title: Screening for pulmonary arterial hypertension in patients with scleroderma--a New Zealand perspective.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) in scleroderma (SSc) patients is a devastating complication with high mortality if untreated. Early recognition and specific treatment of PAH may improve outcome. Regular interval screening for PAH is generally recommended in scleroderma patients especially with the availability of emerging new therapies. The aim of this study is to determine the self-reported screening and treatment practices for SSc-PAH amongst rheumatologists in New Zealand (NZ).
  
  
  METHODS
  An anonymous online questionnaire survey was emailed to all rheumatologists in New Zealand.
  
  
  RESULTS
  Responses were received from 65% (39/60) of rheumatologists. The majority of patients had limited SSc (lcSSc) (57%) versus diffuse SSc (dcSSc) (34%). Twelve percent of patients had PAH. Eighty-two percent of rheumatologists screened for PAH in all SSc patients regardless of symptoms. The most commonly used screening modalities were pulmonary function tests (PFT) (97%) followed by clinical examination (95%) and echocardiogram (TTE) (92%). The majority of rheumatologists performed screening tests on a yearly basis (80% used PFT and 64% used TTE). A right heart catheter was used to confirm PAH in 70% of patients. Sixty-four percent of rheumatologists extend screening interval time if their patients were clinically stable. The most common PAH-specific therapy used was sildenafil (57%) followed by bosentan (19%). Sixty-four percent of rheumatologists supported a national PAH-SSc screening guideline.
  
  
  CONCLUSIONS
  This study has shown a wide variability of how NZ rheumatologists screen for PAH in scleroderma patients. The development of a PAH-SSc guideline for screening and diagnosis may help standardise treatment practices in NZ.
authors.forename: Sanjib K
authors.initials: SK
authors.lastname: Ghosh
authors.forename: Michael M
authors.initials: MM
authors.lastname: Corkill
authors.forename: Hamish H
authors.initials: HH
authors.lastname: Hart
authors.affiliation: Department of Medicine (Rheumatology), North Shore Hospital, Private Bag 93503, Takapuna, Auckland 0740, New Zealand. kristine.ng@waitematadhb.govt.nz.
authors.forename: Kristine P
authors.initials: KP
authors.lastname: Ng
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D006328:Cardiac Catheterization / Q000706:statistics & numerical data; D004452:Echocardiography / Q000706:statistics & numerical data; D004562:Electrocardiography / Q000706:statistics & numerical data; D005080:Exercise Test / Q000706:statistics & numerical data; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000188:drug therapy; D008403:Mass Screening / Q000379:methods / Q000706:statistics & numerical data*; D009520:New Zealand / Q000453:epidemiology; D010808:Physical Examination / Q000706:statistics & numerical data; D010879:Piperazines / Q000627:therapeutic use; D010818:Practice Patterns, Physicians' / Q000706:statistics & numerical data*; D011687:Purines / Q000627:therapeutic use; D012129:Respiratory Function Tests / Q000706:statistics & numerical data; D012219:Rheumatology; D012595:Scleroderma, Systemic / Q000150:complications / Q000453:epidemiology*; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use; D013450:Sulfones / Q000627:therapeutic use; D011795:Surveys and Questionnaires; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate; D000077300:Bosentan