Síndrome antifosfolípido catastrófico: caso clínico / Catastrophic antiphospholipid syndrome: clinical case

El síndrome antifosfolípido (SAF) fue descrito y caracterizado durante la segunda mi-tad del siglo XX inicialmente como un fenómeno protrombótico secundario en con-texto de otras enfermedades del tejido conectivo, principalmente lupus. Sin embargo, el estudio de pacientes con enfermedad primaria impulsó a distintos consensos, tan-to clínicos como de laboratorio para su correcta identificación. Entre los pacientes con SAF destaca la forma de presentación catastrófica, de baja prevalencia, pero impor-tante por su mal pronóstico, caracterizada por el compromiso de múltiples sistemas en corto tiempo. Presentamos el caso de una paciente del Hospital Clínico San Borja-Arriarán con diag-nóstico de SAF primario, que presentó en su evolución la forma catastrófica. Este caso sirve de base para una revisión del proceso diagnóstico del SAF en relación a otras patologías reumatológicas y las características propias del SAF catastrófico.

Antiphospholipid syndrome (APS) was described and characterized during the second half of the 20th century initially as a secondary prothrombotic phenome-non in the context of other connective tissue diseases, mainly lupus. However, the study of patients with primary disease prompted different consensus, both clin-ical and laboratory for their correct identification. Among patients with APS, the catastrophic presentation is of low prevalence, but important because of its poor prognosis, characterized by the commitment of multiple systems in a short time. We present the case of a patient from the San Borja-Arriaran Clinic Hospital with di-agnosis of primary APS, which presented the catastrophic form in its evolution. This case serves as a basis for a review of the diagnostic process of APS in relation to other rheumatologic pathologies and the characteristics of catastrophic APS.

Primary Antiphospholipid Antibody Syndrome: Neuro radiologic Findings in 11 Patients

OBJECTIVE: To describe the neuroradiologic findings of primary antiphospholipid antibody syndrome (PAPS). MATERIALS AND METHODS: During a recent two-year period, abnormally elevated antiphospholipid antibodies were detected in a total of 751 patients. In any cases in which risk factors for stroke were detected-hypertension, diabetes mellitus, hyperlipidemia, smoking, and the presence of SLE or other connective tissue diseases-PAPS was not diagnosed. Neuroradiologic studies were performed in 11 of 32 patients with PAPS. We retrospectively reviewed brain CT (n = 7), MR (n = 8), and cerebral angiography (n = 8) in 11 patients with special attention to the presence of brain parenchymal lesions and cerebral arterial or venous abnormalities. RESULTS: CT or MR findings of PAPS included nonspecific multiple hyper-inten-sity foci in deep white matter on T2-weighted images (5/11), a large infarct in the territory of the middle cerebral artery (4/11), diffuse cortical atrophy (2/11), focal hemorrhage (2/11), and dural sinus thrombosis (1/11). Angiographic findings were normal (5/8) or reflected either occlusion of a large cerebral artery (2/8) or dural sinus thrombosis (1/8). CONCLUSION: Neuroradiologic findings of PAPS are nonspecific but in young or middle-aged adults who show the above mentioned CT or MR findings, and in whom risk factors for stroke are not present, the condition should be suspected.