomal and produce joint destruction. Studying the past allows to better understanding the present and it outlined yesterday is a reality to day and currently the RA is considered an autoimmune disease with expression not just articulate, but systemic disease. Since the presence of the RA cell, is has jumped to a big cell family, that participates through complex immunological mechanism involved in the pathogenesis of a disease, which has found an increasingly early diagnosis and more effective treatment allowing turn a better quality of life to those who present.

Amiloidosis: revisión a propósito de un caso clínico / Amyloidosis: review of clinical case purpose

La amiloidosis constituye un grupo de enfermedades caracterizadas por el depósito extracelular de material proteico autólogo, fibrilar e insoluble. Existe una variedad que se asocia a enfermedades inflamatorias crónicas mal controladas que presentan manifestaciones orientadoras al diagnóstico, lo que es importante de conocer, ya que hace variar el pronóstico de la enfermedad de base. A continuación se presenta un caso clínico de amiloidosis secundaria a artropatía psoriásica, discutiendo su diagnóstico y posibilidades terapéuticas tanto para la enfermedad de base como para su asociación y complicación por amiloidosis.

Amyloidosis is a group of diseases characterized by the extracellular deposition of protein material autologous fibrillar insoluble. There is a variety that is associated with poorly controlled chronic inflammatory diseases that have manifestations in the diagnosis. Below we present a clinical case of secondary amyloidosis in psoriatic arthropathy and discuss its diagnosis and further management.

Polimialgia reumática: experiencia basada en 22 casos / Polymyalgia rheumatica: experience based on 22 cases

Introducción. La polimialgia reumática (PMR) se caracteriza por dolor y rigidez de las cinturas cervicoescapular y pelviana; preséntase en mayores de 50 años, asociada frecuentemente a arteritis de células gigantes (ACG), y tiene excelente respuesta a dosis bajas de corticosteroides. Objetivo. Evaluar sus características clínicas, de laboratorio, terapéuticas y evolutivas en nuestro medio. Pacientes y método: 22 pacientes diagnosticados según criterios establecidos y controlados entre 1998 y 2008. Según protocolo se analizan sexo, edad, diagnósticos previos, clínica, laboratorio, tratamiento y evolución. Resultados. Edad 52 a 81 años (mediana 69,3); predominio femenino (17/5), y 9,2 meses promedio de evolución prediagnóstico. Todos presentaron dolor y rigidez cervicoescapular y pelviana, 2 pacientes (9 por ciento) tuvieron artritis y 2 (9 por ciento), características clínicas de arteritis de células gigantes (ACG). El laboratorio fue inespecífico, excepto velocidad de sedimentación elevada entre 38 y 131 mm/hora (promedio 80,7). Todos tuvieron factor reumatoídeo (-) y anticuerpos antinucleares (-). Todos, excepto los portadores de ACG, respondieron a dosis bajas de corticosteroides (20 mg/día de prednisona o menos), con dosis de mantención entre 5 y 10 mg/día. Excelente evolución en todos, excepto uno, con desaparición de síntomas, normalización de VHS y reintegración a actividades previas. Conclusiones. La PMR constituye un diagnóstico diferencial en personas mayores de 50 años con dolor de cintura escapular y pelviana, que suele asociarse con ACG. El diagnóstico se basa en criterios bien establecidos, destacando la VHS persistentemente alta y la respuesta a dosis bajas de corticosteroides, que es además la terapéutica de elección.

