{
  "abstract": "Introduction The history and pattern of progression of SSc-ILD are highly variable.No consensus exists regarding which assessment tools and predictors are most useful for monitoring disease progression.Thus,useful biomarkers associated with the progression of SSc-ILD are needed.Changes in the level of pneumoproteins as specific markers of lung damage and disease monitoring are of great interest.The greatest importance is attached to surfactant proteins A,D(Sp-A,Sp-D) and Krebs von den Lungen glycoprotein-6(KL-6).ObjectiveTo study the possibilities of using of KL-6,Sp-A,Sp-D for the diagnosis,assessment of the progression of ILD lesions and the effectiveness of therapy in SSc-ILD.Material and Methods 66 patients with SSc-ILD were enrolled into the study(SSc-duration-7.2±5.6 years,diffused/limited-1.7/1,average age-50.3 ± 12.6-years,females 77%).The duration of follow-up was 19±14.3 months.All patients received glucocorticoids and immunosuppressants,45(68%)-rituximab(RTM) at cumulative dose 2.4±1.5 grams.Subsequently correlation analysis was made to clarify the association of the KL-6,Sp-A,Sp-D with the FVC,DCLO,ILD-X-ray-symptoms, SSc-activity, laboratory parameters(ESR,CRP,ANA-HEP-2,a-Scl-70,B-cell-count),therapy.The control group included people without autoimmune diseases(n= 38).Results The levels of the studied biomarkers in the main group did not significantly differ from the control group. There was no significant correlation between the level of biomarkers and the presence of ILD according to MSCT, X-ray symptoms of ILD,FVC,DLCO.The exception was the level of Sp-D,which correlated with the presence of reticular changes(r=0.32).An inverse correlation was found between the level of KL-6 at the time of inclusion in the study and delta FVC(R=-0.310) on the background of active therapy.The level of KL-6 was significantly correlated with the B-cell count at the beginning of the study(R=0,306).A direct correlation of Sp-A with the activity of SSc(r= 0.339) and with the level of CRP(r = 0.510) and an inverse correlation with the skin score(r=-0.271) and with the total dose of rituximab(r=-0.38) was established.There was no correlation between the investigated biomarkers level with other studied indicators.Conclusions Among the biomarkers studied,none showed sufficient sensitivity to confirm the diagnosis of ILD.A higher level of Sp-A was detected in patients with more severe SSc.The association of a higher level of KL-6 with a lower increase in FVC during therapy may indicate the possibility of using KL-6 to predict the effectiveness of therapy and the progression of the disease.The association of the KL-6 level with the B cells count allows discussing the study of this biomarker to determine the indications for the appointment of anti-B cell therapy.The association of total RTM-dose with a decrease in Sp-A level probably indicates the potential role of Sp-A in evaluating the effectiveness of therapy.",
  "authors": [
    {
      "affiliations": [
        "V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia"
      ],
      "name": "Olga Konev"
    },
    {
      "affiliations": [
        "V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia"
      ],
      "name": "Liudmila Garzanova"
    },
    {
      "affiliations": [
        "V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia"
      ],
      "name": "Rushana Shayachmetova"
    },
    {
      "affiliations": [
        "V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia"
      ],
      "name": "Anastasia Avdeeva"
    },
    {
      "affiliations": [
        "V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia"
      ],
      "name": "Michail Diatroptov"
    },
    {
      "affiliations": [
        "V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia"
      ],
      "name": "Olga Ovsyannikova"
    },
    {
      "affiliations": [
        "V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia"
      ],
      "name": "Lidia Ananyeva"
    }
  ],
  "title": "P.101 Markers of alveolar epithelial injury in patients with systemic scleroderma and interstitial lung disease (SSc-ILD): clinical and laboratory associations",
  "uid": "4b9e521d-0891-5df7-8d34-7e556f8760ac"
}