[Temporal arteritis: treatment controversies]. / En la arteritis de la temporal: controversias terapéuticas.

INTRODUCTION: Although giant cell or temporal arteritis represents 5-10% of ischaemic optic neuropathies and is the most common arteritis in people over 60 years old. Currently there is no established treatment with oral glucocorticoids available. BACKGROUND: Glucocorticoid (GC) is still the treatment of choice but has to be started without delay in order to prevent neurological or systemic complications. However, we can resort to intravenous treatment in cases where there is loss of sight or other neurological symptoms. In cases refractory to GC or in those in whom we wish to decrease the dose due to adverse effects, individualised treatment with methotrexate or TNF blockers could be indicated. There is insufficient evidence to recommend other treatments, such as azathioprine, dapsone, cyclosporine, cyclophosphamide or imitinib. In patients with vascular risk factors, anti-platelet therapy with ASA should be assessed. Surgical treatment should be considered in selected cases with symptoms due to arterial stenosis. CONCLUSIONS: The corticoids continue to be the treatment of choice in temporal arteritis, however, given the clinical variability of the disease and the special characteristics of this group of patients, usually elderly and with systemic diseases, we believe that individualised treatment with coherent therapeutic guidelines are essential. Currently there is not only treatment with oral glucocorticoids available, although in our patients we can choose to use intravenous mega-doses, anti-platelet treatment, resort to methotrexate or TNF inhibitors in refractory cases, or even consider surgical approaches.

En la arteritis de la temporal: controversias terapéuticas / Temporal arteritis: treatment controversies

Introducción: Aunque la arteritis de células gigantes o temporal representa un 5-10% de las neuropatías ópticas isquémicas y es la arteritis más frecuente en las personas mayores de 60 años, aún no existen pautas de tratamiento establecidas. Desarrollo: El tratamiento con glucocorticoides continúa siendo el de elección y debe iniciarse sin demora en aras de prevenir complicaciones neurológicas o sistémicas. Sin embargo, podemos recurrir a tratamiento intravenoso en casos con pérdida de visión u otros síntomas neurológicos. En casos refractarios a glucocorticoides o en los que deseamos reducir la dosis por la aparición de efectos adversos, el metotrexato y los agentes bloqueadores del factor de necrosis tumoral podrían estar indicados de manera individualizada. De otros tratamientos —como azatioprina, dapsona, ciclosporina, ciclofosfamida o imitinib— no hay evidencias suficientes para recomendarlos. En pacientes con factores de riesgo vascular se debe valorar la antiagregación con ácido acetil salicílico. El tratamiento quirúrgico se debe valorar en casos seleccionados con síntomas debidos a estenosis arteriales. Conclusiones:El tratamiento de elección de la arteritis de la temporal continúan siendo los corticoides; sin embargo, dada la variabilidad clínica de la enfermedad y las características especiales del grupo de paciente, habitualmente mayores y con enfermedades sistémicas, consideramos fundamental el tratamiento individualizado según unas pautas terapéuticas coherentes. Actualmente no sólo disponemos de tratamiento con corticoides orales, sino que en nuestros pacientes podremos elegir el uso de megadosis intravenosas, antiagregar, en casos refractarios recurrir a metotrexato o inhibidores del factor de necrosis tumoral o incluso valorar aproximaciones intervencionistas (AU)

Introduction: Although giant cell or temporal arteritis represents 5-10% of ischaemic optic neuropathies and is the most common arteritis in people over 60 years old. Currently there is no established treatment with oral glucocorticoids available.Background: Glucocorticoid (GC) is still the treatment of choice but has to be started without delay in order to prevent neurological or systemic complications. However, we can resort to intravenous treatment in cases where there is loss of sight or other neurological symptoms. In cases refractory to GC or in those in whom we wish to decrease the dose due to adverse effects, individualised treatment with methotrexate or TNF blockers could be indicated. There is insufficient evidence to recommend other treatments, such as azathioprine, dapsone, cyclosporine, cyclophosphamide or imitinib. In patients with vascular risk factors, anti-platelet therapy with ASA should be assessed. Surgical treatment should be considered in selected cases with symptoms due to arterial stenosis. Conclusions: The corticoids continue to be the treatment of choice in temporal arteritis, however, given the clinical variability of the disease and the special characteristics of this group of patients, usually elderly and with systemic diseases, we believe that individualised treatment with coherent therapeutic guidelines are essential. Currently there is not only treatment with oral glucocorticoids available, although in our patients we can choose to use intravenous mega-doses, anti-platelet treatment, resort to methotrexate or TNF inhibitors in refractory cases, or even consider surgical approaches (AU)

Rotura espontánea de los tendones extensores de la muñeca y la mano en pacientes con artritis reumatoide. Estudio retrospectivo / Spontaneous rupture of extensor tendons in the hands of patients with rheumatoid arthritis. A retrospective study