Polymyalgia rheumatica (PMR) is characterized by cervicoscapular and pelvic pain and rigidity, it manifests in people older than 50 years and is frequently associated with giant cell arteritis (GCA). It responds very well to low doses of corticosteroids. Objective: Evaluate clinical, laboratory, therapeutic and evolutionary characteristics in our environment. Patients and method: 22 patients diagnosed according to set criteria and controlled between 1998 and 2008. Sex, age, previous clinical and laboratory diagnosis, treatment and evolution were analyzed. Results: Age 52 to 81 (average 69.3); 17 females,5 males; and 9.2 month average prediagnostic evolution. All presented cervicoscapular and pelvic pain and rigidity, 2 patients (9 percent) had arthritis, and 2 (9 percent) presentedclinical characteristics of GCA. Laboratory tests were nonspecific, except for high sedimentation speed between 38 and 131 mm/hour (average 80.7). All patients presented rheumatoid factor (-) and antinuclear antibodies (-). All, except GCA carriers, responded to low doses of corticosteroids (20 mg/day or less of Prednisona), followed by a maintenance dose of 5 - 10 mg/day. Evolution was excellent in all patients except one, symptoms disappeared, ESR normalized and patients were reintegrated to their previous activities. Conclusions: PMR constitutes a differential diagnosis in people over 50 years of age, with cervicoscapular and pelvic pain, and is commonly associated with GCA. Diagnosis is based on well established criteria, with persistently high ESR and response to low doses of corticosteroids - the treatment therapy of choice - among the most significant.

Satisfacción del paciente y su relación con el médico y el equipo de salud en una unidad de reumatología docente asistencial de un hospital público: estudio preliminar / Survey on patient´s satisfaction and relationship with physicians and health care team in an assistential and teaching rheumatology unit of a public hospital

En una época que se caracteriza por el culto a la tecnología, en la que predomina la relación impersonal, en que es imperioso lograr metas y en las que el rendimiento se mide por la cantidad de pacientes que tienen acceso a la atención médica, consideramos de interés conocer la evaluación de parte del paciente, de la calidad de la atención que se le entrega en un servicio público de la especialidad, tanto desde el punto de vista de la accesibilidad a la prestación y el lugar donde esta se realiza, como a la relación humana que se establece con el médico y el equipo de salud. Basado en preguntas de fácil comprensión, que fueron contestadas en forma anónima por 203 pacientes en control habitual en la Unidad de Reumatología del Hospital San Juan de Dios, se concluye que actualmente son insuficientes las horas médicas de la especialidad y que al menos un tercio de los consultantes no logra obtener sus horas por el conducto regular (SOME). Lo mismo ocurre también con la posibilidad que éstos tienen de acceder a los exámenes radiológicos y a los medicamentos entregados por el hospital. Destaca, sin embargo, una significativa aprobación de la relación con el equipo de salud en general, y con el médico tratante en particular, pese a que sólo poco más de la mitad de los enfermos considera que su afección está mejor. Esta disociación puede vincularse a una buena relación humana médico-paciente, derivada de la cual hay satisfacción por la ayuda recibida y la asistencia otorgada, pese a tratarse de enfermedades crónicas y limitadas.

Satisfacción del paciente y su relación con el médico y el equipo de salud en una unidad de reumatología docente-asistencial de un hospital público: estudio preliminar / Patient satisfaction and their relationship with the doctor and the health team: preliminary study

In a time where technology has taken on a cult following and where impersonal relations prevail, and where meeting goals in which performance is measured in number of patients who have access to medical care are more pressing than ever, we present a study of the patients’ evaluation as to the quality of attention that they receive in a public specialized service, both from the accessibility to care and the place where this is done, as well as the human relationship with the doctor and the medical team. Based on easy to understand questions that were answered anonymously by 23 patients undergoing routine controls at the rheumatology unit of the San Juan de Dios Hospital, we found that medical hours of the specialty are currently insufficient and at least one third of the patients are unable to obtain an appointment through regular channels (SOME, Statistical Medical Orientation Service in its Spanish acronym), this includes the possibility to access radiological examinations, and medications given by the hospital. Nevertheless, an important approval rate between patients and the health team in general and the treating doctor in particular does stand out, even though little more than half of the patients consider that their illnesses have improved. This dissociation may be due to a good doctor/patient relationship, from which we can conclude that, in spite of dealing with usually chronic and limiting diseases, there is a degree of satisfaction derived from the help and service given.

