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Isolated pulmonary hypertension in diffuse cutaneous systemic sclerosis successfully treated with long-term plasma
C Ferri1, M Emdin, F A Storino
1Department of Internal Medicine, University of Pisa, Italy. c.ferri@int.med.unipi.it
Scandinavian Journal of Rheumatology
|July 18, 2000
Abstract:
Isolated pulmonary hypertension (PHT) in patients with diffuse cutaneous systemic sclerosis (dSSc) is one of the most severe complication. Here we report the case of a 22 year-old white woman with anti-U3RNP antibody-positive dSSc complicated by severe, isolated PHT successfully treated with long-term plasma exchange. This beneficial effect persisted for two years, even after plasma exchange discontinuation. This is the first observation of isolated PHT in dSSc responsive to plasma exchange therapy.