title: Treatment of pulmonary hypertension in patients with connective tissue disease and interstitial lung disease.
abstract: BACKGROUND
  Pulmonary hypertension (PH) in patients with connective tissue disease (CTD) can occur in isolation or concomitantly with interstitial lung disease (ILD). Targeted therapies for PH can mitigate clinical deterioration in CTD patients with isolated PH; however, the effect of these therapies in CTD patients with PH and ILD (CTD-PH-ILD) are poorly characterized.
  
  
  OBJECTIVE
  To investigate outcomes following long-term treatment of PH in patients with CTD-PH-ILD.&nbsp;
  
  
  METHODS
  &nbsp;&nbsp;A retrospective evaluation of 13 CTD-PH-ILD patients who were treated with bosentan, sildenafil or bosentan plus sildenafil, was conducted. Immunosuppressants were prescribed as indicated. Patients underwent pulmonary function testing and assessment of 6 min walk distance at the time of treatment initiation and during follow-up. Patients were followed until time of death, lung transplantation or the end of the study. Kaplan-Meier estimates of survival were calculated and log-rank testing was used to analyze survival differences according to CTD subtype.
  
  
  RESULTS
  Thirteen patients (seven with systemic sclerosis [SSc], four with overlap syndrome, and two with rheumatoid arthritis) were followed for a mean (± SD) duration of 33.8±21.7 months. The survival estimate at a median duration of 34 months was 85%; two patients with SSc died. Mortality rates were greater among patients with SSc versus other CTD subtypes (P=0.04). No changes from baseline to follow-up in mean forced vital capacity or exercise capacity, and no treatment-related toxicity, were observed.
  
  
  CONCLUSIONS
  Treatment using PH-specific therapies in patients with CTD, PH and ILD was well tolerated. Further studies to investigate the efficacy of PH-specific therapies in CTD-PH-ILD patients are warranted.
authors.affiliation: Department of Medicine, University of Manitoba, Winnipeg, Manitoba. smittoo@mtsinai.on.ca
authors.forename: Shikha
authors.initials: S
authors.lastname: Mittoo
authors.forename: Thomas
authors.initials: T
authors.lastname: Jacob
authors.forename: Andrea
authors.initials: A
authors.lastname: Craig
authors.forename: Zoheir
authors.initials: Z
authors.lastname: Bshouty
mesh_terms: D000328:Adult; D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D003240:Connective Tissue Diseases / Q000150:complications* / Q000188:drug therapy / Q000401:mortality; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality; D053208:Kaplan-Meier Estimate; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000188:drug therapy / Q000401:mortality; D008297:Male; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use*; D010879:Piperazines / Q000627:therapeutic use*; D011687:Purines / Q000627:therapeutic use; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use*; D013450:Sulfones / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D058986:Phosphodiesterase 5 Inhibitors; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D000068677:Sildenafil Citrate; D000077300:Bosentan