Isolated pulmonary hypertension in diffuse cutaneous systemic sclerosis successfully treated with long-term plasma exchange.
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.
Ferri
C
C
Department of Internal Medicine, University of Pisa, Italy. c.ferri@int.med.unipi.it
Emdin
M
M
Storino
F A
FA
Giuggioli
D
D
Longombardo
G
G
Greco
F
F
Fertig
N
N
Medsger
T A
TA
D000328:Adult; D001324:Autoantigens / Q000097:blood; D002868:Chromosomal Proteins, Non-Histone / Q000097:blood; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000209:etiology / Q000628:therapy*; D010951:Plasma Exchange; D012595:Scleroderma, Systemic / Q000097:blood / Q000150:complications / Q000628:therapy*
D001324:Autoantigens; D002868:Chromosomal Proteins, Non-Histone; C047458:fibrillarin