Fundamento: La rotura espontánea de los tendones extensores de la mano es una complicación relativamente frecuente en pacientes con artritis reumatoide (AR) de larga evolución. Por ello es importante conocer los factores locales predisponentes para la rotura espontánea, así como las posibilidades quirúrgicas, las complicaciones y los resultados de la reconstrucción de los tendones afectados. Material y métodos: Estudio retrospectivo de todos los pacientes con AR seguidos en nuestro hospital y diagnosticados de rotura espontánea de tendones extensores de la mano desde octubre de 1995 hasta diciembre de 2000. Se identificó un total de 18 pacientes intervenidos quirúrgicamente con un seguimiento adecuado de al menos un año. Se describen los factores locales predisponentes, el tiempo de evolución de la enfermedad desde el diagnóstico hasta que ocurrió la rotura, el tiempo de espera hasta la cirugía, las opciones quirúrgicas, las complicaciones y el resultado funcional (basado en el test de función de la mano de Jebsen). Resultados: Los factores locales predisponentes más frecuentes fueron la inestabilidad cubital distal y la tenosinovitis crónica de extensores (presentes en el 61% de los casos). El tiempo medio de evolución de la enfermedad hasta que ocurrió la rotura espontánea fue de 122 ± 73,19 meses, con un tiempo medio de espera desde el diagnóstico de la rotura hasta la cirugía reparadora de 2,38 ± 1,75 meses. La opción quirúrgica más frecuentemente realizada consistió en la transferencia del tendón extensor propio del índice al cabo roto y sutura término-lateral (en el 55,5% de los pacientes). Las complicaciones posquirúrgicas se observaron en 3 pacientes (16,6%). El resultado funcional fue excelente o bueno en 16 pacientes (89%) y se consideró regular en 2 pacientes (11%). Discusión: Es importante identificar a los pacientes con AR que presentan inestabilidad radiocubital distal y tenosinovitis crónica de extensores de la mano por el riesgo de rotura espontánea de dichos tendones. Una valoración conjunta y consensuada con un cirujano ortopédico y un rehabilitador con especial dedicación a enfermos con AR ayuda a una cirugía temprana y unos resultados favorables en la mayoría de los casos (AU)

Background: Spontaneous rupture of extensor tendons in the hand is a relatively frequent complication in patients with rheumatoid arthritis (RA). Consequently, it is important to consider local risk factors, treatment options, complications, and functional results after surgery. Material and methods:: A retrospective study of all the patients with RA diagnosed with spontaneous extensor tendon ruptures in the hand in our hospital between October 1995 and December 2000 was performed. Eighteen patients with a follow-up of at least one year after surgery were selected. The following data were analyzed: local risk factors, interval between diagnosis and rupture, time to reconstructive surgery, treatment options, and complications and functional results after surgery (according to Jebsen’s test of hand function). Results: The most common risk factors were distal ulnar instability and chronic dorsal tenosynovitis (61%). The mean disease duration until tendon rupture was 122 ± 73.19 months, whereas the mean period before surgery was 2.38 ± 1.75 months. The preferred surgical option was terminolateral suture and extensor indicis proprius transfer for ruptured tendon (55%). Postsurgical complications were observed in 3 patients (16.6%). Functional results were excellent or good in 16 patients (89%). Discussion: In patients with RA, distal ulnar instability and chronic dorsal tenosynovitis are significant risk factors for spontaneous tendon rupture in the hand. A multidisciplinary approach, involving the rheumatologist, orthopedic surgeon and therapist, is crucial for prompt surgery and good functional results (AU)

Vasculitis: ¿hablamos de la misma cosa? / Vasculitis: do we mean the same entity?

No disponible

[Lhermitte's sign in three oncological patients]. / Fenómeno de Lhermitte en tres pacientes oncológicos.

INTRODUCTION: Lhermitte's sign was first described by Pierre Marie and Chatelin in 1917. Lhermitte published his report in 1920 and reviewed this in 1924. This phenomenon is characterized by the occurrence of an electric shock-like sensation going along the spine in a cervico-caudal direction with flexion of the neck, and may also be felt in the upper and lower limbs. Clinical cases. Case 1. A 49 year-old woman diagnosed as having breast cancer and being treated with cisplatin presented with Lhermitte's sign. On physical examination the osteotendinous reflexes were absent but the abdominal cutaneous reflexes were present. There was reduced sensitivity to vibration. Case 2. An 80 year-old man, previously operated on for adenocarcinoma of the colon, without further treatment, presented with progressive weakness of all four limbs and Lhermitte's syndrome. On examination there was obvious weakness of all four limbs, with the sensory level at C3. A cervical MR scan showed a metastasis in the vertebral body of C3 with epidural involvement and compression of the spinal cord. Case 3. A 54 year-old man was being treated by radiotherapy for cancer of the larynx. He presented at the onset of Lhermitte's sign but MR and physical examination were normal. CONCLUSION: Lhermitte's sign is non-specific, although in oncological patients a detailed clinical history and examination should be done seeking data regarding chemotherapy, radiotherapy and spinal compression.

