title: Pulmonary vasospasm in systemic sclerosis: noninvasive techniques for detection.
abstract: In a subgroup of patients with systemic sclerosis (SSc), vasospasm affecting the pulmonary circulation may contribute to worsening respiratory symptoms, including dyspnea. Noninvasive assessment of pulmonary blood flow (PBF), utilizing inert-gas rebreathing (IGR) and dual-energy computed-tomography pulmonary angiography (DE-CTPA), may be useful for identifying pulmonary vasospasm. Thirty-one participants (22 SSc patients and 9 healthy volunteers) underwent PBF assessment with IGR and DE-CTPA at baseline and after provocation with a cold-air inhalation challenge (CACh). Before the study investigations, participants were assigned to subgroups: group A included SSc patients who reported increased breathlessness after exposure to cold air (n = 11), group B included SSc patients without cold-air sensitivity (n = 11), and group C patients included the healthy volunteers. Median change in PBF from baseline was compared between groups A, B, and C after CACh. Compared with groups B and C, in group A there was a significant decline in median PBF from baseline at 10 minutes (-10%; range: -52.2% to 4.0%; P < 0.01), 20 minutes (-17.4%; -27.9% to 0.0%; P < 0.01), and 30 minutes (-8.5%; -34.4% to 2.0%; P < 0.01) after CACh. There was no significant difference in median PBF change between groups B or C at any time point and no change in pulmonary perfusion on DE-CTPA. Reduction in pulmonary blood flow following CACh suggests that pulmonary vasospasm may be present in a subgroup of patients with SSc and may contribute to worsening dyspnea on exposure to cold.
authors.affiliation: Royal Brompton Hospital, London, United Kingdom ; Princess Alexandra Hospital, Brisbane, Australia.
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Keir
authors.affiliation: Royal Brompton Hospital, London, United Kingdom.
authors.forename: Arjun
authors.initials: A
authors.lastname: Nair
authors.affiliation: Imperial College, London, United Kingdom.
authors.forename: Stamatia
authors.initials: S
authors.lastname: Giannarou
authors.affiliation: Imperial College, London, United Kingdom.
authors.forename: Guang-Zhong
authors.initials: GZ
authors.lastname: Yang
authors.affiliation: Royal Brompton Hospital, London, United Kingdom.
authors.forename: Paul
authors.initials: P
authors.lastname: Oldershaw
authors.affiliation: Royal Brompton Hospital, London, United Kingdom.
authors.forename: S John
authors.initials: SJ
authors.lastname: Wort
authors.affiliation: St. Vincent's Hospital, Sydney, Australia.
authors.forename: Peter
authors.initials: P
authors.lastname: MacDonald
authors.affiliation: Royal Brompton Hospital, London, United Kingdom.
authors.forename: David M
authors.initials: DM
authors.lastname: Hansell
authors.affiliation: Royal Brompton Hospital, London, United Kingdom.
authors.forename: Athol U
authors.initials: AU
authors.lastname: Wells
keywords: noninvasive; pulmonary vasospasm; scleroderma