Diagnóstico y tratamiento de la hipertensión pulmonar / Diagnosis and treatment of pulmonary hypertension

La hipertensión arterial pulmonar es un proceso idiopático o puede estar asociado a otras circunstancias como enfermedades del tejido conectivo, cardiopatías congénitas, hipertensión portal, exposición a inhibidores del apetito u otras drogas, o a agentes infecciosos como en el virus de la inmunodeficiencia humana (VIH). En la mayor parte de los pacientes se llega al diagnóstico como resultado de la valoración de sus síntomas, mientras que otros son diagnosticados fortuitamente o durante la revisión sistemática de individuos asintomáticos pertenecientes a grupos de riesgo. Se revisan los métodos de valoración útiles para el diagnóstico de la hipertensión arterial pulmonar. Un algoritmo diagnóstico puede servir de guía en dicha evaluación, aunque puede ser modificado de acuerdo con circunstancias clínicas específicas. El número de opciones terapéuticas se ha incrementado en los últimos años. Se revisa la utilización de los bloqueadores de los canales de calcio, prostaciclina y análogos, antagonistas de los receptores de la endotelina, inhibidores de la fosfodiesterasa-5 y del tratamiento combinado y se proporcionan recomendaciones específicas acerca del tratamiento actual

Pulmonary arterial hypertension is an idiopathic process or can be associated with another circumstances (connective tissue diseases, congenital heart disease, portal hypertension, exposure to appetite suppressants or anoher drugs or infectious agents such as HIV). Most patients are diagnosed as the result of an evaluation of symptoms, whereas others are diagnosed incidentally or during screening of asymptomatic populations at risk. We reviews systematic screening for the approach to diagnosing pulmonary arterial hypertension. A diagnostic algorithm can guide the evaluation but it can be modified according to specific clinical circumstances. The number of therapeutic options has increased.in the last years. We reviews the use of calcium-channel blockers, prostacyclin (and analogues), endothelin-receptor antagonists, and phosphodiesterase-5 inhibitors, and the use of combination therapy, and provides specific recommendations about the actual treatment (AU)

Enfermedad de Vogt-Koyanagi-Harada. Características de un grupo de pacientes andaluces / Vogt-Koyanagi-Harada disease. Characteristics of a series of patients

Objetivo. Describir las características de una serie de pacientes diagnosticados de enfermedad de Vogt-Koyanagi-Harada (EVKH) seguidos en una Unidad de Uveítis (multidisciplinaria, formada por oftalmólogos e internistas) en nuestro medio. Pacientes y método. Estudio retrospectivo descriptivo de 11 pacientes con EVKH (5 varones y 6 mujeres; edad media al diagnóstico: 32,6 años) seguidos entre 1980 y 2003. Resultados. Todos sufrieron panuveítis y/o desprendimiento exudativo de retina. Aparecieron alteraciones extraoculares en todos: neurológicas en el 63,7% (meningitis aséptica y/o síntomas focales), dermatológicas en el 81,8% (vitíligo, canicie, poliosis, alopecia), hipoacusia neurosensorial (el 50% de pacientes con audiometría) y síntomas generales en el 25%. Todos recibieron glucocorticoides sistémicos. Se añadió ciclosporina en 5 pacientes (45,4%) con inflamación de polo posterior y azatioprina en 2 por inflamación anterior. La agudeza visual final fue > 0,5 en el 81,8% de los casos, pero 2 tuvieron mala evolución (uno, con catarata y queratopatía en banda en ojo izquierdo y deterioro visual grave, precisó vitrectomía derecha y en otro se desarrolló hipotensión ocular grave). Aparecieron complicaciones en otros 2 pacientes: catarata y glaucoma en uno y sinequias iridianas sin glaucoma en otro. Conclusiones. Encontramos menor frecuencia de desprendimiento exudativo de retina y mayor de alteraciones dermatológicas que lo comunicado. Un porcentaje importante de casos precisó inmunosupresores. La agudeza visual final fue buena en la mayoría de los pacientes (AU)