Fenómeno de lhermitte en tres pacientes oncológicos / Lhermitte's sign in three oncological patients

Introducción. El fenómeno de Lhermitte fue descrito por primera vez por Pierre Marie y Chatelin en 1917. Lhermitte realiza su comunicación en 1920 y la reanaliza en 1924. Este fenómeno se caracteriza por la aparición de una sensación de corriente eléctrica que recorre en sentido cervicocaudal la columna vertebral con la flexión del cuello, y puede proyectarse también por las extremidades superiores e inferiores. Casos clínicos. Caso 1. Mujer de 49 años diagnosticada de cáncer de mama en tratamiento con cisplatino que presenta un fenómeno de Lhermitte. En la exploración se halló ausencia de reflejos osteotendinosos con cutáneo-abdominales presentes. La sensibilidad vibratoria estaba disminuida. Caso 2. Varón de 80 años intervenido de adenocarcinoma de colon sin otro tratamiento posterior, que presenta cuadro progresivo de pérdida de fuerza en las cuatro extremidades y fenómeno de Lhermitte. En la exploración se observa pérdida de fuerza en las cuatro extremidades, con nivel sensitivo C3.Una RM cervical confirmó metástasis en el cuerpo vertebral de C3 con afectación epidural y compresión medular. Caso 3. Varón de 54 años que recibió radioterapia por un carcinoma de laringe. Comienza con fenómeno de Lhermitte, y la RM y la exploración fueron normales. Conclusiones. El fenómeno de Lhermitte es inespecífico, aunque en los pacientes oncológicos debemos realizar una exploración e historia clínica detallada en busca de quimioterápicos, radioterapia y datos de compresión medular (AU)

[Idiopathic thrombocytopenic purpura as first manifestation of systemic lupus erythematosus lupus]. / Lupus eritematosos sistémicos que debutan con púrpura trombopénica idiopática.

OBJECTIVES: We studied SLE whose initial symptoms were related to idiopathic thrombocytopenic purpura (ITP) in order to determine if they share clinical and immunologic manifestations. METHODS: We reviewed the clinical backgrounds of 150 SLE (according to the ACR criteria) periodically followed from 1980 until 1998. We found 12 patients with both these conditions. RESULTS: All patients were female with a mean age of 32 at the time of ITP diagnosis and 36 at the time of SLE diagnosis. The most usual clinical manifestations were: arthritis (92%), cutaneous (58%) and hematologic involvement with lymphopenia (58%) and thrombocytopenia again (33%) after the initial ITP episode, always together with autoimmune hemolytic anemia (Evans syndrome). None of these patients presented with neurologic involvement and only one presented with renal involvement. 50% were positive for anti-DNA antibodies, 50% were Ro(+) and 16% were RNP (+). 66% were positive for antiphospholipid antibodies and 33% for lupus anticoagulant. Thrombocytopenia was controlled just with steroids in only 16% of the patients. Splenectomy controlled thrombocytopenia with complete remission achieved in 80% (4 from 5) of the patients and 20% (1 from 5) were refractory to this therapy after a medium follow-up time of 6.5 years. CONCLUSIONS: 1) SLE whose initial symptoms are related to ITP were characterized by joints, cutaneous and hematologic involvement without renal and neurologic manifestations. 2) Splenectomy was able to control refractory thrombocytopenia in the majority of these patients.

[Clinical manifestations associated with antiphospholipid antibodies]. / Manifestaciones clínicas asociadas a anticuerpos antifosfolípido.

The antiphospholipid antibodies are immunoglobulins able to join negative charge phospholipids. The have been related to a great variety of conditions, specially among connective tissue illness although the idiopathic form seems to be the most frequent. Their presence must be ruled out in cases of young patients with stroke, deep veins thrombosis, acute heart attack and woman suffer multiple abortions and foetal death. These antibodies appear to be related to different clinical entities like Sneddon syndrome. Evans syndrome, "chorea gestationis", migraine. The laboratory determinations are based in direct methods (ELISA, RIA, ...) as well as in indirect ones (activated partial thromboplastin time, reptilase time, ...). The appropriate management and treatment may be based upon clinical expression, in case of arterial thrombosis (type II APS), or deep vein thrombosis (Type II) long term anticoagulation is indicated; Association with pentoxifylline in the case of retinal thrombosis (type IIIa), Stroke (type IIIb) cases may require long term anticoagulation as well as aspirin. Type IV cases are better managed with an individualised treatment.