[Idiopathic portal hypertension, multiple focal nodular hepatic hyperplasia, the CREST syndrome and protein S deficiency]. / Hipertensión portal idiopática, hiperplasia nodular focal hepática múltiple, síndrome de CREST y déficit de proteína "S".

A 54 year old woman with idiopathic portal hypertension, CREST syndrome, protein "S" deficiency and multiple focal nodular hyperplasia is reported. The patient presented several episodes of upper digestive bleeding due to portal hypertension gastropathy and to esophageal varices. Treatment with propanolol and isosorbide 5-mononitrate failed to control recurrent bleeding and a portacaval shunt was performed. At operation, ten hepatic nodes were found, being diagnosed as focal nodular hyperplasia in the pathologic study. Congestive gastropathy, esophageal varices and splenomegaly disappeared after portacaval shunt and bleeding did not recur after a follow-up of twelve months. This results support the role of portal decompressive surgery in the bleeding due to portal hypertension gastropathy.

[Necrotizing vasculitis of the panarteritis nodosa type in a long-course primary Sjögren's syndrome]. / Vasculitis necrotizante tipo panarteritis nodosa en un síndrome de Sjögren primario de larga evolución.

Vasculitis is a complication happening in a third of patients with primary Sjögren's Syndrome. It can be of 2 types: neutrophilic or mononuclear and sometimes mixed. Very occasionally, a necrotizing vasculitis polyarteritis nodosa type during the evolution of Sjögren's Syndrome has appeared. A case of a female patient with a Sjögren's Syndrome of large evolution, who suddenly showed a poly-systemic affliction (nervous system, kidneys, muscles and digestive system) secondary to a necrotizing vasculitis type polyarteritis nodosa with a good response to immunosuppressors, is presented.