Edema de la médula ósea de la rodilla / Bone marrow edema of knee

Bone marrow edema (BME) or transient bone marrow edema syndrome (TMES) affects the joints, particularly thehip and less frequently the knee and ankle. Knee problemsare usually related to traumatic causes, nevertheless, this can also be caused by micro traumatisms brought on bydaily life or overload. Diagnosis is confirmed by usingnuclear magnetic resonance (NMR), since both x-rays andcomputer assisted tomographies (CAT) are unsupportive,and scintigraph, though sensitive, is none specific. It isdescribed as the cause of pain in osteoarthritis and canalso be observed in transitory osteoporosis, in migratoryregional osteoporosis, and in the first stage of avascularbone necrosis (ABN), which is its main differential diagnosis. We present three cases of patients with knee pain, with no history of contusion. Two cases presented minor signs of osteoarthritis, two presented a decrease in bone density, and one presented hyperlaxity. NMRs were required, since normal procedures led to no results, and showed the existence of BME. Treatment was conservative, specially oriented towards unloading the joints, with a favorable evolution prior to six months.

Lupus y embarazo: consideraciones en relación a situaciones críticas / Lupus and pregnancy: critical situations considerations

There is a reciprocal relationship between lupus and pregnancy, which implies greater risk for both mother and fetus, and even though this was very important until the middle of the last century, it has currently decreased significantly due to increased awareness of the disease, its treatment, and when certain basic conditions are considered prior to conception. It is also important to unserstand situations that increase risk, as well as others that are completely contrary to pregnancy. Nevertheless, a well-planned pregnancy is not always the case and different courses of action must be adequately implemented if needed. We review options when faced with pregnancy with lupus, in presence of antiphospholipids, when the mother is a carrier of anti-Ro and anti-La antibodies; and finally the problems of pregnancy with arterial hypertension and proteinuria. We emphasis the importance of a close relation between rheumatologists and gynecologist/obstetricians, and conclude that the more informed we are, both in terms of the disease itself and the risks implied for a pregnant women with lupus, the better we can treat and face this situation with tranquility and optimism.

Médicos y profesionalismo: Pacientes e información / Physicians and professionalism: Patients and information

In the present time, the advances in sciences brought by technology are worshiped. This contributes to dehumanize human relationships. Medical profession is a commitment to oneself, society and social justice. Scientific and humanist formation must coexist during the training of new physicians. The main objective of medical profession is the relationship with patients. Patients have acquired increasing autonomy and a deliberative relationship is the closest to the ideal medical action. The relationship must be based in autonomy, beneficence, lack of maleficence and justice. The patient must receive all the information about his ailment and the diagnostic and therapeutic alternatives in a clear and intelligible language. This will allow to obtain a fully conscious informed consent from the patient. Asking for this consent, gives the patient the opportunity to practice his responsability and to decide the best for him in a friendly and confident environment. In the middle of science and technology, a close contact with ethics and humanism will allow a better understanding of the integrity of subjects, specially when they are ill.

Uso de metotrexato en pacientes con lupus eritematoso sistÚmico / Methotrexate use in patients with systemic lupus erythematosus

Background: In the past ten years, methotrexate (MTX) has appeared as an alternative for the treatment of systemic lupus erythematosus (SLE). Aim: To evaluate the use of MTX and the results of treatment in a group of patients with SLE. Patients and Methods: Retrospective review of 426 files of patients with SLE. Of these, all patients treated with MTX were selected. A review protocol was designed, stating sex, age, time of disease evolution, previous treatment, MTX prescription, doses, effectiveness and side effects. Results: Seven patients were selected. All were women, with an age range of 26 to 62 years old and with 5 to 34 years of disease evolution. Previous treatment with non steroidal anti-inflammatory drugs, steroids, hydroxychloroquine and azathioprine ranged from 6 to 631 months (average=147.3). Persistent joint and/or skin manifestations were the main cause for the use of MTX. The dose ranged from 7.5 to 15 mg and the treatment lapse from 6 to 106 months. It was effective in all patients, allowing a reduction in prednisone doses. Two patients experienced a rise in serum transaminases and one had gastric intolerance, that required treatment discontinuation. Conclusions: MTX is rarely used in SLE. Its main prescription and effectiveness is in joint and skin manifestations, allowing to decrease steroid doses. However, side effects are frequent.