[Iloprost for severe Raynaud's phenomenon and ischaemic ulcers related with systemic diseases]. / Iloprost en el fenómeno de Raynaud grave y las úlceras isquémicas de las enfermedades sistémicas.

BACKGROUND AND OBJECTIVE: To evaluate the long term efficacy of treatment with intravenous iloprost for severe Raynaud's phenomenon (RP) and ischemic leg ulcers in patients with autoimmune systemic diseases. PATIENTS AND METHOD: Prospective observational study over 2 years with iloprost (intravenous infusions, 0.5 to 2 ng/kg/min, initial cycle of 5 consecutive days and maintenance infusions during 24 h monthly, lengthened when it was needed) in patients with severe RP and ischemic leg ulcers whithout response to conventional therapy. Treatment was halted in patients with a good response after one year of treatment, with regular clinical controls. RESULTS: We treated 23 patients. Iloprost reduced significantly the mean number (SD) of monthly episodes of RP (150.38 [102.04] initially and 40.05 [78.06] at the end; p < 0.0005), the mean highest duration of episodes of RP (21.86 [26.96] min initially and 7.14 [9.87] min at the end; p = 0.013), the associated pain (p = 0.005), and the mean number of ischemic digital (4.25 [2.86] initially and 0.63 [2.25] at the end; p = 0.003) and leg ulcers (1.67 [0.52] initially and 0.33 [0.52] at the end; p = 0.01). Articular symptoms and inflammatory markers did not improve. Treatment was stopped in 8 patients (in 5 for a very good evolution and in 3 for other causes), and only 1 of them needed to be treated again. Side effects were seen in all cases but always disappeared after slowing infusion. CONCLUSIONS: Iloprost was effective in the long term treatment of severe RP and ischemic leg ulcers in patients with autoimmune systemic diseases.

[Combined treatment with intravenous prostacyclin and sildenafil in patients with pulmonary hypertension: report of 4 cases]. / Tratamiento combinado con prostaciclina intravenosa y sildenafilo en pacientes con hipertensión arterial pulmonar: descripción de 4 casos.

BACKGROUND AND OBJECTIVE: Here we report the experience obtained from a combined treatment with intravenous (i.v) prostacyclin and oral sildenafil in patients with severe pulmonary hypertension (PHT) who had a poor response to prior treatment with prostacyclin alone. PATIENTS AND METHOD: Sildenafil was added to the treatment in four patients with PHT (primary in two patients and secondary to collagenosis in the other two) with no adequate response to i.v. prostacyclin treatment. The clinical course, 6minutes walking test and echocardiogram were evaluated. RESULTS: Initial sildenafil dose was 12.5 mg three times daily, which was increased up to 50 mg three times daily in one patient and up to 50 mg four times daily in the other three. The symptoms of right heart failure were controlled in all cases. Before the start of sildenafil administration, two patients had class III dyspnea and two patients had class IV dyspnea. Two patients converted to class I (previously class III and IV), and the other two converted to class II. The distance walked within 6 minutes increased (average increase 55%) and systolic pulmonary artery pressure decreased in all patients (average reduction 27%). Effects of sildenafil were substained. The only side effect seen was mild headache. CONCLUSIONS: Our experience supports the value of sildenafil in the treatment of PHT and suggests that combined treatment is useful for rescuing patients who fail to respond to initial treatment with i.v. prostacyclin.

Tratamiento combinado con prostaciclina intravenosa y sildenafilo en pacientes con hipertensión arterial pulmonar: descripción de 4 casos / Combined treatment with intravenous prostacyclin and sildenafil in patients with pulmonary hypertension: report of 4 cases

FUNDAMENTO Y OBJETIVO: Se describe la experiencia con el tratamiento combinado con prostaciclina intravenosa y sildenafilo oral en pacientes con hipertensión arterial pulmonar grave con mala respuesta a prostaciclina sola. PACIENTES Y MÉTODO: Se asoció sildenafilo al tratamiento en 4 pacientes con hipertensión arterial pulmonar (primaria en dos y secundaria a colagenosis en dos) sin respuesta favorable a prostaciclina intravenosa. Se valoró la evolución clínica, la prueba de caminar durante 6 min y el ecocardiograma. RESULTADOS: La dosis inicial de sildenafilo fue 12,5 mg tres veces al día, con incremento hasta 50 mg tres veces al día en un paciente y 50 mg 4 veces al día en los otros tres. Se controlaron los síntomas de insuficiencia cardíaca derecha en todos los casos. Antes de iniciar la administración de sildenafilo, dos pacientes tenían disnea de clase III y dos de clase IV. Dos pacientes (antes clases III y IV, respectivamente) pasaron a clase I y los otros dos pasaron a clase II. Tras alcanzar la dosis final, la distancia caminada en 6 min aumentó (incremento medio del 55 por ciento) y la presión arterial pulmonar se redujo (porcentaje medio de reducción del 27 por ciento para la sistólica) en todos los pacientes, efecto que se mantuvo durante el seguimiento. El único efecto secundario observado fue cefalea leve. CONCLUSIONES: Nuestra experiencia apoya la posible utilidad del sildenafilo en el tratamiento de la hipertensión arterial pulmonar y sugiere su utilidad en tratamiento combinado para rescatar a pacientes que no responden al tratamiento inicial con prostaciclina intravenosa (AU)

