Ocular involvement in primary antiphospholipid syndrome: results of an extensive ophthalmological evaluation performed in the APS-Rio cohort.

OBJECTIVE: To study ophthalmological manifestations in a well-characterized primary antiphospholipid syndrome (PAPS) cohort (APS-Rio) and compare them with a healthy control group. METHODS: We examined PAPS patients and controls with an extensive ophthalmological evaluation, which included anamnesis, visual acuity, slit-lamp biomicroscopy, binocular indirect ophthalmoscopy, and retinography of the anterior and posterior segments of the eye. PAPS group also underwent angiography exam and optical coherence tomography using spectral domain technology (SD-OCT). RESULTS: 98 PAPS patients and 102 controls were included. The most common symptom in PAPS was amaurosis fugax (34.7% vs. 6.9%; p = .001). In the multivariate analyses, Raynaud's phenomenon was associated with amaurosis fugax (OR 3.71, CI:1.33-10.32; p = .012), and livedo correlated with hemianopia (OR 6.96, CI:1.11-43.72, p = .038) and diplopia (OR 3.49, CI:1.02-11.53, p = .047). After ophthalmological evaluation, 84 PAPS patients had ocular involvement (1.0% glaucoma, 94.0% posterior findings, 62.7% anterior findings, and 56.6% both posterior and anterior findings). Vascular tortuosity was more frequent in the PAPS group (63.2% vs. 42.2%; p = .002), as well as peripheral tortuosity (29.6% vs. 7.8%; p < .001). After excluding patients with atherosclerotic risk factors, peripheral vascular tortuosity was still statistically associated with PAPS (35.0 vs. 7.8%, p < .001). Triple positivity was more frequent in PAPS patients with peripheral vascular tortuosity than in those without this ocular finding (34.5% vs. 15.9%, p = .041). CONCLUSION: Vasomotor phenomena are importantly related to ocular symptoms in PAPS. Vascular tortuosity was a frequent finding in PAPS patients. Peripheral vascular tortuosity was associated with triple positivity and might be a biomarker of ischemic microvascular retinopathy due to PAPS.

Gastrinoma parapapilar: relato de caso / Parapapillary gastrinoma: a case report

Os tumores neuroendócrinos do duodeno compreendem 2-3 por cento de todos os tumores endócrinos do trato gastrointestinal, sendo o gastrinoma o mais freqüente, representando aproximadamente 48 por cento dos casos. Essas lesões, em 90 por cento dos ca»os, estão localizadas no triângulo do gastrinoma, que compreende, entre outras regiões, a primeira, segunda e terceira porção duodenal. Cerca de 25 por cento dessas lesões estão associadas à neoplasia endócrina múltipla tipo 1 (NEM-l). O presente relato demonstra paciente com familiares de primeiro grau portadores da síndrome e que estava em programa de rastreamento para pesquisa de NEM-l familiar. Durante a realização de ultra-som endoscópico, evidenciou-se lesão em região parapapilar com origem na camada mucosa de parede duodenal. Foram realizadas pun-ções-biópsias ecoguiadas e a análise histopatológica e imunohistoquímica revelou tratar-se de neoplasia endócrina produtora de gastrina. O estudo relatado evidencia um raro caso de gas-trinoma de região parapapilar em indivíduo portador de NEM-l.