Background. To describe the characteristics of a series of patients diagnosed of Vogt-Koyanagi-Harada disease (VKHD) and controlled by an Uveitis Unit (composed of ophthalmologistsand internists) in our population. Patients and method. Retrospective descriptive study of 11 patients with VKHD (5 males and 6 women; median age at diagnosis 32.6 years old) followed-up between 1980 and 2003. Results. All patients suffered panuveitis and/or exudative retinal detachment. Extraocular signs were present in all cases: neurological in 63.7% (aseptic meningitis and/or focal symptoms), cutaneous in 81.8% (vitiligo, whiteness, poliosis, alopecia), neurosensorial hypoacusis (50% of patients with audiometry), and general symptoms in 25%. They all received systemic corticosteroids. Cyclosporine was added in 5 patients (45.4%) with posterior uveitis, and azathioprine in 2 of them for iridocyclitis. The final visual acuity was 0.5 or better in 81.8% of cases, but 2 patients had an unfavourable evolution (one, who had cataract and band keratopathy in the left eye and severe visual worsening, needed right vitrectomy, and the other suffered severe ocular hypotension). Complications developed in 2 other patients: cataract and glaucoma in one, and synechiae without glaucoma in the other. Conclusions. We found less frequency of exudative retinal detachment and greater frequency of cutaneous signs than the communicated. A significant percentage of cases needed immunosuppressive agents. Final visual acuity was good in the majority of patients (AU)

[Vogt-Koyanagi-Harada disease. Characteristics of a series of Andalusian patients]. / Enfermedad de Vogt-Koyanagi-Harada. Características de un grupo de pacientes andaluces.

BACKGROUND: To describe the characteristics of a series of patients diagnosed of Vogt-Koyanagi-Harada disease (VKHD) and controlled by an Uveitis Unit (composed of ophthalmologists and internists) in our population. PATIENTS AND METHOD: Retrospective descriptive study of 11 patients with VKHD (5 males and 6 women; median age at diagnosis 32.6 years old) followed-up between 1980 and 2003. RESULTS: All patients suffered panuveitis and/or exudative retinal detachment. Extraocular signs were present in all cases: neurological in 63.7% (aseptic meningitis and/or focal symptoms), cutaneous in 81.8% (vitiligo, whiteness, poliosis, alopecia), neurosensorial hypoacusis (50% of patients with audiometry), and general symptoms in 25%. They all received systemic corticosteroids. Cyclosporine was added in 5 patients (45.4%) with posterior uveitis, and azathioprine in 2 of them for iridocyclitis. The final visual acuity was 0.5 or better in 81.8% of cases, but 2 patients had an unfavourable evolution (one, who had cataract and band keratopathy in the left eye and severe visual worsening, needed right vitrectomy, and the other suffered severe ocular hypotension). Complications developed in 2 other patients: cataract and glaucoma in one, and synechiae without glaucoma in the other. CONCLUSIONS: We found less frequency of exudative retinal detachment and greater frequency of cutaneous signs than the communicated. A significant percentage of cases needed immunosuppressive agents. Final visual acuity was good in the majority of patients.

Experiencia con rituximab en el tratamiento de pacientes con lupus eritematoso sistémico / Experience with rituximab in the treatment of systemic erythematosus lupus

Objetivo: Analizar la efectividad y tolerancia a corto plazo de la terapia con rituximab (RTX) en pacientes con lupus eritematoso sistémico (LES) y diferentes manifestaciones clínicas. Pacientes y métodos: Se estudió a un total de 13 pacientes. El RTX fue indicado en 6 ocasiones para el tratamiento de nefritis refractaria, en 5 para el tratamiento de trombocitopenia grave, en 1 para el tratamiento de aplasia medular y en 1 para el tratamiento de vasculitis peritoneal asociada a nefritis. Los 13 pacientes fueron tratados con 4 dosis semanales de 375 mg/m2 de RTX. El tiempo medio de seguimiento fue 12 ± 8,5 meses. Nueve pacientes experimentaron una respuesta favorable; 3 con nefropatía, 5 con trombocitopenia y 1 con vasculitis peritoneal y nefritis. El SLEDAI medio pasó de 11 a 6,5. Dos pacientes con trombocitopenia presentaron recidivas; y la respuesta al retratamiento fue buena. Conclusiones: El presente estudio demuestra que RTX es un fármaco efectivo y seguro, a corto plazo, en el tratamiento de diferentes manifestaciones clínicas asociadas al LES(AU)

Objective: To assess the short-term effectiveness and tolerance of rituximab in patients with systemic lupus erythematous and distinct clinical manifestations. Patients and methods: Thirteen patients were studied. Rituximab (RTX) was indicated for refractory nephritis in 6 patients, severe thrombocytopenia in 5, aplastic anemia in 1 and peritoneal vasculitis associated with nephritis in 1. All patients received 4 weekly doses of 375 mg/m2 of RTX. The mean length of follow-up was 12 ± 8.5 months. Response was favorable in 9 patients: 3 with nephritis, 5 with thrombocytopenia and 1 with peritoneal vasculitis and nephritis. The mean SLE disease activity index decreased from 11 to 6.5 points. Thrombocytopenia recurred in 2 patients, who responded well to retreatment. Conclusions: The present study demonstrates that RTX is safe and effective as short-term therapy for distinct clinical manifestations associated with SLE(AU)

[Experience with rituximab in the treatment of systemic erythematosus lupus]. / Experiencia con rituximab en el tratamiento de pacientes con lupus eritematoso sistémico.