Angina vasospástica: una manifestación excepcional en el síndrome de Churg-Strauss / Prinzmetal´s angina pectoris: an usual clinical manifestation of Churg-Strauss syndrome

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[Usefulness of brain SPECT with HMPAO-99mTc and psychological tests for diagnosis of neurological involvement in Behçet's disease]. / Utilidad de la SPECT cerebral con HMPAO-99mTc y de los tests psicométricos en el diagnóstico de la afección neurológica de la enfermedad de Behçet.

BACKGROUND: The purpose of this study was to establish the usefulness of single photon emission computed tomography (SPECT) and psychological tests for diagnostic of neuro-Behçet (NB) and to evaluate the clinical significance of neurological symptoms that are difficult to interpret and asymptomatic abnormalities in diagnostic tests. PATIENTS AND METHOD: Forty patients with Behçet's disease (BD) were enrrolled for being studied with magnetic resonance imaging (MRI), SPECT and psychological tests. RESULTS: MRI findings were abnormal in 52,9% of patients with neurological involvement and 23.1% without it (p < 0.1), whereas SPECT findings were abnormal in 82.3% and 61.5%, respectively (no significant difference). The difference between MRI and SPECT findings was significant (p < 0.02 for the complete group; p < 0.05 for patients without neurological symptoms; p < 0.08 for patients with them). The mean follow-up period was 42.6 months, and no patient without neurological involvement or those only with neurological symptoms that are difficult to interprete developed definite neurological involvement. The results of cognitive tests were not significantly different among patients with or without neurological involvement, neither among patients and controls. The scale 2 (depression) of the personality test was more frequent in patients with definite neurological involvement (p < 0.05). CONCLUSIONS: SPECT seems more sensible and less specific than MRI for diagnostic of NB. Although SPECT findings were frequently abnormal in patients with BD without neurological involvement or with neurological symptoms hard to interpret, no patient from this group developed a NB flare after a long follow-up period. A characteristic personality was found for patients with BD.

Utilidad de la SPECT cerebral con HMPAO-99mTc y de los tests psicométricos en el diagnóstico de la afección neurológica de la enfermedad de Behçet / Usefulness of brain SPECT with HMPAO-99mTc and psychological tests for diagnostic of neurological involvement in Behçet's disease

FUNDAMENTO: Establecer el papel de la tomografía computarizada por emisión de fotón simple (SPECT) y los tests psicológicos en el diagnóstico del neuro-Behçet (NB) y evaluar la significación de los síntomas neurológicos de difícil interpretación y las alteraciones asintomáticas en las pruebas diagnósticas. PACIENTES Y MÉTODO: Se estudió a 40 pacientes con enfermedad de Behçet (EB) mediante resonancia magnética (RM), SPECT y tests psicológicos. RESULTADOS: La RM estaba alterada en el 52,9 por ciento de los pacientes con clínica neurológica y en el 23,1 por ciento sin síntomas neurológicos (p < 0,1), mientras que la SPECT lo estaba en el 82,3 y el 61,5 por ciento, respectivamente (no significativo). La diferencia entre hallazgos en RM y SPECT fue significativa (p < 0,02 para el grupo completo; p < 0,05 para pacientes sin antecedentes neurológicos; p < 0,08 para aquellos con clínica neurológica). Tras 42,6 meses de seguimiento medio, ningún paciente sin antecedentes neurológicos o con alteraciones aisladas de difícil interpretación desarrolló síntomas de afección neurológica definida. Los resultados de los tests cognitivos de pacientes con y sin antecedentes neurológicos no difirieron significativamente, y tampoco los de pacientes y controles. En el test de personalidad, la escala 2 (depresión) fue significativamente más frecuente entre pacientes con afección neurológica definida (p < 0,05). CONCLUSIONES: La SPECT parece más sensible y menos específica que la RM en el diagnóstico del NB. Aunque la SPECT estaba frecuentemente alterada en pacientes con EB sin antecedentes neurológicos o con clínica de difícil interpretación, ninguno desarrolló clínica neurológica definida tras su seguimiento prolongado. Se encontró un perfil de personalidad característico de los pacientes con EB. (AU)