The authors present a patient with Sjögren's syndrome with a fluctuating and then progressive myelopathic syndrome, and optic nerve involvement. Treatment with chlorambucil and prednisone improved the patient's function from being wheelchair bound to walking unaided. Spinal MRI showed multiple, extensive intraparenchymal areas of abnormal T2-weighted signal intensity, gadolinium enhancement, and cord swelling, which also improved during the period of treatment.

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Objectives To analyze the demographic, clinical, laboratory features and outcome of patients with EGPA in a large cohort of Spanish patients with AAV Methods multicenter retrospective-longitudinal study that included patients diagnosed with AAV between January 1995 and December 2012 in 19 Hospitals from Spain (REVAS Study). Statistical analysis was performed using the SPSS vs17. Results 87 patients with EGPA (mean age 52.5±16.1 yr) from 405 with AAV were analyzed. Asthma was present in all cases and preceded EGPA by 6 months to 29 yr (mean 81.6 mo) except in 6 cases in which began simultaneously. A history of allergic rhinitis was noted in 42.5% cases with nasal polyposis in 23% and recurrent sinusitis in 49.4%. ANCA were positive in 66% cases (91% MPO ANCA). Mean follow-up was 82.5±75.3 months. The most frequent clinical manifestations at diagnosis were fever (44%), arthralgia (47%), constitutional symptoms (41%), neurological symptoms (54%), and purpura (35%). Renal failure was present in 14% cases, reno-pulmonary sd. in 4.6% and cardiac involvement in 27.6%. Renal and neurological involvement were more frequent in positive ANCA patients (p=0.005) and cardiac involvement in ANCA negative (p=0.001). Chest-x-ray showed lung infiltrates (56.3%) and nodules (16%). Eosinophilia was present in all cases. Serum IgE levels were raised in 73.3% of tested patients. A total of 101 biopsies were done (25 nerve,15 lung,6 renal,7 nasal,38 skin,4 bowel,2 pericardial,2 liver,2 pleural) and showed eosinophil infiltrates in 52 cases and necrotizing vasculitis in 47. Oral prednisone (1mg/kg) was given to all patients, iv metilprednisolone to 45%, iv cyclo-phosphamide (CF) to 41.4%, oral CF to 24.1%, azathioprine to 21.8%, and mycophenolate to 5.7%. Rituximab was successfully administered in 2 cases with refractory disease. Leukopenia was more frequent in patients treated with oral CF (p<0.000). During the follow-up, 27.6% patients developed bacterial infections, 11.5% opportunistic infections and 9 (10.3%) died. Dead was mainly related to infections (p=0.011) and refractory disease. EGPA patients had less renal involvement (p=0.005) and more cardiac involvement (p=0.002) than those with GPA and MPA and a lower mortality (p=0.001) Conclusions patients with EGPA have less severe clinical manifestations than those with GPA and MPA and a lower mortality. Renal involvement is rare and cardiac involvement frequent, as previously described. Most patients with FFS>1 improve with corticosteroids and CF. RTX may be useful in patients with refractory disease. The overall EGPA survival rate is good and dead is mainly related to infections and refractory disease Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4714

Introducción: el estudio de intestino delgado es un desafío, motivo de avance médico y de interés. La enteroscopia de doble balón (EDB) permite una visualización directa del intestino delgado. Objetivo: describir indicaciones, características del procedimiento, hallazgos, complicaciones y seguimiento de pacientes llevados a EDB en el Hospital de San José de Bogotá, entre noviembre de 2011 y abril de 2019. Metodología: estudio de serie de casos. Se incluyeron pacientes con indicación clínica o por imágenes diagnósticas sugerentes de lesión a nivel de intestino delgado. Se empleó estadística descriptiva. Resultados: se realizaron 45 enteroscopias a 44 pacientes, con una mediana de edad de 58 años. La principal indicación fue hemorragia digestiva potencial de intestino delgado (53,3 %) y diarrea crónica (11,1 %); fueron más frecuentes los procedimientos por vía anterógrada. La mayoría de los resultados fue normal (46,7 %); entre los hallazgos más frecuentes estuvieron los tumores (11,1 %), enfermedad de Crohn (8,9 %) y úlceras/erosiones (6,7 %). El rendimiento diagnóstico fue 53,3 %, la correlación con la videocápsula endoscópica (VCE) fue 37,5 % y con estudios de imagen, 42,9 %. No se presentaron complicaciones. Se hizo seguimiento al 91,1 % de los pacientes con una mediana de 56 meses y se observó la resolución de síntomas en el 78,1 %. La finalidad de la enteroscopia fue diagnóstica en el 84,4 %. Conclusiones: La EDB es útil en la evaluación del intestino delgado con posibilidad terapéutica. Los resultados son similares a los reportados en la literatura mundial. Si se realiza seguimiento, se puede definir la necesidad de repetición del procedimiento, realización de estudios adicionales u observar resolución de los síntomas.