OBJECTIVE: To assess the short-term effectiveness and tolerance of rituximab in patients with systemic lupus erythematous and distinct clinical manifestations. PATIENTS AND METHODS: Thirteen patients were studied. Rituximab (RTX) was indicated for refractory nephritis in 6 patients, severe thrombocytopenia in 5, aplastic anemia in 1 and peritoneal vasculitis associated with nephritis in 1. All patients received 4 weekly doses of 375 mg/m(2) of RTX. The mean length of follow-up was 12±8.5 months. Response was favorable in 9 patients: 3 with nephritis, 5 with thrombocytopenia and 1 with peritoneal vasculitis and nephritis. The mean SLE disease activity index decreased from 11 to 6.5 points. Thrombocytopenia recurred in 2 patients, who responded well to retreatment. CONCLUSIONS: The present study demonstrates that RTX is safe and effective as short-term therapy for distinct clinical manifestations associated with SLE.

Reacción de hipersensibilidad a ciclofosfamida en una paciente con lupus eritematoso sistémico / Hypersensitivy response to cyclophosphamide in a patient with systemic lupus erythematous

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Frecuencia de anticuerpos antinucleares en ancianos sanos / Frequency of antinuclear antibodies in the healthy elderly

Fundamento: Conocer la frecuencia de anticuerpos antinucleares (AAN) en población anciana en Andalucía. Pacientes y métodos: Se estudiaron 100 ancianos sanos (edad media, 81,6 años) y un grupo control de 199 donantes de sangre (edad media, 33,5 años). Los AAN se determinaron mediante inmunofluorescencia indirecta (IFI; sustratos triple de rata y HEp-2), los anticuerpos anti-ENA mediante contrainmunoelectroforesis y los anticuerpos anti-ADNn con IFI (Crithidia luciliae). Resultados: En ancianos, el título de AAN fue > 1/40 en el 51 por ciento y > 1/160 en el 36 por ciento (sustrato triple de rata), y > 1/40 en el 74 por ciento y > 1/160 en el 64 por ciento (sustrato HEp-2). El patrón más frecuente fue el moteado fino. Los anticuerpos anti-ADNn y antiENA fueron negativos. En controles, la frecuencia de AAN > 1/40 (HEp-2) fue del 7,5 por ciento (p < 0,001). Conclusión: La alta frecuencia de AAN en ancianos obliga a valorarlos con cautela en ausencia de indicios clinicobiológicos de enfermedad autoinmunitaria. (AU)

Lupus eritematoso sistémico inducido porinterferón alfa-2b en una paciente con infeccióncrónica por el virus de la hepatitis C / Alfa-2b interferon-induced systemic lupus erythematosus in a patient with hepatitis C virus chronic infection

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[Iliopsoas abscess and systemic lupus erythematosus]. / Absceso del músculo iliopsoas y lupus eritematoso sistémico.

Iliopsoas muscle abscess (IPA) is an uncommon condition, and it is usually associated with immunosuppression. Three out of a cohort of 552 patients diagnosed of systemic lupus erythematosus (SLE) developing an IPA, are reported herein. Patients showed fever and other symptoms related to SLE. They improved only partially under SLE therapy, and showed pain suggestive of IPA. It was confirmed by CT in all cases. S. aureus was isolated in one patient (primary IPA), and M. tuberculosis in the others. Specific antimicrobial therapy and surgical drainage were required. In summary, SLE might be considered as a risk condition for the development of IPA, due to the immunosuppression inherent in the disease and its treatment.

