title: [Markers of thrombophilia in pulmonary fibrosis complicated by pulmonary hypertension].
abstract: OBJECTIVE
  To determine the role of enhanced blood coagulation in pathogenesis of pulmonary hypertension (PH) at an early stage of fibrosing alveolitis (FA).
  
  
  METHODS
  Clinical, functional, roentgenological, coagulation and immunological examinations were performed in 17 patients with idiopathic FA (IFA), in 6 patients with exogenic allergic alveolitis (EAA), in 15 FA patients with diffuse diseases of the connective tissue (FA-DDCT). The diagnosis was verified with high resolution computed tomography (HRCT). Lesser circulation was assessed by Doppler echocardiography. Morphological impairment of the lungs was specified in all the patients using analysis of the bronchoalveolar lavage. In 9 FA patients the diagnosis was verified at thoracoscopic biopsy of the lung. The control group consisted of 16 healthy volunteers. Thrombin-antithrombin complex (TAT) and thrombocytic factor 4 (TF-4) were estimated with ELISA as stable, highly sensitive markers of thrombophilia.
  
  
  RESULTS
  The TF-4 level was elevated in all IPD patients (p < 0.05), the elevation being highest in FA-DDCT (p < 0.007). With FA progression, TF-4 concentration went down. A weak negative correlation (p < 0.047, r = -0.38) was found with average pressure in the pulmonary artery (PAAP). TAT was higher than control in all the groups (p < 0.05). Maximal TAT values were registered in EAA. If HRCT detected active inflammation and in development of irreversible fibrous changes TAT was higher vs control. A direct correlation between TAT level and PAAP was not found.
  
  
  CONCLUSIONS
  Disorders in thrombocytic and plasmic links of hemostasis are detectable early in IPD. Stable markers of thrombophilia (TAT and TF-4) reflect activity of inflammation in FA. They can be also used as sensitive diagnostic tests for diagnosis of PH and diagnosis of patients with activated coagulation system in IPD.
authors.forename: D V
authors.initials: DV
authors.lastname: Arkhipova
authors.forename: E V
authors.initials: EV
authors.lastname: Privalova
authors.forename: L V
authors.initials: LV
authors.lastname: Kozlovskaia
authors.forename: B M
authors.initials: BM
authors.lastname: Kornev
authors.forename: E N
authors.initials: EN
authors.lastname: Popova
authors.forename: V V
authors.initials: VV
authors.lastname: Kraeva
authors.forename: V I
authors.initials: VI
authors.lastname: Osipenko
authors.forename: V O
authors.initials: VO
authors.lastname: Bitsadze
mesh_terms: D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000097:blood; D001775:Blood Circulation / Q000502:physiology; D001777:Blood Coagulation / Q000502:physiology; D001779:Blood Coagulation Factors / Q000032:analysis*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood* / Q000150:complications / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000097:blood* / Q000150:complications / Q000503:physiopathology; D019851:Thrombophilia / Q000097:blood* / Q000209:etiology / Q000503:physiopathology
chemical_list: D015415:Biomarkers; D001779:Blood Coagulation Factors