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The course of systemic scleroderma (SSD) in 80% of patients (pts) is complicated by pulmonary fibrosis (PF) with the pulmonary insufficiency PF progression. <bold>Objective:</bold> to determine additional prognostic markers of the activity of fibrous process formation in the lungs. Materials and methods. A prospective study involved 28 pts with SSD with PF, 2 men and 26 women. Examination: general clinical data, mMRS scale, computer tomography, spirography, blood trypsin-like activity (TLA), STATISTICA 10.0 program. <bold>Results:</bold> Complaints of cough and dyspnea in 61% of pts with SSD and PF and in 18% without PF. According to the mMRC scale and spirogram indicators, pts with SSD and PF were divided into group I (7 pts) with severe manifestations of PI and group II – moderately severe PI – 21 pts. The TLA median in pts with SSD and PF was twice as high as in the control group – 64.9 [48.9 76.3] IU/ml versus 32.2 [22.5–52.7] IU/ml (p<0.001). In group I, the TLA level was 84.7 [76.3–96.1] IU/ml and was 1.5 times higher than in group II – 55.3 [45.4–69.0] IU/ml (p<0.001). Observation a year later: the level of SpO2 continued to be reduced compared to the control group (p<0.01), The degree of dyspnea severity corresponded to the initial indicators – 3.0 [3.0-3.0] and 2.5 [2.0-3.0] points, respectively (p>0.05). A significant decrease in the TLA was noted in pts of group II from 55.3 [45.4–69.0] IU/ml to 35.6 [27.2–47.0] IU/ml (p<0.001), in pts of group I the TLA continued to be high – 85.7 [74.2–98.5] IU/ml. <bold>Conclusions:</bold> Assessment of the blood TLA in pts with SSD complicated by the PF progression may be an additional prognostic indicator of the pulmonary fibrotic process and severity.