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Pulmonary hypertension associated with non-cirrhotic portal hypertension in systemic lupus erythematosus.
  1. D. Woolf,
  2. M. D. Voigt,
  3. K. Jaskiewicz,
  4. A. A. Kalla
  1. Rheumatic Diseases Unit, University of Cape Town, Observatory, South Africa.

    Abstract

    A case of non-cirrhotic portal hypertension in a patient with systemic lupus erythematosus, the first of our knowledge, is described. Severe pulmonary hypertension was associated with the portal hypertension and with markers of active auto-immunity. Pulmonary hypertension has not previously been associated with non-cirrhotic portal hypertension. The coexistence of vasculopathy of the portal and pulmonary vascular beds in this patient with active autoimmunity supports the postulate that portal-pulmonary hypertension may be immunologically mediated.

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