title: Computed tomography findings of pulmonary venoocclusive disease in scleroderma patients presenting with precapillary pulmonary hypertension.
abstract: OBJECTIVE
  Pulmonary venoocclusive disease (PVOD) is an uncommon form of pulmonary hypertension (PH) characterized by obstruction of small pulmonary veins. Pulmonary venous involvement has been reported in pathologic assessment of patients with systemic sclerosis (SSc) presenting with precapillary PH. High-resolution computed tomography (HRCT) of the chest is a noninvasive diagnostic tool used to screen for PVOD. No HRCT data are available on SSc patients with precapillary PH. We undertook this study to evaluate the frequency and effect on prognosis of HRCT signs of PVOD in SSc patients with precapillary PH.
  
  
  METHODS
  We reviewed chest HRCT data from 26 SSc patients with precapillary PH and 28 SSc patients without pulmonary arterial hypertension (PAH) or interstitial lung disease (ILD).
  
  
  RESULTS
  The radiographic triad of HRCT signs of PVOD (lymph node enlargement [57.7% versus 3.6%], centrilobular ground-glass opacities [46.2% versus 10.7%], and septal lines [88.5% versus 7.1%]) was significantly more frequent in SSc patients with precapillary PH than in SSc patients without PAH or ILD (all P < 0.005). Indeed, 61.5% of SSc patients with precapillary PH had ≥ 2 of these signs. Cardiomegaly (P < 0.0001), pulmonary artery enlargement (P < 0.0001), and pericardial effusion (P < 0.0005) were also significantly more frequent in SSc patients with precapillary PH. Pulmonary venous involvement was histologically confirmed in 2 patients with radiographic signs of PVOD. The presence of ≥ 2 radiographic signs of PVOD was associated with the occurrence of pulmonary edema after initiation of PAH-specific therapy (in 8 of 16 patients) and with more rapid progression from diagnosis of PH to death.
  
  
  CONCLUSIONS
  HRCT signs of PVOD are frequently observed in SSc patients with precapillary PH, correlated with histologic assessment, and were associated with a high risk of pulmonary edema.
authors.affiliation: Université Paris-Sud, Hôpital de Bicêtre, AP-HP, Le Kremlin-Bicêtre, France.
authors.forename: S
authors.initials: S
authors.lastname: Günther
authors.forename: X
authors.initials: X
authors.lastname: Jaïs
authors.forename: S
authors.initials: S
authors.lastname: Maitre
authors.forename: A
authors.initials: A
authors.lastname: Bérezné
authors.forename: P
authors.initials: P
authors.lastname: Dorfmüller
authors.forename: A
authors.initials: A
authors.lastname: Seferian
authors.forename: L
authors.initials: L
authors.lastname: Savale
authors.forename: O
authors.initials: O
authors.lastname: Mercier
authors.forename: E
authors.initials: E
authors.lastname: Fadel
authors.forename: O
authors.initials: O
authors.lastname: Sitbon
authors.forename: L
authors.initials: L
authors.lastname: Mouthon
authors.forename: G
authors.initials: G
authors.lastname: Simonneau
authors.forename: M
authors.initials: M
authors.lastname: Humbert
authors.forename: D
authors.initials: D
authors.lastname: Montani
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000000981:diagnostic imaging*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications / Q000000981:diagnostic imaging*; D011859:Radiography; D012595:Scleroderma, Systemic / Q000150:complications / Q000000981:diagnostic imaging*