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Neurological involvement as a poor prognostic factor in catastrophic antiphospholipid syndrome: autopsy findings in 12 cases

Rheumatology Department, Hospital de Especialidades, Centro Médico la Raza IMSS, Mexico City, Mexico

L J Jara

Clinical Epidemiology Research Unit, Hospital Especialidades, Centro Médico la Raza IMSS, Mexico City, Mexico

M Becerra

Pathology Department, Hospital de Especialidades, Centro Médico la Raza IMSS, Mexico City, Mexico

R Ariza

Internal Medicine Department, Hospital de Especialidades, Centro Médico la Raza IMSS, Mexico City, Mexico

L Espinoza

Section of Rheumatology, LSU Health Sciences Center, New Orleans, USA

W Wilson

Section of Rheumatology, LSU Health Sciences Center, New Orleans, USA

L Barile-Fabris

Clinical Epidemiology Research Unit, Hospital de Especialidades, Centro Médico Nacional SXXI IMSS, Mexico City, Mexico, lbarile{at}infosel.net.mx

CAPS is an uncommon disease, characterized by clinical evidence of multiple organ involvement and histopathological evidence of multiple vessel occlusions, in patients with either primary or secondary antiphospholipid syndrome. The present series describes the clinical manifestations and autopsy findings of 12 patients with CAPS. Neurological involvement was considered the main cause of death in all of them. CNS pathology revealed thrombotic microangiopathyas well as small and large vessel occlusions in several brain areas. Neurological involvement in CAPS is strongly associated with thrombotic microangiopathy and should be considered a potential cause of death in these patients.

Key Words: autopsy findings in catastrophic antiphospholipid syndrome • catastrophic antiphospholipid syndrome • neurological involvement in antiphospholipid syndrome

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