Absceso del músculo iliopsoas y lupus eritematoso sistémico / Iliopsoas abscess and systemic lupus erythematosus

El absceso del músculo iliopsoas (AIP) es una patología infrecuente, que se asocia a situaciones de inmunodeficiencia. Se presentan 3 casos de lupus eritematoso sistémico (LES), de una serie de 552 pacientes, que desarrollaron un AIP. El cuadro clínico se caracterizó por fiebre y otras manifestaciones relacionadas con su enfermedad de base, que cursaron de forma tórpida hasta que apareció dolor sugestivo de la existencia de un AIP. El agente etiológico fue S. aureus en un paciente (AIP primario), y M. tuberculosis en los otros 2 (AIP secundario). Para su tratamiento se requirió, además de antibioterapia específica, drenaje quirúrgico de la colección (fracaso previo del drenaje percutáneo en 2 casos). En la opinión de los autores, el LES, por la inmunodepresión subyacente relacionada tanto con la enfermedad en sí como con la medicación utilizada en el tratamiento, debe incluirse entre las circunstancias de riesgo para el desarrollo de AIP. (AU)

[The use of interferon alfa-2r in the treatment of autoimmune uveitis (primary or associated with Behçet's disease)]. / Utilización de interferón alfa 2r en el tratamiento de las uveítis autoinmunes (primarias o asociadas a enfermedad de Behçet).

BACKGROUND: The improvement in ocular inflammation in patients with cyclosporine A resistant Behçet disease (CyA) during therapy of severe aphthosis with recombinant interferon-alpha 2b (IFN alpha) prompted its evaluation for treatment of refractory autoimmune uveitis. METHOD: IFN alpha was used in 18 patients with posterior uveitis, nine with Behçet disease and nine with primary uveitis (mean evolution time 3.8 years) previously treated with corticosteroids (18), CyA (16) and azathioprine (1). The dose was 5 MU/day (4 weeks) and then twice weekly (12 weeks) maintaining the previous therapy. Evaluations were made at the start of the study, two weeks and two months and a cross-sectional study in February 1995. RESULTS: At the early phase both vitreal cellularity (p = 0.01) and macular edema (p = 0.003) improved; at the late phase improvements were noted in vitreal cellularity (p < 0.0001), macular (p < 0.0001) and papillar edema (p = 0.04) and visual acuity (p = 0.006). In February 1995 (mean evolution time 30 months), ten patients (56%) remain without treatment with inactive disease, six (33%) with CyA and two (11%) with CyA and IFN alpha. Improvements in vitreal cellularity (p = 0.0001), macular edema (p = 0.0001) and visual acuity (p = 0.013) were still present although macular ischemia was more severe than at the beginning (p = 0.035). The most important adverse reactions (late reactions) included depression (three cases) and thyroid changes (two cases). CONCLUSIONS: IFN alpha is an important therapeutic alternative for posterior uveitis refractory to corticosteroid therapy (included CyA).

[Serologic and molecular HLA typing in patients from Andalucia with Behçet's disease. Genetic and clinical correlations]. / Tipificación HLA serológica y molecular en pacientes andaluces con enfermedad de Behçet. Correlaciones geneticoclínicas.

BACKGROUND: HLA typing was performed in 67 patients with Behçet's disease from Andalucia, Spain to: 1) analyze the association of class I and II molecules with Behçet's disease in Spain; 2) study the clinical correlations and 3) evaluate its diagnostic and/or prognostic role. PATIENTS AND METHODS: 1) Serologic typing (microlymphocytotoxicity): class I molecules in 67 patients and class II molecules in 47 patients (controls: 223 healthy volunteers). 2) Oligotyping (PCR-SSO): class II molecules (DQB1 and generic DRB1) in 47 patients and 189 controls. 3) STATISTICAL ANALYSIS: chi square test (dicotomic variables) and Student t test (continuous variables), and calculation of the relative risk by the Wolff and Haldane formulas. RESULTS: The HLA B51 antigen was the most frequently observed in the whole series (p = 0.003) in males with ocular disease (p = 0.0001) and in patients with cutaneous (p = 0.001) and digestive involvement (p = 0.05). The HLA B51-positive males were younger at disease onset (p = 0.01) with neurologic involvement being infrequent (p = 0.03). The HLA B51 antigen was associated with neurologic (p = 0.06) and articular involvement (p = 0.05). The DQB1*0303 was associated with uveitis of bad evolution (p = 0.01). The DR11 and DQB1*0301 were more frequent in HLA B51-positive patients and the DQ5 was negatively associated with Behçet's disease, particularly in the HLA B51-positive patients. CONCLUSIONS: The study of the HLA antigens provides useful information for the diagnosis of Behçet's disease, aids in differentiating the different clinical forms and has prognostic significance.